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

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