Welcome to NH Blog. This is Dr Kiran. We have been exploring the wonderful world of pediatric cardiology for past few months. As previous, we shall continue with small anecdotes that convey more meanings.
The following story reached me via internet. One of the followers of this blog asked me to share this others. Due to credit to him, please go through this wonderful anecdote.
“How many times have you appreciated someone for no reason?” asked a renowned speaker in an annual meeting of a big company.
Eyebrows rose. “Why should we?” questioned the minds of top brass. “They have no eyes to appreciate the good, forget the same for no reason” mumbled the lower rung.
“Now think honestly for yourself; how many times you really felt like appreciating someone for their good work and did not do it for reasons that need not be disclosed?”
The hall went silent. The introspection was evident.
“After sometime”, continued the speaker, “it is very easy to forget the good job and the person who did it. Even if we try hard, we may not recall both the person and work. As the human nature goes, we forget the entire episode very easily.”
“So”, cried a voice from the middle of the crowd.
“Don’t postpone” said the wise man. “You have lost lot of opportunities to sincerely represent your feelings. Now, you can atone for it by appreciating someone who has done service to you. Let it be for no reason. Let it be a surprise for them. Let it come from nowhere for them. Try it out. Both of you will really feel better” he concluded.
The CEO of the firm thanked the speaker in his vote of thanks. "Our distinguished speaker made an exemplary point in his speech. We have been working on traditional moulds and outsets which preach that our relationships must be all business, cognitive, stand-offish and nonemotional. The most powerful thing he has taught us today is to inculcate a corporate culture with emotional human connection. From now on, I request all of you to freely express your appreciation to each other. This stuff could be contagious for a better world. Pass it on!"
When the function got over, the CEO was still haunted by the happenings of the day. On his way back home, he thanked his driver. "Thanks," he said. "For four years you have been my company driver and I don't think I have ever told you how wonderful you are. You are the best driver on the road. You are a first-class gentleman and an asset to our organization. I need you and deeply appreciate your friendship and loyalty."
The chauffeur was ecstatic. The CEO of the firm saying all the good things to him looked unbelievable. He was whistling and smiling like never before when he reached home. He went to his wife, hugged her and told, “I am feeling great today. I feel I have really achieved something. Lots of it is because of you. You are such a support and love. I don’t think I would have been the same without you. Thank you. Let’s go out for dinner tonight.”
The couple went to a nearby restaurant which was a tad expensive for them, but the man was too happy to afford that the day. Their son, who was sent out of the house for some reason a year back, worked there. The son didn't know what to say when he saw the parents. His father did something he could not believe. The father walked straight over to his son and hugged him. With tears in his eyes he said, "I love you, son. I appreciate you for what you are. Please come home. I miss you and need a relationship with you."
The boy went nervous. He had never seen his father like this. He excused himself from his parents and went into the manger’s chamber. "I've decided not to quit this job, sir. I have decide not to move out of this town," he told the manager. "I've just decided to move back in with my parents.” “I always thought your father was a strict disciplinarian. He never approved the free lifestyle you wanted to have. The boys tell me that you were sent out of the house when you got your body tattooed and changed your hair colour to join the music band. Why is the sudden change of decision?" the manager asked. "I just discovered my parents actually care about me and I'm sick of letting them down trying to prove my independence. It's time to make them proud!"
The story opens up a lot of things. There are lot of ways this can be interpreted. A genuine expression of appreciation is contagious and leads to good cascade. It is just the story of one person who followed it. The others who followed it might have had similar effects. The corporate culture has to break away from the mould. But, one word of warning. People with lesser maturity may not appreciate the difference between independence and indiscipline. So, even when a word of appreciation is handed over, the recipient should be really worth it. Try it out. It may be worth it.
With this, let us move on to our learning scenarios segment:
LOCATION: MATTERS?
Does the location of VSD matter for the velocity of the development of PVRI? In one of our meetings it was mentioned that between perimembranous VSD and subpulmonic VSD of same size, the latter develops early PVRI. I could not find any reference for the same. It can be logically concluded that the perimembranous VSDs have a chance of developing RVOT muscle bundles, but the subpulmonics would not. Also, the closer the VSD to the pulmonic valve, higher the chances of them behaving like aortopulmonary shunts. Does this theory find any takers? Are there any studies or observations to prove them? If similar findings are observed by you, please let me know.
JUXTAPOSED TGA
How common is it to find juxtaposed atrial appendage in TGA? We had a 2-month-old with TGA, intact IVS with juxtaposed atrial appendages. We had no recall of the last time we had this combination. Also, this baby had a smallish RPA with a normal sized LPA. How would one go about treating this combination? Our surgical team felt that a Senning would not be possible due to juxtaposition. The age and anatomy were not in favour of arterial switch. But, how to explain the disparity in the sizes of PAs? Has the appendage juxtaposition got anything to do with branch PA anatomy? Are they related in any way? Please let me know your ideas on it.
DISCONTINUITY
One of the major criteria for diagnosis of DORV is mitral-aortic discontinuity. When the great arteries are malposed, it would be mitral-pulmonic discontinuity. Does the same criteria hold good for DOLV too? Recently, we had a 6-month-old with this picture. AV concordance was clear. But, the PA was posterior and was completely committed to LV. There was a perimembranous malaligned VSD, with 25% override of d-posed anterior aorta. The mitral valve was clearly discontinuous with the posterior pulmonic valve. I reported it as TGA. However, my senior consultant changed the report to DORV based on the mitral-pulmonic discontinuity. Is it acceptable to call it a DORV when the posterior great vessel is totally committed to LV? Is the discontinuity clause a part of any Double outlet physiology or meant only for DORV? Since the number of DOLV are less, none of were very sure of this. I would like to know the opinion from the readership on this issue. If you come across such a picture, how would the report go? Please let me know.
TAPVC INTACT IAS
It was the second time in the recent past that we have come across a baby with TAPVC and intact IAS. The first one had a non-restrictive VSD and MR. The second one was latest – cardiac TAPVC with no detectable ASD or VSD and with only a restrictive PDA on echo. The surgical table found the final truth. There was indeed a small PFO, no VSD, restrictive PDA. But the surprise element was a partially unroofed coronary sinus, which went unnoticed on echo. It was very nice to know how the nature devises methods for the survival of the life. Any TAPVC with no flow across the MV is not known to survive, making the ASD near mandatory. Among the children of TAPVC with no ASD in our observation, the first one achieved a flow across the MV by MR and second one by a partially unroofed CS. It would be interesting to know the experiences of the readership. If you have come across any TAPVC with intact IAS, please let us know what other lesions led to a successful survival of the baby.
PRIMARY PROBLEM
Sometimes, certain situations can be perplexing with opinions on both poles. We had a 4-month-old with a large VSD shunting Right to Left, along with dilatation of RA and RV. There were no syndromic associations in this baby. It is very likely that the primary pulmonary resistance in this baby never dropped. It is extremely unlikely that the baby has already into Eisenmengarization. Now, in such scenarios, any further investigation may not yield useful information. How should this baby be approached? One end of spectrum is for a trial of sildenafil. On the other end lies suggestion for surgery. What way the readership vote? Is there a via-media way too? Please let me know your approach.
PEDIATRIC CARDIOLOGY PEARLS:
6. In a patient with typical auscultatory findings of an ASD and a P-wave axis of <30 degrees on the electrocardiogram, one should think immediately of a sinus venosus defect (Davia et al AHJ, 1973 page 180)
7. AV Canal defect is found commonly in children with Down syndrome. However, few specific characteristics are noted in Down syndrome:
• Situs and splenic anomalies are rare.
• They usually do not have associated LVOT obstruction, left ventricular hypoplasia, coarctation of the aorta, or additional muscular VSDs.
• Balanced AV canal defect is the commonest variant
(Marino. Cardiology in the Young, 1992, page 308)
8. Tachypneoa is a common symptom found in heart lesions with increased pulmonary blood flow (eg: ASD, VSD, PDA etc).
• In the absence of infection, the cardiovascular basis for the respiratory symptoms in such conditions probably is pulmonary edema of mild to moderate degree with elevated pulmonary venous pressure and decreased lung compliance.
• In infants with a large left-to-right shunt secondary to a VSD, dyspneoa occurs when the mean left atrial pressures reaches around 15 mm Hg.
(Donald. Progress in cardiovascular diseases, 1958, page 298)
9. In the fetus, the right ventricle ejects about two thirds of combined ventricular output, and because lung flow is only 6% to 8%, the ductus arteriosus carries 55% to 60% of combined ventricular output. (Heymann et al, Physiology review, 1975, page 65)
10. In any child with LV dysfunction, ECG is mandatory. If the ECG shows abnormal Q waves in leads I, aVL, and precordial leads V4 to V6 with or without abnormal R waves or R-wave progression in the left precordial leads, the likelihood of ALCAPA (Abnormal Left Coronary from Pulmonary Artery) is very high. All such children should undergo immediate Echocardiography in expert hands with an echo machine offering maximum resolution. (Moss and Adams Pediatric cardiology text book, 7th Ed, page 708)
With this, we come to the end of another post. Please mail your suggestions and feedback to drkiranvs@gmail.com or use the comments section. I am yet to receive comments on improving the new segment. Please ask your general pediatric friends about the blog and get their feedback.
Regards
Kiran
ಶನಿವಾರ, ಏಪ್ರಿಲ್ 17, 2010
ಮಂಗಳವಾರ, ಏಪ್ರಿಲ್ 13, 2010
Welcome to NH Blog. This is Dr Kiran anchoring you to this journey. We shall continue with anecdotes that convey more meanings.
I recalled this story when my erstwhile boss was narrating her experience in one of the hospital as attendant of a patient.
Divya was a cherubic, bubbly girl of 4 years. She started going to a nearby school. She was supposed to come back from the school with another baby from the neighbourhood along with an attendant. One day, the attendant of the neighbour boy told Divya’s mother that Divya refused to come with him and she is staying back in the school.
Her mom was surprised. Although Divya was naughty girl, she never did anything similar before in the past 4 months of school. A bit tensed, her mom walked towards the school.
At a small bench near the play area, Divya was sitting alone. Her face was not its usual bright.
“What happened to you?” demanded her mom.
“Nothing, mom”, replied Divya, still with some gloom on her face.
“Did anyone say anything to you?”
“Nope!”
“Then why are you not home yet? Why did you refuse to come with that uncle?” her mother sounded angry now.
“Actually, mom” Divya started. “My friend, you know, dropped her doll and it broke”
“So?”
“I was with her.”
“Did you stay back to repair her broken doll?” ridiculed her mother in a taunting way.
“Oh! mom. I don’t know how to repair her doll. I just stayed back when she was crying”
“Which friend? What is her name? Where is she now?
“I don’t know her name. She is my friend. She left just now with her dad. I saw you coming and stayed back.”
“What did you say when she was crying?” asked her mom, getting more curious now.
“I did not say anything. I just cried with her”
Her mom was speechless with overwhelming emotions.
It is quite satisfying to have a person standing by you silently in times of agony. It is probably one place where the hospital management has to look at. We usually have indifferent staff, busy nurses, super-busy doctors and all the other people in the hospital. Management is usually engaged in the balance sheets. Hardly anyone thinks of the situation from the point of patient’s family. Should we really face the situation head-on as a patient before realising the feelings they go through? That is too expensive way of learning things. Can we be more sympathetic for situations which demand common emotions from the entire humankind? The little girl in the above anecdote is probably teaching us a superior method of living as humans.
Let us get back to the section of interesting learning scenarios once again:
TOF with TAPVC
We recently had a couple of children with abnormal pulmonary venous connections associated with tetralogy of Fallot. First baby which was 1-year-old had two pulmonary veins entering the RA and the other 2 entering LA. This had gone to OT table, unnoticed in the echo. Second one was a much higher shocker! This 4-year-old had very poor echo windows and cath study was suggested to evaluate for additional VSDs. In the cath, the RA catheter entered RUPV. Hand injection of contrast showed the dye entering RA. The surgeon was informed about the association of PAPVC. However, on the table, the surprise was revealed. The baby had TAPVC! Was the hand injection of dye in cath not enough to opacify common confluence? We went back to the echo recordings, but the windows were so poor that we could not get any information on pulmonary venous return, especially in this baby with very small Qp. I am seriously considering a prospective study on the differences found on the OT table against the final diagnosis provided by the cardiology team. How are the experiences of other centres? Please let us know your experiences.
CHANGING PATH(OE)S!
We came across a 3-year-old operated for Tetralogy 2 years back. The boy was followed up in his native and came to us for the first time after the surgery. He was otherwise doing well, with no symptoms. On echo, we could not find LPA either in 2D or colour in any of the conventional views. The flow across RPA appeared normal. However, on clinical examination, his left lung had normal breath sounds, no added sounds. We wondered why the LPA was not visible. Small clue on chest radiograph was that the Left dome of diaphragm was high up with normal vascular pattern on both lung fields. It was probably paralysed during the surgical procedure and must be partially recovering now. It was likely that the orientation of lung has changed in such a way that the LPA must be traversing in a very odd angle and limited the visibility of LPA. This was only a hypothesis and we could not find any better explanation. We did not get any further investigations, as the clinical picture was very satisfactory and virtually no clues of abnormality in physical examination. Any experiences with such a pattern? If anyone has any better explanations, I would like to hear. How do you think such kids should be investigated? Please let me know your opinions on it.
NO COMPACT OPINION
Many times, we come across number of children with ventricles looking similar to non-compaction. What are the actual criteria for this diagnosis? Is a simple visual impression enough to say this? Are there any clear cut guidelines to say so? The text books describe it as spongy appearance of ventricular surface with thin epicardial layer and a thickened endocardial layer with prominent trabeculations and deep recesses. How to objectively evaluate this entity, which has only visual description? How many times do we really see ventricular dysfunction associated with this picture? How are the experiences with other centres? Please let me know your take on it.
DOUBLE DYSPLASIA
It was a child which disturbed our team a lot. This 3-month-old boy had truncus arteriosus. However, the truncal valve was quadricuspid and dysplastic. There was no other reason why this baby should not have had further procedures. However, is it worthwhile to convert this leaking dysplastic valve (which is not even repairable) into the future aortic valve? Is there any other option than rejecting the scenario as “untreatable”? Has anyone come across a similar phenomenon earlier? Please let me know your views on this.
REGURGITATIONS IN OPERABILITY
On an interesting note, we had three children with AP Window on a single day in our OPD. It was more than a coincidence. One among them was a 9-month-old with type 1 APW. This child also had mitral and tricuspid valve prolapses with severe regurgitations along with moderate aortic regurgitation. The net result was dilatation of all the cardiac chambers with reversal of flow in the aortic arch. The pulmonary venous return on the echo could not be assessed satisfactorily. Now the question was: How would one assess the operability on the echo for such children? The child saturated 93% in room air. Is that enough to claim operability? Is the cath study mandatory? Let me know how you would handle this issue.
Some of my friends who are pediatricians with lots of academic interest had an objection about the blog. They claim that it is too much of pediatric cardiology alone. They wanted something that would interest the general pediatric person with interest in Pediatric cardiology. Also, the post-graduate students in pediatrics wanted something that would interest them. With these in mind, I have decided to start a new section from now on. This section would be called “Pediatric cardiology pearls”. I would give 5 interesting and practical points in pediatric cardiology from a known work, along with the source. It should be good for the practitioner and students for management and quoting in the scientific meetings as well. Here goes the first instalment:
PEDIATRIC CARDIOLOGY PEARLS:
1. In the ECG of ASD, one can often see a notch near the apex of R wave in leads 2, 3 and avF which resembles the croche needle. This is called the crochetage sign. This has high specificity for the diagnosis of ASD if found along with the rsR’ pattern in the ECG. Contrary to the popular belief, the crochetage sign does not have a steady correlation with the shunt severity and is often found with PFO also. (Heller et al in JACC 1996, page 877)
2. An Eisenmenger VSD (also called Eisenmenger malalignment) is a perimembranous VSD with an anteriror deviation of infundibular septum. This has nothing to do with Eisenmenger syndrome or Eisenmenger complex (shunt reversal due to high pulmonary vascular resistance). (Fukuda et al in Cardiology in the Young 2000, page 343)
3. PDA is classified into 5 types: wide aortic end, wide pulmonary end, tubular, multiple constrictions and bizarre. (Krichenko et al AJC, 1989, page 879)
4. In the AV canal defect, distance from the cardiac crux to the left ventricular apex is foreshortened, and the distance from the apex to the aortic valve is increased. As a result, the LVOT is longer and narrower than normal and produces the “gooseneck” deformity. (Moss and Adams Pediatric cardiology text book, 7th Ed, page 646)
5. A combination of persistent left superior vena cava terminating in left atrium, atrial septal defect, and absence of roof of coronary sinus is called Raghib complex. It is associated with cyanosis – one of the few causes for cyanosis in ASD without Eisenmengarization (Raghib et al Circulation 1965, page 912)
With this, we come to the conclusion of another post. Please mail your suggestions and feedback to drkiranvs@gmail.com or use the comments section. Thanks for all the new followers. Please let me know how the new segment can be improved with the defined objectives that I have mentioned.
Regards
Kiran
I recalled this story when my erstwhile boss was narrating her experience in one of the hospital as attendant of a patient.
Divya was a cherubic, bubbly girl of 4 years. She started going to a nearby school. She was supposed to come back from the school with another baby from the neighbourhood along with an attendant. One day, the attendant of the neighbour boy told Divya’s mother that Divya refused to come with him and she is staying back in the school.
Her mom was surprised. Although Divya was naughty girl, she never did anything similar before in the past 4 months of school. A bit tensed, her mom walked towards the school.
At a small bench near the play area, Divya was sitting alone. Her face was not its usual bright.
“What happened to you?” demanded her mom.
“Nothing, mom”, replied Divya, still with some gloom on her face.
“Did anyone say anything to you?”
“Nope!”
“Then why are you not home yet? Why did you refuse to come with that uncle?” her mother sounded angry now.
“Actually, mom” Divya started. “My friend, you know, dropped her doll and it broke”
“So?”
“I was with her.”
“Did you stay back to repair her broken doll?” ridiculed her mother in a taunting way.
“Oh! mom. I don’t know how to repair her doll. I just stayed back when she was crying”
“Which friend? What is her name? Where is she now?
“I don’t know her name. She is my friend. She left just now with her dad. I saw you coming and stayed back.”
“What did you say when she was crying?” asked her mom, getting more curious now.
“I did not say anything. I just cried with her”
Her mom was speechless with overwhelming emotions.
It is quite satisfying to have a person standing by you silently in times of agony. It is probably one place where the hospital management has to look at. We usually have indifferent staff, busy nurses, super-busy doctors and all the other people in the hospital. Management is usually engaged in the balance sheets. Hardly anyone thinks of the situation from the point of patient’s family. Should we really face the situation head-on as a patient before realising the feelings they go through? That is too expensive way of learning things. Can we be more sympathetic for situations which demand common emotions from the entire humankind? The little girl in the above anecdote is probably teaching us a superior method of living as humans.
Let us get back to the section of interesting learning scenarios once again:
TOF with TAPVC
We recently had a couple of children with abnormal pulmonary venous connections associated with tetralogy of Fallot. First baby which was 1-year-old had two pulmonary veins entering the RA and the other 2 entering LA. This had gone to OT table, unnoticed in the echo. Second one was a much higher shocker! This 4-year-old had very poor echo windows and cath study was suggested to evaluate for additional VSDs. In the cath, the RA catheter entered RUPV. Hand injection of contrast showed the dye entering RA. The surgeon was informed about the association of PAPVC. However, on the table, the surprise was revealed. The baby had TAPVC! Was the hand injection of dye in cath not enough to opacify common confluence? We went back to the echo recordings, but the windows were so poor that we could not get any information on pulmonary venous return, especially in this baby with very small Qp. I am seriously considering a prospective study on the differences found on the OT table against the final diagnosis provided by the cardiology team. How are the experiences of other centres? Please let us know your experiences.
CHANGING PATH(OE)S!
We came across a 3-year-old operated for Tetralogy 2 years back. The boy was followed up in his native and came to us for the first time after the surgery. He was otherwise doing well, with no symptoms. On echo, we could not find LPA either in 2D or colour in any of the conventional views. The flow across RPA appeared normal. However, on clinical examination, his left lung had normal breath sounds, no added sounds. We wondered why the LPA was not visible. Small clue on chest radiograph was that the Left dome of diaphragm was high up with normal vascular pattern on both lung fields. It was probably paralysed during the surgical procedure and must be partially recovering now. It was likely that the orientation of lung has changed in such a way that the LPA must be traversing in a very odd angle and limited the visibility of LPA. This was only a hypothesis and we could not find any better explanation. We did not get any further investigations, as the clinical picture was very satisfactory and virtually no clues of abnormality in physical examination. Any experiences with such a pattern? If anyone has any better explanations, I would like to hear. How do you think such kids should be investigated? Please let me know your opinions on it.
NO COMPACT OPINION
Many times, we come across number of children with ventricles looking similar to non-compaction. What are the actual criteria for this diagnosis? Is a simple visual impression enough to say this? Are there any clear cut guidelines to say so? The text books describe it as spongy appearance of ventricular surface with thin epicardial layer and a thickened endocardial layer with prominent trabeculations and deep recesses. How to objectively evaluate this entity, which has only visual description? How many times do we really see ventricular dysfunction associated with this picture? How are the experiences with other centres? Please let me know your take on it.
DOUBLE DYSPLASIA
It was a child which disturbed our team a lot. This 3-month-old boy had truncus arteriosus. However, the truncal valve was quadricuspid and dysplastic. There was no other reason why this baby should not have had further procedures. However, is it worthwhile to convert this leaking dysplastic valve (which is not even repairable) into the future aortic valve? Is there any other option than rejecting the scenario as “untreatable”? Has anyone come across a similar phenomenon earlier? Please let me know your views on this.
REGURGITATIONS IN OPERABILITY
On an interesting note, we had three children with AP Window on a single day in our OPD. It was more than a coincidence. One among them was a 9-month-old with type 1 APW. This child also had mitral and tricuspid valve prolapses with severe regurgitations along with moderate aortic regurgitation. The net result was dilatation of all the cardiac chambers with reversal of flow in the aortic arch. The pulmonary venous return on the echo could not be assessed satisfactorily. Now the question was: How would one assess the operability on the echo for such children? The child saturated 93% in room air. Is that enough to claim operability? Is the cath study mandatory? Let me know how you would handle this issue.
Some of my friends who are pediatricians with lots of academic interest had an objection about the blog. They claim that it is too much of pediatric cardiology alone. They wanted something that would interest the general pediatric person with interest in Pediatric cardiology. Also, the post-graduate students in pediatrics wanted something that would interest them. With these in mind, I have decided to start a new section from now on. This section would be called “Pediatric cardiology pearls”. I would give 5 interesting and practical points in pediatric cardiology from a known work, along with the source. It should be good for the practitioner and students for management and quoting in the scientific meetings as well. Here goes the first instalment:
PEDIATRIC CARDIOLOGY PEARLS:
1. In the ECG of ASD, one can often see a notch near the apex of R wave in leads 2, 3 and avF which resembles the croche needle. This is called the crochetage sign. This has high specificity for the diagnosis of ASD if found along with the rsR’ pattern in the ECG. Contrary to the popular belief, the crochetage sign does not have a steady correlation with the shunt severity and is often found with PFO also. (Heller et al in JACC 1996, page 877)
2. An Eisenmenger VSD (also called Eisenmenger malalignment) is a perimembranous VSD with an anteriror deviation of infundibular septum. This has nothing to do with Eisenmenger syndrome or Eisenmenger complex (shunt reversal due to high pulmonary vascular resistance). (Fukuda et al in Cardiology in the Young 2000, page 343)
3. PDA is classified into 5 types: wide aortic end, wide pulmonary end, tubular, multiple constrictions and bizarre. (Krichenko et al AJC, 1989, page 879)
4. In the AV canal defect, distance from the cardiac crux to the left ventricular apex is foreshortened, and the distance from the apex to the aortic valve is increased. As a result, the LVOT is longer and narrower than normal and produces the “gooseneck” deformity. (Moss and Adams Pediatric cardiology text book, 7th Ed, page 646)
5. A combination of persistent left superior vena cava terminating in left atrium, atrial septal defect, and absence of roof of coronary sinus is called Raghib complex. It is associated with cyanosis – one of the few causes for cyanosis in ASD without Eisenmengarization (Raghib et al Circulation 1965, page 912)
With this, we come to the conclusion of another post. Please mail your suggestions and feedback to drkiranvs@gmail.com or use the comments section. Thanks for all the new followers. Please let me know how the new segment can be improved with the defined objectives that I have mentioned.
Regards
Kiran
ಗುರುವಾರ, ಮಾರ್ಚ್ 11, 2010
Welcome back to NH blog. This is Dr Kiran. We are in the process of finding something pertinent to our medical lives hidden in some of the known anecdotes.
The following anecdote was quoted by one of my friends. He had read it in a magazine few years back. He found the end very touching and narrated to me.
The story goes like this:
Mr and Mrs Shaw were worried. Unlike other members of their family, their youngest son Rahul was not an academic achiever, despite reaching his fifth standard.
The parents gave him everything a child could desire. Their only motive was to make him an academic success. They wanted their son to top the class, as did their other children. They were telling Rahul the virtues of good academic career. They would quote how his elder siblings are respected in the family for their academic excellence and so on. Not that Rahul was stupid. He was naughty, mischievous, playful, spoke a lot of nonsense, played well, but could not study and get good grades. His teachers were fed up with him. They would often call the parents to school to report him. They advised the parents to seek the help of a counsellor to rule out attention deficit hyperactivity. Many teachers could not just bear his presence in their class. All these were reflected in his report card as low grades. The school authorities threatened the parents of a transfer certificate if they did not get any better of him.
And, suddenly Rahul became better. His grades started improving. His attention span increased. There were literally no complaints from the school for more than a fortnight. It all sounded and felt magical for the parents. They had not done anything from their end, but still, something seemed to work.
This time when the parents were called to the school, they expected another taunt, but were taken by a surprise. The teacher had called them to congratulate on their effort. Rahul had got good grades in the unit test. The parents could not digest this. They started feeling guilty for the appreciation bestowed on them for no effort of theirs.
But the event deserved celebration. Both parents took Rahul for an ice-cream. In the parlour, Mr Shaw asked his son: “How are you doing all this?” He was worried if his son had started copying from his neighbour.
“I did not see anyone’s paper. It is all my own effort” said the lad.
“But how?” demanded Mrs Shaw this time.
“I have got a new friend. She works in my school. I talk to her all the time. I feel great with it”
“In the school?” thought Mr Shaw. “If the effort is done by the school to improve him, why are they complimenting us?”
“Something’s not fitting” he told his wife. “Can you show me your new friend?” he asked his son.
“Of course. She would still be at school. We can go now and meet her” said Rahul with a happy note in the voice.
They turned their car towards the school. Rahul led them towards the library. They found a lady in her twenties, arranging the books in the racks.
“What is her name?” asked Mr Shaw.
“I don’t know” was the prompt response from the son!
They waited till the lady was done with her work and returned to the counter. She saw Rahul and looked happy.
“These are my parents” introduced Rahul.
The lady bowed to them gracefully and smiled.
“Thanks for helping my son. We don’t know how to thank you enough”, said Mrs Shaw gently.
The lady did not say anything. She showed few gestures with her hands, which neither of the Shaws understood. All that they could make out was that she was using sign language used by verbally handicapped.
“Oh my God! She cannot speak?” said a shocked Mrs Shaw.
“Not just that. She can’t hear, read or write either. She is employed by the school to keep the library clean” said the Library in-charge who was passing by.
The parents looked perplexed. Mr Shaw came out of the shock and demanded with his son: “What sort of help have you got from her that improved you so much? She can’t teach you any academics; she doesn’t know how to write. Forget other things – she can’t even hear you.”
Smile could not leave Rahul’s face. “She does far more than hearing, Dad. She LISTENS!!”
We often miss this point. Most of the times, we medical professionals hear or preach. We do not listen. I have a nice recall from my medical school times. One doctor who was considered just mediocre for his medical knowledge was very popular with patients. Similarly, another doctor we used to adore for the clinical acumen and great subject knowledge was a total flop with patients. When this was discussed amongst friends, we would flatly declare that the patients are foolish; they are so ignorant and dumb that they can’t even recognise a good doctor from a bad one. We felt that we knew the definition of “good” far better than the patient.
On the hind sight, now I feel that the patients were not outright foolish. They sought what they wanted and got it. The doctor who was mediocre in our view was good for them, because he LISTENED. Our “great man” either heard or preached. We often witness how emotional catharsis helps the patients in psychiatry. But, we fail to extrapolate the same for ourselves. Many a times, the patients need a soothing soul to pour out their inner self. A good listener. But how many of us can afford to do so in our busy practise? When time becomes money for the professional, where is the scope to be a good listener? But, there should be a way out. There are many doctors who have successfully balanced both very nicely during their entire career. If you ARE one or know one, please communicate with our readership. Let the world “LISTEN” to you this time.
With this, let’s get back to our regular feature: Challenges in Pediatric Cardiology!
VIAGRA CONTROL
It has been few years that we are using sildenafil for the pulmonary hypertension and high PVRI. Indiscriminate use is one of the biggest challenges bothering the congenital heart care. We have seen doctors equating PAH with sildenafil. Despite all the efforts of journals and experts trying to create awareness on judicious use of sildenafil, the misuse is rampant. Is there is any role of Bosentan? How are comparative analogies between Bosentan and Sildenafil? Please let us know if any studies on this comparison are published.
LIMITATIONS OF VALVE
We had a 32-year-old with small subpulmonic VSD, bicuspid aortic valve and moderate AR. The issue was with the valve. No doubt that the VSD needs closure as per guidelines. But, is the valve repairable? If yes, then the effort is worth it. If not, then the patient needs valve replacement. Is the valve replacement worth in a 32-year-old, especially when the hemodynamic effect of VSD is small? What happens if the lesion is left untouched now? Is it possible to close VSD alone and not attempt the valve repair at all? All these questions bothering us, the patient data was placed in front of the surgeons. The surgical team decided to go ahead with the VSD closure and assured that the valve can be repaired. Their analogy was simple. If the VSD is closed with the AR left alone, the natural history of bicuspid aortic valve would be far superior to that with the VSD pulling the aortic valve cusp. If VSD is not tackled now, the patient may deteriorate faster. What is the experience with other centres? How would you tackle this scenario? Please let me know.
CONVENTIONAL CONFUSION
Sometimes, we wonder how fast we forget! Once we reach a level of practical comfort, the theory that was read becomes easily effaced. Same happened during one of our seminars recently. All of us remembered the classification of Tricuspid atresia as I, II, III based on great artery relations and A, B, C based on the pulmonary valve and pressures. Although I am quite fond of history, I had forgotten that the mentioned classification goes with the eponym of Kuhne. Our fellow, Dr Shweta, was presenting the seminar. She told us that the class IA was intact IVS AND pulmonary atresia, class IB was restrictive VSD AND pulmonary valvar stensosis. I wondered if the term AND is correct. The feeling was that AND should be replaced by OR/AND. However, she showed the reference from the text book (Moss and Adams). I was unable to get the original article by Kuhne et al. If anyone has any recall or has the original article, I request you to pass it on for the benefit of others.
LOCATION, LOCATION, LOCATION
I am yet to find a person who has completely understood the VSDs! This simple looking lesion can pose so many surprises, that the moment you get comfortable with it, a new problem, hitherto not experienced, pops up. In one of our discussions, our senior surgeon opined that the outlet VSDs have development of early PVRI compared to VSDs in other locations. The opinion was seconded by others. I could not recall any statement of this sort in the texts. I presumed the probable reason to be the proximity with pulmonic valve. It is likely that the hemophysiology of outlet VSDs simulate AP Windows to a certain extent. Is my presumption correct? If anyone can come with proper explanation with proof, you will be rewarded by a special mention in the blog; your name would be written in CAPITAL LETTERS!!
BLUE OR NOT
We were coursing through some of the interesting cases seen off late. We do have a series of single pulmonary arteries unpublished yet. One of such cases was a hemitruncus with intact septae. The question was: would this patient ever by cyanosed? If yes, why and how? I was emphatic that such patients would never have a cardiac cyanosis. The pulmonary artery exposed to the high pressure would eventually develop high PVRI and restricts the blood flow. The entire right heart output is going to single lung and it may eventually develop high PVRI. Till then, the patient should be pink. After bilateral high PVRI, the patient may have signs of low cardiac output and failure rather than cyanosis, unless the PFO opens up or pulmonary AV fistulae develop. Hence, clinically, the loudness of S2 would determine the status of the patient. Soft S2 shows patient has at least one good lung and loud P2 shows the development of high PVRI in the hitherto good lung. However, there were some arguments against my logic. What is your opinion? We may not have seen such a scenario, but may logically conclude the outcome. Let me know your takes on this issue.
This scenario marks the end of another post. Let me get the suggestions and answers to all these queries. Put them in the comments box or mail them to drkiranvs@gmail.com Let’s see how best this platform can be utilised for the purpose of mutual learning.
Regards
Kiran
The following anecdote was quoted by one of my friends. He had read it in a magazine few years back. He found the end very touching and narrated to me.
The story goes like this:
Mr and Mrs Shaw were worried. Unlike other members of their family, their youngest son Rahul was not an academic achiever, despite reaching his fifth standard.
The parents gave him everything a child could desire. Their only motive was to make him an academic success. They wanted their son to top the class, as did their other children. They were telling Rahul the virtues of good academic career. They would quote how his elder siblings are respected in the family for their academic excellence and so on. Not that Rahul was stupid. He was naughty, mischievous, playful, spoke a lot of nonsense, played well, but could not study and get good grades. His teachers were fed up with him. They would often call the parents to school to report him. They advised the parents to seek the help of a counsellor to rule out attention deficit hyperactivity. Many teachers could not just bear his presence in their class. All these were reflected in his report card as low grades. The school authorities threatened the parents of a transfer certificate if they did not get any better of him.
And, suddenly Rahul became better. His grades started improving. His attention span increased. There were literally no complaints from the school for more than a fortnight. It all sounded and felt magical for the parents. They had not done anything from their end, but still, something seemed to work.
This time when the parents were called to the school, they expected another taunt, but were taken by a surprise. The teacher had called them to congratulate on their effort. Rahul had got good grades in the unit test. The parents could not digest this. They started feeling guilty for the appreciation bestowed on them for no effort of theirs.
But the event deserved celebration. Both parents took Rahul for an ice-cream. In the parlour, Mr Shaw asked his son: “How are you doing all this?” He was worried if his son had started copying from his neighbour.
“I did not see anyone’s paper. It is all my own effort” said the lad.
“But how?” demanded Mrs Shaw this time.
“I have got a new friend. She works in my school. I talk to her all the time. I feel great with it”
“In the school?” thought Mr Shaw. “If the effort is done by the school to improve him, why are they complimenting us?”
“Something’s not fitting” he told his wife. “Can you show me your new friend?” he asked his son.
“Of course. She would still be at school. We can go now and meet her” said Rahul with a happy note in the voice.
They turned their car towards the school. Rahul led them towards the library. They found a lady in her twenties, arranging the books in the racks.
“What is her name?” asked Mr Shaw.
“I don’t know” was the prompt response from the son!
They waited till the lady was done with her work and returned to the counter. She saw Rahul and looked happy.
“These are my parents” introduced Rahul.
The lady bowed to them gracefully and smiled.
“Thanks for helping my son. We don’t know how to thank you enough”, said Mrs Shaw gently.
The lady did not say anything. She showed few gestures with her hands, which neither of the Shaws understood. All that they could make out was that she was using sign language used by verbally handicapped.
“Oh my God! She cannot speak?” said a shocked Mrs Shaw.
“Not just that. She can’t hear, read or write either. She is employed by the school to keep the library clean” said the Library in-charge who was passing by.
The parents looked perplexed. Mr Shaw came out of the shock and demanded with his son: “What sort of help have you got from her that improved you so much? She can’t teach you any academics; she doesn’t know how to write. Forget other things – she can’t even hear you.”
Smile could not leave Rahul’s face. “She does far more than hearing, Dad. She LISTENS!!”
We often miss this point. Most of the times, we medical professionals hear or preach. We do not listen. I have a nice recall from my medical school times. One doctor who was considered just mediocre for his medical knowledge was very popular with patients. Similarly, another doctor we used to adore for the clinical acumen and great subject knowledge was a total flop with patients. When this was discussed amongst friends, we would flatly declare that the patients are foolish; they are so ignorant and dumb that they can’t even recognise a good doctor from a bad one. We felt that we knew the definition of “good” far better than the patient.
On the hind sight, now I feel that the patients were not outright foolish. They sought what they wanted and got it. The doctor who was mediocre in our view was good for them, because he LISTENED. Our “great man” either heard or preached. We often witness how emotional catharsis helps the patients in psychiatry. But, we fail to extrapolate the same for ourselves. Many a times, the patients need a soothing soul to pour out their inner self. A good listener. But how many of us can afford to do so in our busy practise? When time becomes money for the professional, where is the scope to be a good listener? But, there should be a way out. There are many doctors who have successfully balanced both very nicely during their entire career. If you ARE one or know one, please communicate with our readership. Let the world “LISTEN” to you this time.
With this, let’s get back to our regular feature: Challenges in Pediatric Cardiology!
VIAGRA CONTROL
It has been few years that we are using sildenafil for the pulmonary hypertension and high PVRI. Indiscriminate use is one of the biggest challenges bothering the congenital heart care. We have seen doctors equating PAH with sildenafil. Despite all the efforts of journals and experts trying to create awareness on judicious use of sildenafil, the misuse is rampant. Is there is any role of Bosentan? How are comparative analogies between Bosentan and Sildenafil? Please let us know if any studies on this comparison are published.
LIMITATIONS OF VALVE
We had a 32-year-old with small subpulmonic VSD, bicuspid aortic valve and moderate AR. The issue was with the valve. No doubt that the VSD needs closure as per guidelines. But, is the valve repairable? If yes, then the effort is worth it. If not, then the patient needs valve replacement. Is the valve replacement worth in a 32-year-old, especially when the hemodynamic effect of VSD is small? What happens if the lesion is left untouched now? Is it possible to close VSD alone and not attempt the valve repair at all? All these questions bothering us, the patient data was placed in front of the surgeons. The surgical team decided to go ahead with the VSD closure and assured that the valve can be repaired. Their analogy was simple. If the VSD is closed with the AR left alone, the natural history of bicuspid aortic valve would be far superior to that with the VSD pulling the aortic valve cusp. If VSD is not tackled now, the patient may deteriorate faster. What is the experience with other centres? How would you tackle this scenario? Please let me know.
CONVENTIONAL CONFUSION
Sometimes, we wonder how fast we forget! Once we reach a level of practical comfort, the theory that was read becomes easily effaced. Same happened during one of our seminars recently. All of us remembered the classification of Tricuspid atresia as I, II, III based on great artery relations and A, B, C based on the pulmonary valve and pressures. Although I am quite fond of history, I had forgotten that the mentioned classification goes with the eponym of Kuhne. Our fellow, Dr Shweta, was presenting the seminar. She told us that the class IA was intact IVS AND pulmonary atresia, class IB was restrictive VSD AND pulmonary valvar stensosis. I wondered if the term AND is correct. The feeling was that AND should be replaced by OR/AND. However, she showed the reference from the text book (Moss and Adams). I was unable to get the original article by Kuhne et al. If anyone has any recall or has the original article, I request you to pass it on for the benefit of others.
LOCATION, LOCATION, LOCATION
I am yet to find a person who has completely understood the VSDs! This simple looking lesion can pose so many surprises, that the moment you get comfortable with it, a new problem, hitherto not experienced, pops up. In one of our discussions, our senior surgeon opined that the outlet VSDs have development of early PVRI compared to VSDs in other locations. The opinion was seconded by others. I could not recall any statement of this sort in the texts. I presumed the probable reason to be the proximity with pulmonic valve. It is likely that the hemophysiology of outlet VSDs simulate AP Windows to a certain extent. Is my presumption correct? If anyone can come with proper explanation with proof, you will be rewarded by a special mention in the blog; your name would be written in CAPITAL LETTERS!!
BLUE OR NOT
We were coursing through some of the interesting cases seen off late. We do have a series of single pulmonary arteries unpublished yet. One of such cases was a hemitruncus with intact septae. The question was: would this patient ever by cyanosed? If yes, why and how? I was emphatic that such patients would never have a cardiac cyanosis. The pulmonary artery exposed to the high pressure would eventually develop high PVRI and restricts the blood flow. The entire right heart output is going to single lung and it may eventually develop high PVRI. Till then, the patient should be pink. After bilateral high PVRI, the patient may have signs of low cardiac output and failure rather than cyanosis, unless the PFO opens up or pulmonary AV fistulae develop. Hence, clinically, the loudness of S2 would determine the status of the patient. Soft S2 shows patient has at least one good lung and loud P2 shows the development of high PVRI in the hitherto good lung. However, there were some arguments against my logic. What is your opinion? We may not have seen such a scenario, but may logically conclude the outcome. Let me know your takes on this issue.
This scenario marks the end of another post. Let me get the suggestions and answers to all these queries. Put them in the comments box or mail them to drkiranvs@gmail.com Let’s see how best this platform can be utilised for the purpose of mutual learning.
Regards
Kiran
ಮಂಗಳವಾರ, ಮಾರ್ಚ್ 9, 2010
Welcome to NH blog. This is Dr Kiran.
From last post, we decided to put some anecdotes which carry a greater meaning than what meets the eye. In the attempt, I have picked up this small story one of my friends told me once. She also had no recall where she heard the story. It may very well be getting transmitted verbally from many. If this story is in print anywhere, I would be indebted to know the same from the readership.
The story goes something like this:
A board hung on a pet store caught the attention of this young boy of nine.
PUPPIES FOR SALE
Boy slowly went into the shop and smiled at the plump owner.
“How much are the puppies?”
“It would be Rs 300/- each. We have left with only seven more. Have cash or wanna come back with dad?”
The boy meddled with his pockets, brought out some change and counted.
“I have Rs 45/- only now. Can I see the puppies?”
The store owner was a kind man. “Come on in!” he said.
As the boy got in, he found seven cute creamish puppies running hither and thither. A smile laced his face. To his surprise, he found the eighth puppy limping along.
“I heard seven. But they are eight out there!”
The shop keeper looked down the place and said:
“Oh, that’s a defective one. We showed him to the doctor. This puppy has some problem in legs by birth. I have not included him in the list”
“Will he always limp like that?”
“Yes, my boy. Thats why I did not tell you about him”
“I want to buy him!” the boy said.
“No point, child. I don’t even have him in the list. I don’t want to sound cheating a little boy. If you still think you want him, I will give him to you for free.”
The boy’s face grew red. He sounded angry. “Please don’t use the word ‘free’. I don’t want him free. I want to buy him. He is worth the same as all his other siblings. You please keep this Rs 45 as advance and book him for me. I will send my father to pay the rest.”
The shop owner was perplexed. He also had the admiration for this small boy at the same time. He tried to convince the boy.
“I think you should consult your parents before making this decision. You see, this puppy is never going to be a normal, playful one. You will never be able to take him out for fun. There is no chance of you jumping and playing with him.
The boy smiled, “You are wrong”. He slowly pulled his pant up. It showed steel braces around his deformed leg. “It is not about this puppy. Even I can’t run around and jump either. We make a pair because we can understand each other!”
The shop-owner was speechless.
It takes lot of empathy to understand the feelings of patients’ companions. It becomes glaring when the tables turn. I remember our erstwhile boss writing to all of us when her father was in the ICU of a prestigious hospital at Hyderabad. She came back after the demise of her dad and was so full of new ideas about patient care and communication. She could feel for all the parents waiting in the lobby just to hear something about their patient by one of the personnel from inside. She emphasized the need for more frequent, easy and meaningful communications with patient party. She urged the importance of supporting the family at the times of distress and impending death. She conceived the idea of keeping the patient happy till the last moment by creating an atmosphere of solace for everyone. She suggested that we should allow the parents to stay bedside during the last moments of the child and the mutual satisfaction that can be provided when the parents can hold the baby’s hands in those moments. She mooted the proposal of making the end as beautiful as possible for the tender hearts.
Not that our brilliant boss could not have thought of all these issues prior. She probably had in bits and pieces. However, everything got crystallised into a solid structure when she went through the experience with tables turned around. She could empathize with her patients better after her experience with her father’s demise. One example of how a committed person would see the changes around with a positive outlook.
Let me know your views on it. Also, if you have read or heard of small anecdotes, please send them to me. Your contribution would be promptly acknowledged!
With this, let us get back to our regular feature: Challenging scenarios.
IS THIS TRUNCUS POSSIBLE?
I remember sometime back when a cardiologist from a small volume centre presented Truncus arteriosus with intact interventricular septum. We were smiling at the possible embryological nonsense. The shock was on us when we saw a baby with same diagnosis. Our surgical team questioned the well being of cardiology team when we mentioned this to them. We showed them the echo images to stun them. Our enthusiastic fellow, Dr Karunakar had already gone a step ahead and pulled out a couple of articles from his bag on the previous case reports of Truncus with intact IVS! The question was of the management. This 2-month-old was not very well at presentation. The surgeon went through the literature and had a couple of plans for him based on the anatomy on the table. Sadly, the baby did not make it to surgery and expired in the ICU. This is the first time we had come across any Truncus with intact IVS (we have operated 115 cases of truncus so far at NH). Please let me know if you have seen a truncus with intact IVS and how it was managed.
UNANSWERED McGOON
This has been a perpetual question that might have haunted generations. Probably, I have posted it earlier also. When one of the pulmonary arteries is far smaller than the other and the McGoon’s ratio becomes acceptable, what is the approach to be taken? We often see the surgical and cardiology teams split on the issue. What are the opinions from other centres? Please let me know your expertise in it.
WHOLE OR HOLE?
We had an eight year old with DORV, d-malposed great arteries, VSD, pulmonary atresia and intact IAS. This child saturated 78%. Although there were 2 good sized ventricles, the VSD was not routable. The question was whether to create a BTT shunt or to go for a BD Glenn shunt. The house was spilt on the decision. One half felt that the BTT shunt might be better, as BD Glenn has long term issues. The other half felt argued that the volume load on the ventricle by a BTT shunt would be detrimental on a long run and BD Glenn would serve more than one purpose. It was decided to measure the PA pressures on the table and go with BD Glenn if OK; otherwise to settle for BTT shunt. The other interesting question was – whether an ASD would be required in this case? Since the aorta was more committed to RV and VSD was just reasonable subpulmonic type, should this baby have an ASD to compliment mixing? This was a tough question to answer, as the surgeries proposed were off-pump and to create an ASD, one need to go on-pump. The surgical team could not recall any similar situation from their kitty of experience. Neither was the cardiology team sure of the answer. If anyone had such experience, please enlighten us.
BEHAVIOUR: BEFORE AND AFTER
We had a couple of children with VSD, PS, small RV and had gone through BD Glenn shunt few years back. Now, they returned with progressive restriction of VSD and symptoms. RV was suprasystemic on cath study. The RV EDP had gone up beyond the permissible level for Fontan completion. Although the Glenn shunt was still partially functioning, it was probably a matter of time. How should one go about such situations? Creation of a larger ASD may serve them for few more years and not a long term solution. Is there anything like enlarging or creating a VSD? The surgical team quoted “Prohibitive risk”. Is it the final word? Is there anything that can better such situations? Please tell us about your experiences.
ORTHOTOPY Vs HETEROTOPY
An interesting observation was quoted by one of our senior cardiologists, Dr PV Suresh. Amidst his ever-flowing OPD patients, he had observed the natural history of pulmonary homografts in Rastelli Vs Ross procedures. His observations showed that the Ross homografts behave far superior to the Rastelli homografts. He was seeking the possible explanations for this. The surgical team was represented by Dr Shekar Rao, who has exhaustive experience in both the procedures. Apart from the individual magnitude of both procedures and patient selection, Dr Shekar Rao quoted the advantages of orthotopic positioning of Ross homograft and possible disadvantages of heterotopic positioning of Rastelli homgrafts. The discussion was interesting for senior group and eye-opening for the freshers. We ended up understanding the hemodynamic variations of a 3-D angulation of cardiac structures. It is always emphasized that we cardiologist should have a mental 3-D reconstruction of anatomy with the 2-D data what we get from conventional imaging. This time, we could gauge the 3-D physiology also! We are indebted to both our senior consultants for the new light. Not to boast of ourselves, that’s probably what makes NH a sought after place for students!!
END OF AN ERA
After going through a series of emotional tides, the NH team is settling now. Dr Sunita Maheshwari, our beloved “Boss” decided to quit her post from NH to make space for her dreams. The decision was greeted with surprise, anguish, shock, anger, sorrow and mix of emotions from other team members. The Fellows were not very happy with the development, although it does not affect them on a long term. Such was the gravity created within. On the day of commemoration of a decade of Pediatric cardiology in NH, we also witnessed the handing over of charge to Dr PV Suresh. Although Dr Sunita has promised to come once a week for teaching, we know it is not long term happening. Personally, I feel that Dr Sunita’s achievement lies in the fact that she has prepared the department for her absence. It is no doubt the sign of a leader with a positive vision. Now, it is left for the team to honour her by achieving greater heights despite her absence. That’s one way I think we can thank her. We all wish for an uninterrupted flow of her creative energies and scaling of greater heights she is ever capable of. Adieu.
That brings us to the end of another post. I am yet to receive any comments for the new effort. Please let me know if it is worth continuing. Come out with any better formats for presentation. Put them in the comments box or mail them to drkiranvs@gmail.com I shall consider them seriously and get back to the readership. I hope to get more frequent henceforth.
Regards
Kiran
From last post, we decided to put some anecdotes which carry a greater meaning than what meets the eye. In the attempt, I have picked up this small story one of my friends told me once. She also had no recall where she heard the story. It may very well be getting transmitted verbally from many. If this story is in print anywhere, I would be indebted to know the same from the readership.
The story goes something like this:
A board hung on a pet store caught the attention of this young boy of nine.
PUPPIES FOR SALE
Boy slowly went into the shop and smiled at the plump owner.
“How much are the puppies?”
“It would be Rs 300/- each. We have left with only seven more. Have cash or wanna come back with dad?”
The boy meddled with his pockets, brought out some change and counted.
“I have Rs 45/- only now. Can I see the puppies?”
The store owner was a kind man. “Come on in!” he said.
As the boy got in, he found seven cute creamish puppies running hither and thither. A smile laced his face. To his surprise, he found the eighth puppy limping along.
“I heard seven. But they are eight out there!”
The shop keeper looked down the place and said:
“Oh, that’s a defective one. We showed him to the doctor. This puppy has some problem in legs by birth. I have not included him in the list”
“Will he always limp like that?”
“Yes, my boy. Thats why I did not tell you about him”
“I want to buy him!” the boy said.
“No point, child. I don’t even have him in the list. I don’t want to sound cheating a little boy. If you still think you want him, I will give him to you for free.”
The boy’s face grew red. He sounded angry. “Please don’t use the word ‘free’. I don’t want him free. I want to buy him. He is worth the same as all his other siblings. You please keep this Rs 45 as advance and book him for me. I will send my father to pay the rest.”
The shop owner was perplexed. He also had the admiration for this small boy at the same time. He tried to convince the boy.
“I think you should consult your parents before making this decision. You see, this puppy is never going to be a normal, playful one. You will never be able to take him out for fun. There is no chance of you jumping and playing with him.
The boy smiled, “You are wrong”. He slowly pulled his pant up. It showed steel braces around his deformed leg. “It is not about this puppy. Even I can’t run around and jump either. We make a pair because we can understand each other!”
The shop-owner was speechless.
It takes lot of empathy to understand the feelings of patients’ companions. It becomes glaring when the tables turn. I remember our erstwhile boss writing to all of us when her father was in the ICU of a prestigious hospital at Hyderabad. She came back after the demise of her dad and was so full of new ideas about patient care and communication. She could feel for all the parents waiting in the lobby just to hear something about their patient by one of the personnel from inside. She emphasized the need for more frequent, easy and meaningful communications with patient party. She urged the importance of supporting the family at the times of distress and impending death. She conceived the idea of keeping the patient happy till the last moment by creating an atmosphere of solace for everyone. She suggested that we should allow the parents to stay bedside during the last moments of the child and the mutual satisfaction that can be provided when the parents can hold the baby’s hands in those moments. She mooted the proposal of making the end as beautiful as possible for the tender hearts.
Not that our brilliant boss could not have thought of all these issues prior. She probably had in bits and pieces. However, everything got crystallised into a solid structure when she went through the experience with tables turned around. She could empathize with her patients better after her experience with her father’s demise. One example of how a committed person would see the changes around with a positive outlook.
Let me know your views on it. Also, if you have read or heard of small anecdotes, please send them to me. Your contribution would be promptly acknowledged!
With this, let us get back to our regular feature: Challenging scenarios.
IS THIS TRUNCUS POSSIBLE?
I remember sometime back when a cardiologist from a small volume centre presented Truncus arteriosus with intact interventricular septum. We were smiling at the possible embryological nonsense. The shock was on us when we saw a baby with same diagnosis. Our surgical team questioned the well being of cardiology team when we mentioned this to them. We showed them the echo images to stun them. Our enthusiastic fellow, Dr Karunakar had already gone a step ahead and pulled out a couple of articles from his bag on the previous case reports of Truncus with intact IVS! The question was of the management. This 2-month-old was not very well at presentation. The surgeon went through the literature and had a couple of plans for him based on the anatomy on the table. Sadly, the baby did not make it to surgery and expired in the ICU. This is the first time we had come across any Truncus with intact IVS (we have operated 115 cases of truncus so far at NH). Please let me know if you have seen a truncus with intact IVS and how it was managed.
UNANSWERED McGOON
This has been a perpetual question that might have haunted generations. Probably, I have posted it earlier also. When one of the pulmonary arteries is far smaller than the other and the McGoon’s ratio becomes acceptable, what is the approach to be taken? We often see the surgical and cardiology teams split on the issue. What are the opinions from other centres? Please let me know your expertise in it.
WHOLE OR HOLE?
We had an eight year old with DORV, d-malposed great arteries, VSD, pulmonary atresia and intact IAS. This child saturated 78%. Although there were 2 good sized ventricles, the VSD was not routable. The question was whether to create a BTT shunt or to go for a BD Glenn shunt. The house was spilt on the decision. One half felt that the BTT shunt might be better, as BD Glenn has long term issues. The other half felt argued that the volume load on the ventricle by a BTT shunt would be detrimental on a long run and BD Glenn would serve more than one purpose. It was decided to measure the PA pressures on the table and go with BD Glenn if OK; otherwise to settle for BTT shunt. The other interesting question was – whether an ASD would be required in this case? Since the aorta was more committed to RV and VSD was just reasonable subpulmonic type, should this baby have an ASD to compliment mixing? This was a tough question to answer, as the surgeries proposed were off-pump and to create an ASD, one need to go on-pump. The surgical team could not recall any similar situation from their kitty of experience. Neither was the cardiology team sure of the answer. If anyone had such experience, please enlighten us.
BEHAVIOUR: BEFORE AND AFTER
We had a couple of children with VSD, PS, small RV and had gone through BD Glenn shunt few years back. Now, they returned with progressive restriction of VSD and symptoms. RV was suprasystemic on cath study. The RV EDP had gone up beyond the permissible level for Fontan completion. Although the Glenn shunt was still partially functioning, it was probably a matter of time. How should one go about such situations? Creation of a larger ASD may serve them for few more years and not a long term solution. Is there anything like enlarging or creating a VSD? The surgical team quoted “Prohibitive risk”. Is it the final word? Is there anything that can better such situations? Please tell us about your experiences.
ORTHOTOPY Vs HETEROTOPY
An interesting observation was quoted by one of our senior cardiologists, Dr PV Suresh. Amidst his ever-flowing OPD patients, he had observed the natural history of pulmonary homografts in Rastelli Vs Ross procedures. His observations showed that the Ross homografts behave far superior to the Rastelli homografts. He was seeking the possible explanations for this. The surgical team was represented by Dr Shekar Rao, who has exhaustive experience in both the procedures. Apart from the individual magnitude of both procedures and patient selection, Dr Shekar Rao quoted the advantages of orthotopic positioning of Ross homograft and possible disadvantages of heterotopic positioning of Rastelli homgrafts. The discussion was interesting for senior group and eye-opening for the freshers. We ended up understanding the hemodynamic variations of a 3-D angulation of cardiac structures. It is always emphasized that we cardiologist should have a mental 3-D reconstruction of anatomy with the 2-D data what we get from conventional imaging. This time, we could gauge the 3-D physiology also! We are indebted to both our senior consultants for the new light. Not to boast of ourselves, that’s probably what makes NH a sought after place for students!!
END OF AN ERA
After going through a series of emotional tides, the NH team is settling now. Dr Sunita Maheshwari, our beloved “Boss” decided to quit her post from NH to make space for her dreams. The decision was greeted with surprise, anguish, shock, anger, sorrow and mix of emotions from other team members. The Fellows were not very happy with the development, although it does not affect them on a long term. Such was the gravity created within. On the day of commemoration of a decade of Pediatric cardiology in NH, we also witnessed the handing over of charge to Dr PV Suresh. Although Dr Sunita has promised to come once a week for teaching, we know it is not long term happening. Personally, I feel that Dr Sunita’s achievement lies in the fact that she has prepared the department for her absence. It is no doubt the sign of a leader with a positive vision. Now, it is left for the team to honour her by achieving greater heights despite her absence. That’s one way I think we can thank her. We all wish for an uninterrupted flow of her creative energies and scaling of greater heights she is ever capable of. Adieu.
That brings us to the end of another post. I am yet to receive any comments for the new effort. Please let me know if it is worth continuing. Come out with any better formats for presentation. Put them in the comments box or mail them to drkiranvs@gmail.com I shall consider them seriously and get back to the readership. I hope to get more frequent henceforth.
Regards
Kiran
ಭಾನುವಾರ, ಫೆಬ್ರವರಿ 21, 2010
This is Dr.Kiran welcoming the readers to NH blog. The blog aims to put something "old and fresh" of Pediatric Cardiology everytime and invites the readership to actively participate in the dialogue process.
Let me render my apologies to begin with. It has been more than one month that I have posted anything. I was a bit busy with an article for publication, under a strict ultimatum! Howsoever small the readership might be, I owe this explanation to them. Hence this.
So far, we have seen something about the historical developments, Nobel prize winners and historical developments of drugs: all in relation to Pediatric Cardiology.
I had run out of the ideas and asked the readers on some suggestions on the topics of possible interest. As usual, I have got none! So, I have tried to do something which was suggested sometime back by one of the very good friends and a fabulous team-member, Dr Vishal Changela. He strongly felt that some personal medical anecdotes about our professional relationship with patients should be put up for others to read. In the absence of any other better things to write, I am trying this from now on.
Long back, I had heard a short story written by one of the greatest short-story writers of Kannada, the legendary Dr Masti Venkatesha Iyengar. It is a simple village story. One evening in one of the villages neighbouring a forest, a cowherd realises that a buffalo and its calf are missing. Since it is already dark, they fear entering the forest in search of the missing duo. He keeps his fingers crossed and waits till the dawn. A search party with the armamentarium enters the forest carefully. After a couple of hours of search in the deep forest, they hear the hunger cries of a calf. They start towards the sound and find the same calf of the buffalo safe and secure. In a short distance from this, they find the wounded buffalo in a pool of blood, breathing heavily. To their shock, they find the paw marks of tiger moving away from the spot with blood splattered all along its course, disappearing into the heart of the forest. They immediately realised the entire sequence of events. The calf must have wandered into the forest unknowingly in the late evening and its mother must have followed it to safeguard the kid. A tiger must have attacked the calf and the docile buffalo had fought the mighty tiger tooth and nail throughout the night to save its baby. It finally succeeded at the cost of its own life, driving the deadly beast wounded and away. Just imagining a tame buffalo fighting a ferocious tiger in the night to protect its calf keeping its own life on stake was nerve wrecking and emotions filled the souls of those poor peasants. The villagers carried the wounded buffalo and the calf back to the village. The buffalo eventually died and the villagers buried it with full honours that befitted a brave person and had a small monument built in the memory of its love and courage. This true story stands as a phenomenal symbol of selfless sacrifice of a mother for its child.
The story brings about various kinds of emotions in the reader. Few imagine the scenario and feel for the motherhood. Few imagine themselves in the scenario and try emoting. Few extrapolate the real life situations and think of cross references. What comes out an anecdote is your experience and empathy inside. We all feel for the sacrifice of the buffalo largely because of the courage this helpless, docile and mute animal showed at the time of danger and crisis to its calf. It is probably the basic nature of this animal that stands contrast to the courage it displayed, which fills the mind.
We do see situations in the hospital similar to the one the anecdotal buffalo underwent. We see parents who would sacrifice what all they can to get their children treated. We see them selling the last piece of jewellery, spreading their hand in front of the last available donor, saving money by choosing the least expensive accommodation for themselves or taking just one lunch and so on when the child is admitted in the hospital. All the gruesome effort happens despite accepting the risk and keeping the outcome sceptical. The sacrifice appears to be hanging only to a 4-letter word: HOPE. I am often reminded of the anecdotal buffalo whenever I come across such situations. The scenario of the otherwise helpless buffalo is no different from helpless parents here. The buffalo fought with mighty, cruel tiger and the parents here fight with mightier, more cruel and more than that - an unseen destiny. I feel the sacrifices on both occasions are no less than each other. The first scenario got noticed. But the latter goes unnoticed all the time.
These are the kind of anecdotes that we would like to present. It need not always be tragical. Some triumphs can also be interspersed. At finale, what re-shines is the humanity. The Mahabharata says, “there needs to be only one religion and one principle in the world: compassion to the fellow life”. It is said that the humanity re-lives every time it triumphs. If you have any such anecdotes, please send them to me via email to drkiranvs@gmail.com I shall acknowledge your contribution and give full credit to you for your story!!
I shall get back to our regular feature: Interesting and perplexing case scenarios
TO OR NOT TO TIGHTEN
When the pre-Glenn shunt cath procedure is done, we often see PA pressures being borderline high, but all other parameters would favour Glenn shunt. In such scenarios, few suggest tightening of MPA and going ahead with Glenn. It may get the circuit OK for Glenn, but may reduce the SO2. Is this acceptable? Isn’t good SO2 one of the objectives of Glenn? By tightening the MPA, do we always achieve the Glenn suitability? If anyone found answers for these, please let me know.
BALLOONING IN REGURGITATION
In cases of severe Aortic stenosis with moderate aortic regurgitation, can we consider ballooning? We had a 13-year-old with this scenario. His aortic annulus was very small and looked mildly dysplastic. Surgical team felt that placing a good sized aortic prosthesis was not possible. Doing a Konno was considered very risky. Annuli were not suitable for Ross. In such cases, can we contemplate controlled balloon dilatation, accepting the eventual AR? Our senior consultant was of the opinion that free AR would be very dangerous in such children and balloon should not be attempted. Is the natural history in this subset any different from any kind of intervention? What would you do in such cases? Please put your comments on it.
Qp OR Qep?..
Numbers are funny. We have seen hair-splitting episodes on the basis of numbers generated after gruelling procedures! Whether operability or fitness for univentricular procedures, numbers do play a vital role in this field. During the combined cath meeting, the house is split many a times on this factor alone. We often find children cathed for operability. When the shunt is bidirectional, as per the standard references, we use the effective shunt – Qep. The Qep, many times, is significantly different from the Qp. In all such cases, should we use Qep or Qp as the denominator for calculating the PVRI? This alone can make the difference for the patient. We had a 7-year-old with VSD, PDA and PAH, whose operability was doubtful on the clinical grounds. Basic investigations could not be deciding. Cath showed reasonable Qp/Qs, but the PVRI was marginally high. Our team felt that the child should be operated, as this might be his last chance. Surgical team quoted the higher number PVRI and mooted inoperability. We were no sure how much to believe the numbers when the denominator was not fully certain. Is there a correction factor to the formulae when Qep is involved? Let me know your experience and literature back up if any.
PLACING THE BAND
An interesting analogy was discussed by the surgical team in one of the meetings. It is probably not textbook written and might be more experience oriented. While witnessing the angio images of a child who had undergone a PA banding as an infant in a hospital abroad, one of the senior surgeons commented that the said PA band was meant for a future single pump physiology. We were surprised how he could be so certain, as he was seeing the details of the patient for the first time! Then the surgeon pointed out that the PA band was placed close to the pulmonary valve, which is done to prevent distortion to the branch PAs. Similarly, he said, the PA bands are placed close to the bifurcation if a future 2-pump repair is contemplated. We were not certain if that was the rule, as we were considering both options in the present patient. Other members of the surgical team agreed with this. However, I could not recall reading about this anywhere, nor my cardiology colleagues. If anyone has read something about this, please enlighten us.
SINGLE VENTRICLE: WOULD LV/RV BE A FACTOR?
This was an interesting one. We had a 5-year-old with tricuspid atresia IIIC, who had undergone a PA band as an infant and saturating 88% at present. We had suggested single pump repair for the child. She fulfilled the criteria for single pump repair. However, the surgical team felt that the child can be followed up, as the saturations are OK. We argued that the long-term ventricular load should be the main consideration for present surgery. At this point, one of the senior surgeons commented that he would have taken it if it were to be a mitral atresia instead of tricuspid! He was of the opinion that the volume load on the ventricle would be a factor only when the pumping single chamber is RV. As this patient would have LV as the pumping single chamber, volume load should not be a consideration for present intervention. In any of the criteria of single ventricle, this factor does not find a place. We were using this logic for long term prognosis of single ventricle repair, but what our surgeon told appeared new. It may sound logical, but is there any consensus among the surgeons on this? How would you tackle this situation and argument? Please let me know your take on this.
TWO IN ONE?!
We had an infant with dTGA, ASD, small VSD and severe peripheral PS. The branch PAs appeared to have a tortuous course. The echo study alone was not enough to understand the branch PA anatomy. It was not very clear if they had multiple constrictions. From our side, we were not in consensus on the plan. We decided to place the scenario in the meeting. We showed the echo images and asked the surgeons to comment on it. Although it was decided for a close follow up at present, one of the surgeons felt that the LeCompte manoeuvre done during arterial switch can straighten the branch PAs and they may not require any additional intervention! This was hitherto unheard of! Again, sounded logical, but is it the fact? One can understand that the logic would work if the branch PAs have just folded upon themselves. Would the same logic apply if there are anatomical constrictions in the branch PAs? If anyone had any similar experiences earlier, please let us know the outcome and the way you went ahead with the plan.
That brings us to the end of the present post. I have tried something different; totally different from my areas of strength! If there are any differences of opinion, please let me know. If you think any better formats for presentation, please suggest. Put them in the comments box or mail them to drkiranvs@gmail.com I shall consider them seriously and get back to the readership
Regards
Kiran
Let me render my apologies to begin with. It has been more than one month that I have posted anything. I was a bit busy with an article for publication, under a strict ultimatum! Howsoever small the readership might be, I owe this explanation to them. Hence this.
So far, we have seen something about the historical developments, Nobel prize winners and historical developments of drugs: all in relation to Pediatric Cardiology.
I had run out of the ideas and asked the readers on some suggestions on the topics of possible interest. As usual, I have got none! So, I have tried to do something which was suggested sometime back by one of the very good friends and a fabulous team-member, Dr Vishal Changela. He strongly felt that some personal medical anecdotes about our professional relationship with patients should be put up for others to read. In the absence of any other better things to write, I am trying this from now on.
Long back, I had heard a short story written by one of the greatest short-story writers of Kannada, the legendary Dr Masti Venkatesha Iyengar. It is a simple village story. One evening in one of the villages neighbouring a forest, a cowherd realises that a buffalo and its calf are missing. Since it is already dark, they fear entering the forest in search of the missing duo. He keeps his fingers crossed and waits till the dawn. A search party with the armamentarium enters the forest carefully. After a couple of hours of search in the deep forest, they hear the hunger cries of a calf. They start towards the sound and find the same calf of the buffalo safe and secure. In a short distance from this, they find the wounded buffalo in a pool of blood, breathing heavily. To their shock, they find the paw marks of tiger moving away from the spot with blood splattered all along its course, disappearing into the heart of the forest. They immediately realised the entire sequence of events. The calf must have wandered into the forest unknowingly in the late evening and its mother must have followed it to safeguard the kid. A tiger must have attacked the calf and the docile buffalo had fought the mighty tiger tooth and nail throughout the night to save its baby. It finally succeeded at the cost of its own life, driving the deadly beast wounded and away. Just imagining a tame buffalo fighting a ferocious tiger in the night to protect its calf keeping its own life on stake was nerve wrecking and emotions filled the souls of those poor peasants. The villagers carried the wounded buffalo and the calf back to the village. The buffalo eventually died and the villagers buried it with full honours that befitted a brave person and had a small monument built in the memory of its love and courage. This true story stands as a phenomenal symbol of selfless sacrifice of a mother for its child.
The story brings about various kinds of emotions in the reader. Few imagine the scenario and feel for the motherhood. Few imagine themselves in the scenario and try emoting. Few extrapolate the real life situations and think of cross references. What comes out an anecdote is your experience and empathy inside. We all feel for the sacrifice of the buffalo largely because of the courage this helpless, docile and mute animal showed at the time of danger and crisis to its calf. It is probably the basic nature of this animal that stands contrast to the courage it displayed, which fills the mind.
We do see situations in the hospital similar to the one the anecdotal buffalo underwent. We see parents who would sacrifice what all they can to get their children treated. We see them selling the last piece of jewellery, spreading their hand in front of the last available donor, saving money by choosing the least expensive accommodation for themselves or taking just one lunch and so on when the child is admitted in the hospital. All the gruesome effort happens despite accepting the risk and keeping the outcome sceptical. The sacrifice appears to be hanging only to a 4-letter word: HOPE. I am often reminded of the anecdotal buffalo whenever I come across such situations. The scenario of the otherwise helpless buffalo is no different from helpless parents here. The buffalo fought with mighty, cruel tiger and the parents here fight with mightier, more cruel and more than that - an unseen destiny. I feel the sacrifices on both occasions are no less than each other. The first scenario got noticed. But the latter goes unnoticed all the time.
These are the kind of anecdotes that we would like to present. It need not always be tragical. Some triumphs can also be interspersed. At finale, what re-shines is the humanity. The Mahabharata says, “there needs to be only one religion and one principle in the world: compassion to the fellow life”. It is said that the humanity re-lives every time it triumphs. If you have any such anecdotes, please send them to me via email to drkiranvs@gmail.com I shall acknowledge your contribution and give full credit to you for your story!!
I shall get back to our regular feature: Interesting and perplexing case scenarios
TO OR NOT TO TIGHTEN
When the pre-Glenn shunt cath procedure is done, we often see PA pressures being borderline high, but all other parameters would favour Glenn shunt. In such scenarios, few suggest tightening of MPA and going ahead with Glenn. It may get the circuit OK for Glenn, but may reduce the SO2. Is this acceptable? Isn’t good SO2 one of the objectives of Glenn? By tightening the MPA, do we always achieve the Glenn suitability? If anyone found answers for these, please let me know.
BALLOONING IN REGURGITATION
In cases of severe Aortic stenosis with moderate aortic regurgitation, can we consider ballooning? We had a 13-year-old with this scenario. His aortic annulus was very small and looked mildly dysplastic. Surgical team felt that placing a good sized aortic prosthesis was not possible. Doing a Konno was considered very risky. Annuli were not suitable for Ross. In such cases, can we contemplate controlled balloon dilatation, accepting the eventual AR? Our senior consultant was of the opinion that free AR would be very dangerous in such children and balloon should not be attempted. Is the natural history in this subset any different from any kind of intervention? What would you do in such cases? Please put your comments on it.
Qp OR Qep?..
Numbers are funny. We have seen hair-splitting episodes on the basis of numbers generated after gruelling procedures! Whether operability or fitness for univentricular procedures, numbers do play a vital role in this field. During the combined cath meeting, the house is split many a times on this factor alone. We often find children cathed for operability. When the shunt is bidirectional, as per the standard references, we use the effective shunt – Qep. The Qep, many times, is significantly different from the Qp. In all such cases, should we use Qep or Qp as the denominator for calculating the PVRI? This alone can make the difference for the patient. We had a 7-year-old with VSD, PDA and PAH, whose operability was doubtful on the clinical grounds. Basic investigations could not be deciding. Cath showed reasonable Qp/Qs, but the PVRI was marginally high. Our team felt that the child should be operated, as this might be his last chance. Surgical team quoted the higher number PVRI and mooted inoperability. We were no sure how much to believe the numbers when the denominator was not fully certain. Is there a correction factor to the formulae when Qep is involved? Let me know your experience and literature back up if any.
PLACING THE BAND
An interesting analogy was discussed by the surgical team in one of the meetings. It is probably not textbook written and might be more experience oriented. While witnessing the angio images of a child who had undergone a PA banding as an infant in a hospital abroad, one of the senior surgeons commented that the said PA band was meant for a future single pump physiology. We were surprised how he could be so certain, as he was seeing the details of the patient for the first time! Then the surgeon pointed out that the PA band was placed close to the pulmonary valve, which is done to prevent distortion to the branch PAs. Similarly, he said, the PA bands are placed close to the bifurcation if a future 2-pump repair is contemplated. We were not certain if that was the rule, as we were considering both options in the present patient. Other members of the surgical team agreed with this. However, I could not recall reading about this anywhere, nor my cardiology colleagues. If anyone has read something about this, please enlighten us.
SINGLE VENTRICLE: WOULD LV/RV BE A FACTOR?
This was an interesting one. We had a 5-year-old with tricuspid atresia IIIC, who had undergone a PA band as an infant and saturating 88% at present. We had suggested single pump repair for the child. She fulfilled the criteria for single pump repair. However, the surgical team felt that the child can be followed up, as the saturations are OK. We argued that the long-term ventricular load should be the main consideration for present surgery. At this point, one of the senior surgeons commented that he would have taken it if it were to be a mitral atresia instead of tricuspid! He was of the opinion that the volume load on the ventricle would be a factor only when the pumping single chamber is RV. As this patient would have LV as the pumping single chamber, volume load should not be a consideration for present intervention. In any of the criteria of single ventricle, this factor does not find a place. We were using this logic for long term prognosis of single ventricle repair, but what our surgeon told appeared new. It may sound logical, but is there any consensus among the surgeons on this? How would you tackle this situation and argument? Please let me know your take on this.
TWO IN ONE?!
We had an infant with dTGA, ASD, small VSD and severe peripheral PS. The branch PAs appeared to have a tortuous course. The echo study alone was not enough to understand the branch PA anatomy. It was not very clear if they had multiple constrictions. From our side, we were not in consensus on the plan. We decided to place the scenario in the meeting. We showed the echo images and asked the surgeons to comment on it. Although it was decided for a close follow up at present, one of the surgeons felt that the LeCompte manoeuvre done during arterial switch can straighten the branch PAs and they may not require any additional intervention! This was hitherto unheard of! Again, sounded logical, but is it the fact? One can understand that the logic would work if the branch PAs have just folded upon themselves. Would the same logic apply if there are anatomical constrictions in the branch PAs? If anyone had any similar experiences earlier, please let us know the outcome and the way you went ahead with the plan.
That brings us to the end of the present post. I have tried something different; totally different from my areas of strength! If there are any differences of opinion, please let me know. If you think any better formats for presentation, please suggest. Put them in the comments box or mail them to drkiranvs@gmail.com I shall consider them seriously and get back to the readership
Regards
Kiran
ಗುರುವಾರ, ಜನವರಿ 14, 2010
Readers are welcome to the NH Blog. We are in the process of learning something about the developmental history of cardiac drugs. This time, we shall see the history of Aspirin in regard to the heart.
Before we start, I have some news which made us proud. Our boss, Dr Sunita Maheshwari was featured on the Cover of the prestigious India Today Woman in the January 2010 issue. (Incidentally, the accompanying regular issue of India Today had Aamir Khan on the Cover!) It was a matter of pride for the entire institute.
The second news was the glory we basked in the Madras Medical Mission. The annual teaching programme of MMM for the DM equivalents was held a week back. Our senior fellow, Dr Vishal Changela got the award for best case presentation. Our fellows literally dominated the session and no answer got missed from them. It stands as the solid testimony for our training and the quality of our fellows.
With this, lets go back to our regular content of blog.
One of the finest accounts of the history of a drug was written by a scientist who was directly involved in the development of the drug. Written in German, its English translation is as fascinating and soul-touching. The author of this historical account is Arthur Eichengrun, whose original article was published in 1949 in a German journal called Pharmazie. It is a “must read” for any enthusiast of medical history and (in my personal opinion) for everyone. The drug is the ubiquitous ASPIRIN. What we shall see is the development of cardiac utility of aspirin, skipping the anti-inflammatory history. For those who would be interested in the entire legacy of Aspirin history, I would strongly recommend a fantastic article by Walter Sneader in BMJ in the year 2000.
Lawrence Craven was a busy surgeon in California. His busy schedule would allow him minimal time to interact with patients and any question of personal touch was usually ruled out. On one of his busy day, he performed a tonsillectomy for a boy. Unable to get in touch with the surgeon after the surgery, the parents of the boy offered him a chewable aspirin for analgesia. The young patient started bleeding, worrying his surgeon for possibility of re-exploration. However, good sense prevailed and the surgeon decided to wait. Under some stern questioning, the parents spilled the beans and the surgeon went into a thinking mode! He made enquiries with other patients asking just 2 questions: Have you taken aspirin as post-surgical analgesia? If yes, did you experience any bleed? To his surprise, the OTC was consumed by many without his knowledge and universally had experienced visible bleed! The scientist in the surgeon made an appearance and he decided to investigate this further. He learnt that one Gibson had tried the use of Aspirin in a small number of patients with vascular diseases with equivocal response. Craven decided to make it big, but ended up being crude.
He tried around 8000 patients who consumed aspirin and came out with a claim that would surprise a seasoned physician! He claimed that none of these 8000 suffered a heart attack!! He could not publish his studies in any major journal as the trail was not a controlled study. The anticlimax of the study was reached when Craven himself died of a heart attack even after taking aspirin. The sceptics would have ended the saga of aspirin with this irony. However, good sense prevailed again and Harvey Weiss in New York could re-establish the role of Aspirin in 1967. He categorically demonstrated the acquired impairment of platelet aggregation in the subjects consuming aspirin by demonstrating prolonged bleeding time. He was the first to suggest the anti-thrombotic nature of aspirin and its use in clinical medicine. He urged the scientists to take up a larger trail in this regard. When it happened, there was no looking back. Aspirin was proved to be an effective anti-thrombotic agent. However, it took many years to come to the right dose that was minimal enough not to cause side-effects, but sufficient enough to provide the desired anti-thrombotic effect. As of today, aspirin stands as one of the most precious drugs in cardiac pharmacology.
With this, I am concluding the “Developmental history of Cardiac drugs”. I have not received any suggestions from any of the readers (if such a population exists!!) on what aspects of history to write next. May be, for next few sessions, I shall write only interesting cases and take up the historical aspects after some thinking.
On a personal note, the year 2010 is seeing lesser number of OPDs in Pediatric side. Possibly the festive season in Tamilnadu might be the reason. We should have taken this opportunity to relax a bit, but some of the events happening never let us!
VENOUS PVRI
I have posted this query in the past also, but it continues to haunt us on a regular basis. Hence, I am posting it again. This time, the patients are different, but the problem remains the same. We had one 16-year-old boy with Supramitral membrane with severe obstruction, Severe LVOT obstruction, large PDA and coarctation. The combination fits into Shone’s complex. The CoA was juxtaductal and the PDA was flowing right to left. The boy was desaturated in the lower limb with a significant drop in the LL saturation. His calculated baseline PVRI was 22 Wood units, which dropped to 9 Wood units with oxygen. Now the question remains the same. Is he operable? Isn’t the PVRI secondary to pulmonary venous hypertension reversible after correcting the cause? How much of PVRI is due to PDA and how much due to left heart obstruction? When put to vote, only a handful of people felt it was operable. The young guns of surgical team felt that the patient deserved to be operated as it might be his last chance for repair. However, the senior rung was not so much for it. They felt that the CoA was not much significant with PDA being the dominant lesion and it clearly shows inoperability. The decision was a bitter pill to swallow, but none of us have a concrete evidence for our stand. Such cases can be argued on either side. I questioned the need for cath study in such cases, as the pressure data and interpretations are dubious (may not be decision making in black and white) and the anatomical data might be much superior with a CT and a 3D reconstruction. However, the opinion favoured the cath study over CT. When I had to counsel the parents after the cath meet, I was choking for words. I personally felt that the boy should be given an opportunity to undergo surgery, accepting the high risk. However, the uneducated parents may not understand what we mean. I directed them to the surgical team. What bothers more is the fact that the next patient with similar problem would restart the enigma and we are no wiser by the experience of the present patient. Can anybody throw some light on this? I badly need some advice on management issues of such class of patients.
BALLOONING FOR SURGERY
We came across an infant who had undergone artereial switch as a neonate. He is otherwise asymptomatic, except a murmur in the precordium. Echo showed bilateral branch PA stenosis. Cath showed a significant gradient in branch PAs. Is there a role of balloon dilatation in such cases? How far is it successful? The age may not permit us to stent. Is isolated balloon dilatation recommended? Should we go ahead with surgery directly or give a trail of ballooning? Put in your ideas.
BAND ENIGMA
This question has come up in the past, but is another case for lack of consensus. We have a 6-month-old boywith single ventricle physiology (DILV) with mild PS. His mean PA pressure in cath was 36mmHg. Now, should we be doing a PA band? How far can we offer a BDG for him in future? Is he better off as he is now? Our chief was in favour of a PA band so as to keep the future options open, but the surgical team vehemently argued against it. I thought of putting the issue with both possibilities to clear the concepts for myself! I questioned the surgical team if we can anticipate any progress in PS as a part of natural history in this subset. It was interesting to know that such incidents have happened when the level of obstruction was at restricting VSD to produce a PS in Holmes heart. My next question was on the naturally history of a scenario with present PA band, but not suitable for future BDG. Would the eventual pressure overload on single ventricle be worse than the otherwise natural history? The answer had 2 interesting facets to it. Not only such a scenario increases the pressure overload on the heart, it also increases the cyanosis, because the afterload of pulmonary circulation would be greater than that of systemic. The band enigma was another unresolved issue which made us not much wiser at the end of it.
NORMALLY TRANSPOSED
Is it possible to have a TGA with normally related great arteries? We did see newborn with this picture. It had a clear VA discordance but the aorta came out of RV posteriorly, followed by the PA from LV, without the great arteries crossing each other. On the short axis, aorta was to the posterior and right with the PA anterior and left. Now, as per the segmental anatomy, they are {S,D,S}. Do they fit into NRGA? Is the component of crossing essential for defining normal relationship of great arteries? Can the term “Transposition” be applied if the vessels are normally related? Should we call it an isolated AV discordance or TGA? If anybody has seen such a picture before and had any thoughts gone into it, please enlighten us.
TRICUSPID SHONES
Does the definition of Shone’s complex involve the mitral valve, aortic valve and arch or does it involve left sided structures? We came across a case of corrected Transposition with sequential left heart obstruction. There was a tight supratricuspid membrane with subaortic membrane and a coarctation. Technically speaking, it does not fit the definition of Shone’s complex. This is the first time I have come across a cTGA with sequential left sided obstructive lesions. Our surgical team had a tough time operating this child. Tell me your experience if you have come across this combination anytime.
Send your comments. You can either use the comments section or use my email drkiranvs@gmail.com for posting your views and questions. Write about the issues you found perplexing and the way you have found the answer for them.
Regards
Kiran
Before we start, I have some news which made us proud. Our boss, Dr Sunita Maheshwari was featured on the Cover of the prestigious India Today Woman in the January 2010 issue. (Incidentally, the accompanying regular issue of India Today had Aamir Khan on the Cover!) It was a matter of pride for the entire institute.
The second news was the glory we basked in the Madras Medical Mission. The annual teaching programme of MMM for the DM equivalents was held a week back. Our senior fellow, Dr Vishal Changela got the award for best case presentation. Our fellows literally dominated the session and no answer got missed from them. It stands as the solid testimony for our training and the quality of our fellows.
With this, lets go back to our regular content of blog.
One of the finest accounts of the history of a drug was written by a scientist who was directly involved in the development of the drug. Written in German, its English translation is as fascinating and soul-touching. The author of this historical account is Arthur Eichengrun, whose original article was published in 1949 in a German journal called Pharmazie. It is a “must read” for any enthusiast of medical history and (in my personal opinion) for everyone. The drug is the ubiquitous ASPIRIN. What we shall see is the development of cardiac utility of aspirin, skipping the anti-inflammatory history. For those who would be interested in the entire legacy of Aspirin history, I would strongly recommend a fantastic article by Walter Sneader in BMJ in the year 2000.
Lawrence Craven was a busy surgeon in California. His busy schedule would allow him minimal time to interact with patients and any question of personal touch was usually ruled out. On one of his busy day, he performed a tonsillectomy for a boy. Unable to get in touch with the surgeon after the surgery, the parents of the boy offered him a chewable aspirin for analgesia. The young patient started bleeding, worrying his surgeon for possibility of re-exploration. However, good sense prevailed and the surgeon decided to wait. Under some stern questioning, the parents spilled the beans and the surgeon went into a thinking mode! He made enquiries with other patients asking just 2 questions: Have you taken aspirin as post-surgical analgesia? If yes, did you experience any bleed? To his surprise, the OTC was consumed by many without his knowledge and universally had experienced visible bleed! The scientist in the surgeon made an appearance and he decided to investigate this further. He learnt that one Gibson had tried the use of Aspirin in a small number of patients with vascular diseases with equivocal response. Craven decided to make it big, but ended up being crude.
He tried around 8000 patients who consumed aspirin and came out with a claim that would surprise a seasoned physician! He claimed that none of these 8000 suffered a heart attack!! He could not publish his studies in any major journal as the trail was not a controlled study. The anticlimax of the study was reached when Craven himself died of a heart attack even after taking aspirin. The sceptics would have ended the saga of aspirin with this irony. However, good sense prevailed again and Harvey Weiss in New York could re-establish the role of Aspirin in 1967. He categorically demonstrated the acquired impairment of platelet aggregation in the subjects consuming aspirin by demonstrating prolonged bleeding time. He was the first to suggest the anti-thrombotic nature of aspirin and its use in clinical medicine. He urged the scientists to take up a larger trail in this regard. When it happened, there was no looking back. Aspirin was proved to be an effective anti-thrombotic agent. However, it took many years to come to the right dose that was minimal enough not to cause side-effects, but sufficient enough to provide the desired anti-thrombotic effect. As of today, aspirin stands as one of the most precious drugs in cardiac pharmacology.
With this, I am concluding the “Developmental history of Cardiac drugs”. I have not received any suggestions from any of the readers (if such a population exists!!) on what aspects of history to write next. May be, for next few sessions, I shall write only interesting cases and take up the historical aspects after some thinking.
On a personal note, the year 2010 is seeing lesser number of OPDs in Pediatric side. Possibly the festive season in Tamilnadu might be the reason. We should have taken this opportunity to relax a bit, but some of the events happening never let us!
VENOUS PVRI
I have posted this query in the past also, but it continues to haunt us on a regular basis. Hence, I am posting it again. This time, the patients are different, but the problem remains the same. We had one 16-year-old boy with Supramitral membrane with severe obstruction, Severe LVOT obstruction, large PDA and coarctation. The combination fits into Shone’s complex. The CoA was juxtaductal and the PDA was flowing right to left. The boy was desaturated in the lower limb with a significant drop in the LL saturation. His calculated baseline PVRI was 22 Wood units, which dropped to 9 Wood units with oxygen. Now the question remains the same. Is he operable? Isn’t the PVRI secondary to pulmonary venous hypertension reversible after correcting the cause? How much of PVRI is due to PDA and how much due to left heart obstruction? When put to vote, only a handful of people felt it was operable. The young guns of surgical team felt that the patient deserved to be operated as it might be his last chance for repair. However, the senior rung was not so much for it. They felt that the CoA was not much significant with PDA being the dominant lesion and it clearly shows inoperability. The decision was a bitter pill to swallow, but none of us have a concrete evidence for our stand. Such cases can be argued on either side. I questioned the need for cath study in such cases, as the pressure data and interpretations are dubious (may not be decision making in black and white) and the anatomical data might be much superior with a CT and a 3D reconstruction. However, the opinion favoured the cath study over CT. When I had to counsel the parents after the cath meet, I was choking for words. I personally felt that the boy should be given an opportunity to undergo surgery, accepting the high risk. However, the uneducated parents may not understand what we mean. I directed them to the surgical team. What bothers more is the fact that the next patient with similar problem would restart the enigma and we are no wiser by the experience of the present patient. Can anybody throw some light on this? I badly need some advice on management issues of such class of patients.
BALLOONING FOR SURGERY
We came across an infant who had undergone artereial switch as a neonate. He is otherwise asymptomatic, except a murmur in the precordium. Echo showed bilateral branch PA stenosis. Cath showed a significant gradient in branch PAs. Is there a role of balloon dilatation in such cases? How far is it successful? The age may not permit us to stent. Is isolated balloon dilatation recommended? Should we go ahead with surgery directly or give a trail of ballooning? Put in your ideas.
BAND ENIGMA
This question has come up in the past, but is another case for lack of consensus. We have a 6-month-old boywith single ventricle physiology (DILV) with mild PS. His mean PA pressure in cath was 36mmHg. Now, should we be doing a PA band? How far can we offer a BDG for him in future? Is he better off as he is now? Our chief was in favour of a PA band so as to keep the future options open, but the surgical team vehemently argued against it. I thought of putting the issue with both possibilities to clear the concepts for myself! I questioned the surgical team if we can anticipate any progress in PS as a part of natural history in this subset. It was interesting to know that such incidents have happened when the level of obstruction was at restricting VSD to produce a PS in Holmes heart. My next question was on the naturally history of a scenario with present PA band, but not suitable for future BDG. Would the eventual pressure overload on single ventricle be worse than the otherwise natural history? The answer had 2 interesting facets to it. Not only such a scenario increases the pressure overload on the heart, it also increases the cyanosis, because the afterload of pulmonary circulation would be greater than that of systemic. The band enigma was another unresolved issue which made us not much wiser at the end of it.
NORMALLY TRANSPOSED
Is it possible to have a TGA with normally related great arteries? We did see newborn with this picture. It had a clear VA discordance but the aorta came out of RV posteriorly, followed by the PA from LV, without the great arteries crossing each other. On the short axis, aorta was to the posterior and right with the PA anterior and left. Now, as per the segmental anatomy, they are {S,D,S}. Do they fit into NRGA? Is the component of crossing essential for defining normal relationship of great arteries? Can the term “Transposition” be applied if the vessels are normally related? Should we call it an isolated AV discordance or TGA? If anybody has seen such a picture before and had any thoughts gone into it, please enlighten us.
TRICUSPID SHONES
Does the definition of Shone’s complex involve the mitral valve, aortic valve and arch or does it involve left sided structures? We came across a case of corrected Transposition with sequential left heart obstruction. There was a tight supratricuspid membrane with subaortic membrane and a coarctation. Technically speaking, it does not fit the definition of Shone’s complex. This is the first time I have come across a cTGA with sequential left sided obstructive lesions. Our surgical team had a tough time operating this child. Tell me your experience if you have come across this combination anytime.
Send your comments. You can either use the comments section or use my email drkiranvs@gmail.com for posting your views and questions. Write about the issues you found perplexing and the way you have found the answer for them.
Regards
Kiran
ಗುರುವಾರ, ಜನವರಿ 7, 2010
Hearty welcome to NH blog again. We are in the process of learning something about the developmental history of cardiac drugs. This time, we shall see the development of Calcium Channel blockers.
The 1,4-dihydropyridines have an important role in the human biochemistry. Despite the role it played and the ease with which it can be evaluated, very surprisingly, minimal research had taken place involving this vital compound. The research scientists group of Bayer laboratories learnt this and started working on it. True to their research reputation, they did not leave any stone unturned. Once they had a lead, they tested for its organ efficiency and found a reasonable change in the cardiac output. They focused their energies on the molecular variant and ended up developing and testing more than 2000 analogues! The result was a patent application for a new drug called Nifedipine in the year 1967 by two scientists named Friedrich Bossert and Wulf Vater. They registered the drug for its supposed activity as coronary vasodilator.
With that started the hunt for the actual action of Nifedipine. Over a couple of years, they found that nifedipine acted by blocking calcium channels in the cardiac conduction tissue and in the smooch muscles of blood vessels. Thereby, Nifedipine led to the relaxation of the myocardial tissue and blood vessels. The resultant action was coronary vasodilatation and drop in both systemic and pulmonary vascular resistance. As an obvious result, this should have reduced the afterload and oxygen consumption. Thus, it was a drug with potential benefit in angina with hypertension. With this background, Nifedipine had a grand entry into the markets in 1975.
The major hitch of Nifedipine was its short duration of action. As the popularity of the drug picked up, this issue was to be sorted out. The research groups started the action in two sides.
The first was to develop analogues of nifedipine for longer duration of action. This would have the advantage of a sustained action on blood pressure. The result was the development of felodipine and amlodipine. In the same bargain, some more drugs were developed, which showed a higher specificity for Calcium channels of other organ systems. Nimodipine was one such development with effect on cerebral blood vessels. The advantage was its specificity, in which it did not affect the systemic blood pressure. However, all this new developments would take high levels of research and finances, not to mention the time.
The second approach was more logical and much simpler: creation of a sustained release preparation of Nifedipine. This happened quite fast and was marketed easily. With the popularity Nifedipine enjoyed, it was not difficult to make the presence for the sustained release preparation. However, the research team was aware of the short life of this strategy and had realised the effacement of molecule once the new preparations took over. But the gamble had paid off. The SR preparation effectively bridged the gap and made the constant market for the drug till the newer generation ones took over. Even today, the dihydropyridine analogues enjoy a great market presence in the field.
Compared to the systematic research of Nifedipine, the other calcium channel blocker, Verapamil was more of serendipitous in its invention. It was supposedly a takeoff from the atropine in the field of antispasmodics. In this group came mebeverine, which enjoyed some success. However, the other molecule called verapamil did not have such luck. It was synthesised by the famous Knoll pharma. Its chief of research, Ferdinand Dengel has been credited for its creation and discovery of possible uses. Once the initial trials were over, Dengel realised that the drug was nowhere near the antispasmodic action. What caught his attention was its potential action as coronary dilator. He led the research to take over towards the use in angina. The success of the trials led to the introduction of the drug in the market in 1968. Soon it was realised that the drug can cause depression of conduction activity. This action prompted Albrecht Fleckenstein at the University of Freiburg to evaluate the molecule on those lines. That explained why the drug failed as an agent for smooth muscle relaxation; it was more specific for cardiac muscles! The much more interesting finding of the research was the reversibility of its action by calcium. This led Fleckenstein to assume that the drug was indeed a calcium blocker. Further research showed that the assumption was correct and Verapamil blocked the movement of calcium ions into cardiac cells. It was credited as the first calcium antagonists. As better understanding of the cardiac physiology and the role of calcium channels became apparent, the anti-arrhythmic action of Verapamil was acknowledged in 1970s. Its action as the relaxation agent also became useful in controlling the hypertension, albeit the use is minimal for this purpose at present.
The other calcium channel blocker, Diltiazem has much more serendipitous history! It was supposed to be sought as a psychotropic drug by the Japanese. The Tanabe Seiyaku Company of Japan was working in this regard. After huge number of possible variations in the combination of the medicational research, what they ended up with was Diltiazem! The initial drug was a racemic mixture of many isomers of the drug. The research team ended up segregating each isomer and started testing them. As a part of serendipity, the dextro isomer showed great vasodilator effect. The team decided to conduct the trial for this molecule. It was finally shown to have vasodilatory action simulating papaverine on coronary circulation. Logically, they tested it against calcium ion movement across cardiac muscle membrane. It was found to be a positive blocker! Thus was born the first coronary vasodilator which was actually a calcium antagonist. Later, as a comparison with the other drugs of the group, it was tested for its action on cardiac rhythm. It was positive for that action also! Theoretically, it stood between the Nifedipine and Verapamil, with good qualities of both in the same drug. Its spectrum makes it a desired preparation even today for selective indications.
Next issue may be the last one for the history of drug development. If anyone has any ideas for future posts, please let me know.
On a personal note, we had our departmental “publication meet” recently. As usual, many a write ups are in the long process of preparation. Our mercurial boss got impatient about the delay of her lethargic team. I was advised to give up blogging and to get serious with research. I think it is a good advice for now, as the number of people reading my blog does not make any justice to my effort. I can as well concentrate on writing papers and getting credit for publication! If you find the frequency of the blog posts getting reduced in the future, you know whom to blame!!
ATRETIC TRILEMMA!
How does a restrictive VSD in Tricuspid atresia type II behave? If anyone says as IIb, they are obviously wrong; it becomes subaortic obstruction! It is clearly unlike that of type I. But a beginner needs to understand the finesse of anatomy and should not give feel that a restrictive VSD in Tricuspid atresia II is type b. We had earlier seen a report from outside in which the baby clinically had PAH, but the echo report said Tricuspid atresia IIb. The present baby we saw had a frank narrowed pulmonary valve with sever PS. We recalled the opportunity we had of learning from others mistake! We understood the importance of measuring the size of VSD in such cases and comparing it with aortic annulus. On cath study, this boy had a mean PA pressure of 16mmHg, which was not very much OK for BD Glenn. How about creating a Glenn and doing a PA band at the same time? What is the future of such a manoeuvre? Is there any experience of doing this combo procedure? We could also recall a baby with Tricuspid atresia IC, who had undergone a PA band at age of 4 months and at the age of 2 years, had a PVWP of 17mmHg. Do the post PA bands behave any different from type B physiology in Tricuspid atresia? Does the initial period of high flow change the pressure dynamics in post Glenn any way? Any role of creating a Glenn with PA band tightening here? Please send me your takes on these.
TO BTT OR NOT TO BTT?!
What are the indicators of RV failure in cTGA, VSD, PS physiology? Can the progressively increasing TR be an indicator? We had a 3-year-old baby with cTGA, VSD and PS with more than mild TR. Since the age of the baby is less than the institute acceptance for RV to PA homograft and the baby was symptomatic, it was the choice between BTT shunt Vs an early Senning + Rastelli. How would each of them go about? The chance of getting an optimal sized homograft and the possibilities of repeat procedure were the caveats for Senning + Rastelli. Compared to this, the BTT shunt would increase the RV volume load in a cTGA. Is it OK to load the fragile RV for another year or so? What would be the impact on eventual RV function? Would a failed RV permit us to proceed with any procedure in future? Is there any way of making a vis-to-vis comparison? Any data on this? Please let me know.
CROSSING ARTERIES IN VA DISCORDANCE?
Can cTGA have the great arteries crossing each other? We had a 2-year-old with situs ambiguous, common atrium with ill-defined pulmonary venous connection with interrupted IVC. Now that the atrial morphology could not be defined, the upper floor anatomy was unclear. However, on the echo, the ventricles appeared to have d loop with the VA concordance. One of our team members pointed towards the dilemma of labelling ventricular cavities in AVCD. Hence, if the loop was presumed to be L, then it would be AV concordance with VA discordance! Now, can a discordant VA connection have crossing great arteries? Although I maintained that it is not possible, I was not very sure of my answer, more so in a situation with situs ambiguous! If you have any data on this, please post it.
CARDIAC OR SUPRA?
What is the definition of cardiac TAPVC? The most common answer would be the pulmonary venous confluence draining into coronary sinus or rarely, to directly at SVC – RA junction. We had an infant who presented with features of TAPVC. On echo, we found the pulmonary veins forming a common confluence and going up via the left ascending vertical vein, which entered the persistent Left SVC. There was no bridging innominate vein in this boy. The LSVC came back to heart and opened into the RA via coronary sinus! Technically, it is common confluence opening into CS. Is the variant cardiac or supracardiac? The question is more etymological, as the surgical team had no reservations on surgery, whatever name we called it! But, as a matter of interest, we debated the name. I felt that the cardiac variant should always be in physical link with the surface of heart throughout its entire course and any deviation from it would not be a cardiac variety, more so if it comes with a vertical vein. One of our team members tried to improve my words by saying that the cardiac variant should always be intrapericardial. We would like to get an opinion from the readership. Does the definition involve only the destination or the journey too? Please write your take on it.
MIXED DUAL
Coming back to TAPVC, we often come across mixed TAPVC. Less common but seen variants are the dual drainage, which involves the different pulmonary vein combinations entering RA via separate entry sites. Technically, dual drainage is different from mixed TAPVC. The question is, should they be considered as a separate entity? The traditional Darling’s classification of TAPVC does not mention dual drainage at all. What is the opinion from the surgical clan? Should the naming follow only when the management differs or should it be adherent to the embryological basis? Definitely, the embryological basis of dual drainage is different from that of other variants. What is your take on it? Pen your opinions.
Let me know your opinions on the issues raised or any issues you want to rise! The whole objective of the Blog is to encourage the exchange of opinions on the issues that may not have a straight forward answer. The opinions from your side would be very valuable to lot many for ages. Don’t hesitate to post questions if any genuine problem is bothering you. Let the enlightened readership be your guide to a “Yet to be opened door”. You can either use the comments section or use my email drkiranvs@gmail.com for posting your views and questions. Best of luck.
Regards
Kiran
The 1,4-dihydropyridines have an important role in the human biochemistry. Despite the role it played and the ease with which it can be evaluated, very surprisingly, minimal research had taken place involving this vital compound. The research scientists group of Bayer laboratories learnt this and started working on it. True to their research reputation, they did not leave any stone unturned. Once they had a lead, they tested for its organ efficiency and found a reasonable change in the cardiac output. They focused their energies on the molecular variant and ended up developing and testing more than 2000 analogues! The result was a patent application for a new drug called Nifedipine in the year 1967 by two scientists named Friedrich Bossert and Wulf Vater. They registered the drug for its supposed activity as coronary vasodilator.
With that started the hunt for the actual action of Nifedipine. Over a couple of years, they found that nifedipine acted by blocking calcium channels in the cardiac conduction tissue and in the smooch muscles of blood vessels. Thereby, Nifedipine led to the relaxation of the myocardial tissue and blood vessels. The resultant action was coronary vasodilatation and drop in both systemic and pulmonary vascular resistance. As an obvious result, this should have reduced the afterload and oxygen consumption. Thus, it was a drug with potential benefit in angina with hypertension. With this background, Nifedipine had a grand entry into the markets in 1975.
The major hitch of Nifedipine was its short duration of action. As the popularity of the drug picked up, this issue was to be sorted out. The research groups started the action in two sides.
The first was to develop analogues of nifedipine for longer duration of action. This would have the advantage of a sustained action on blood pressure. The result was the development of felodipine and amlodipine. In the same bargain, some more drugs were developed, which showed a higher specificity for Calcium channels of other organ systems. Nimodipine was one such development with effect on cerebral blood vessels. The advantage was its specificity, in which it did not affect the systemic blood pressure. However, all this new developments would take high levels of research and finances, not to mention the time.
The second approach was more logical and much simpler: creation of a sustained release preparation of Nifedipine. This happened quite fast and was marketed easily. With the popularity Nifedipine enjoyed, it was not difficult to make the presence for the sustained release preparation. However, the research team was aware of the short life of this strategy and had realised the effacement of molecule once the new preparations took over. But the gamble had paid off. The SR preparation effectively bridged the gap and made the constant market for the drug till the newer generation ones took over. Even today, the dihydropyridine analogues enjoy a great market presence in the field.
Compared to the systematic research of Nifedipine, the other calcium channel blocker, Verapamil was more of serendipitous in its invention. It was supposedly a takeoff from the atropine in the field of antispasmodics. In this group came mebeverine, which enjoyed some success. However, the other molecule called verapamil did not have such luck. It was synthesised by the famous Knoll pharma. Its chief of research, Ferdinand Dengel has been credited for its creation and discovery of possible uses. Once the initial trials were over, Dengel realised that the drug was nowhere near the antispasmodic action. What caught his attention was its potential action as coronary dilator. He led the research to take over towards the use in angina. The success of the trials led to the introduction of the drug in the market in 1968. Soon it was realised that the drug can cause depression of conduction activity. This action prompted Albrecht Fleckenstein at the University of Freiburg to evaluate the molecule on those lines. That explained why the drug failed as an agent for smooth muscle relaxation; it was more specific for cardiac muscles! The much more interesting finding of the research was the reversibility of its action by calcium. This led Fleckenstein to assume that the drug was indeed a calcium blocker. Further research showed that the assumption was correct and Verapamil blocked the movement of calcium ions into cardiac cells. It was credited as the first calcium antagonists. As better understanding of the cardiac physiology and the role of calcium channels became apparent, the anti-arrhythmic action of Verapamil was acknowledged in 1970s. Its action as the relaxation agent also became useful in controlling the hypertension, albeit the use is minimal for this purpose at present.
The other calcium channel blocker, Diltiazem has much more serendipitous history! It was supposed to be sought as a psychotropic drug by the Japanese. The Tanabe Seiyaku Company of Japan was working in this regard. After huge number of possible variations in the combination of the medicational research, what they ended up with was Diltiazem! The initial drug was a racemic mixture of many isomers of the drug. The research team ended up segregating each isomer and started testing them. As a part of serendipity, the dextro isomer showed great vasodilator effect. The team decided to conduct the trial for this molecule. It was finally shown to have vasodilatory action simulating papaverine on coronary circulation. Logically, they tested it against calcium ion movement across cardiac muscle membrane. It was found to be a positive blocker! Thus was born the first coronary vasodilator which was actually a calcium antagonist. Later, as a comparison with the other drugs of the group, it was tested for its action on cardiac rhythm. It was positive for that action also! Theoretically, it stood between the Nifedipine and Verapamil, with good qualities of both in the same drug. Its spectrum makes it a desired preparation even today for selective indications.
Next issue may be the last one for the history of drug development. If anyone has any ideas for future posts, please let me know.
On a personal note, we had our departmental “publication meet” recently. As usual, many a write ups are in the long process of preparation. Our mercurial boss got impatient about the delay of her lethargic team. I was advised to give up blogging and to get serious with research. I think it is a good advice for now, as the number of people reading my blog does not make any justice to my effort. I can as well concentrate on writing papers and getting credit for publication! If you find the frequency of the blog posts getting reduced in the future, you know whom to blame!!
ATRETIC TRILEMMA!
How does a restrictive VSD in Tricuspid atresia type II behave? If anyone says as IIb, they are obviously wrong; it becomes subaortic obstruction! It is clearly unlike that of type I. But a beginner needs to understand the finesse of anatomy and should not give feel that a restrictive VSD in Tricuspid atresia II is type b. We had earlier seen a report from outside in which the baby clinically had PAH, but the echo report said Tricuspid atresia IIb. The present baby we saw had a frank narrowed pulmonary valve with sever PS. We recalled the opportunity we had of learning from others mistake! We understood the importance of measuring the size of VSD in such cases and comparing it with aortic annulus. On cath study, this boy had a mean PA pressure of 16mmHg, which was not very much OK for BD Glenn. How about creating a Glenn and doing a PA band at the same time? What is the future of such a manoeuvre? Is there any experience of doing this combo procedure? We could also recall a baby with Tricuspid atresia IC, who had undergone a PA band at age of 4 months and at the age of 2 years, had a PVWP of 17mmHg. Do the post PA bands behave any different from type B physiology in Tricuspid atresia? Does the initial period of high flow change the pressure dynamics in post Glenn any way? Any role of creating a Glenn with PA band tightening here? Please send me your takes on these.
TO BTT OR NOT TO BTT?!
What are the indicators of RV failure in cTGA, VSD, PS physiology? Can the progressively increasing TR be an indicator? We had a 3-year-old baby with cTGA, VSD and PS with more than mild TR. Since the age of the baby is less than the institute acceptance for RV to PA homograft and the baby was symptomatic, it was the choice between BTT shunt Vs an early Senning + Rastelli. How would each of them go about? The chance of getting an optimal sized homograft and the possibilities of repeat procedure were the caveats for Senning + Rastelli. Compared to this, the BTT shunt would increase the RV volume load in a cTGA. Is it OK to load the fragile RV for another year or so? What would be the impact on eventual RV function? Would a failed RV permit us to proceed with any procedure in future? Is there any way of making a vis-to-vis comparison? Any data on this? Please let me know.
CROSSING ARTERIES IN VA DISCORDANCE?
Can cTGA have the great arteries crossing each other? We had a 2-year-old with situs ambiguous, common atrium with ill-defined pulmonary venous connection with interrupted IVC. Now that the atrial morphology could not be defined, the upper floor anatomy was unclear. However, on the echo, the ventricles appeared to have d loop with the VA concordance. One of our team members pointed towards the dilemma of labelling ventricular cavities in AVCD. Hence, if the loop was presumed to be L, then it would be AV concordance with VA discordance! Now, can a discordant VA connection have crossing great arteries? Although I maintained that it is not possible, I was not very sure of my answer, more so in a situation with situs ambiguous! If you have any data on this, please post it.
CARDIAC OR SUPRA?
What is the definition of cardiac TAPVC? The most common answer would be the pulmonary venous confluence draining into coronary sinus or rarely, to directly at SVC – RA junction. We had an infant who presented with features of TAPVC. On echo, we found the pulmonary veins forming a common confluence and going up via the left ascending vertical vein, which entered the persistent Left SVC. There was no bridging innominate vein in this boy. The LSVC came back to heart and opened into the RA via coronary sinus! Technically, it is common confluence opening into CS. Is the variant cardiac or supracardiac? The question is more etymological, as the surgical team had no reservations on surgery, whatever name we called it! But, as a matter of interest, we debated the name. I felt that the cardiac variant should always be in physical link with the surface of heart throughout its entire course and any deviation from it would not be a cardiac variety, more so if it comes with a vertical vein. One of our team members tried to improve my words by saying that the cardiac variant should always be intrapericardial. We would like to get an opinion from the readership. Does the definition involve only the destination or the journey too? Please write your take on it.
MIXED DUAL
Coming back to TAPVC, we often come across mixed TAPVC. Less common but seen variants are the dual drainage, which involves the different pulmonary vein combinations entering RA via separate entry sites. Technically, dual drainage is different from mixed TAPVC. The question is, should they be considered as a separate entity? The traditional Darling’s classification of TAPVC does not mention dual drainage at all. What is the opinion from the surgical clan? Should the naming follow only when the management differs or should it be adherent to the embryological basis? Definitely, the embryological basis of dual drainage is different from that of other variants. What is your take on it? Pen your opinions.
Let me know your opinions on the issues raised or any issues you want to rise! The whole objective of the Blog is to encourage the exchange of opinions on the issues that may not have a straight forward answer. The opinions from your side would be very valuable to lot many for ages. Don’t hesitate to post questions if any genuine problem is bothering you. Let the enlightened readership be your guide to a “Yet to be opened door”. You can either use the comments section or use my email drkiranvs@gmail.com for posting your views and questions. Best of luck.
Regards
Kiran
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