Dr Kiran welcomes you to the present post of blog.

As usual, I did not receive any comments for the last post. But I have heard that many people do read and follow the blog. Just that they are as lazy as I am when it comes to writing!

Without much ado, let us carry on with the interesting learning scenarios:

SMOOTH FLOW PALLIATION?

We had a 6-year-old with congenitally corrected transposition of great arteries, with small VSD (non-routable) and severe subvalvar and valvar PS. The LV was suprasystemic and TR was moderate. Neither double switch nor Senning-Rastelli were possible. The surgeons were not happy with the tricuspid valve and ruled that the conventional repair (RA to LV to PA and LA to RV to Ao) by pulmonary valve repair was inadequate unless the TV was also intervened. However, their scepticism was, “what if the TV is not repairable?” The child was too young for TV replacement. We argued that the pulmonic valve repair alone should bring down the LV pressures and improve the LV geometry. This would take away the Bernheim-reverse Bernheim effect and would improve the RV geometry in return, thereby reducing the TR. I felt this intervention would be better than leaving a suprasystemic chamber for follow up. But, this logic did not please the surgical team and they ruled out any such improvement. Although it sounds theoretical, I could see practical point in it. How would the readers like to decide? Please let me know the folly in this logic!

INSIDE THE HEART OF FONTAN

Once a single ventricle goes into the Fontan correction, we usually feel that the woes for patients lie outside the heart. We had a 12-year-old with Fontan completion done 5 years back. This child came with a new cardiac murmur, not documented in the previous records. On echo, we found a significant subaortic obstruction! I had not come across this earlier. This actually poses a new issue for the patient. Since the obstruction was moderate, the surgical team suggested close follow up. If this becomes severe, what would be the plan? Is going inside the Fontanised heart simple? How frequent are such complaints? Our experience with Fontan is not huge. Can anyone with more experience help us in this?

MONOCUSP

We had some heated debates and discussions on the utility of monocusp valve in intracardiac repairs of tetralogy? Few argued for long-term benefits and few for short-term. Surgeons discussed the risk-benefit ratio of monocusp valve. However, at the end of all these, no one was ready to change their views and opinions. That left most of the simple audience with more confusion and no conclusion. Can the readers put up their opinions in this issue and get us better understanding?

FONTAN WITH HEART BLOCK

Sinus rhythm is one of the Choussat’s criteria for Fontan completion. We had a 9-year-old boy with BD Glenn shunt done outside 5 years back. He had NYHA class III symptoms. During OPD evaluation, he had an ECG which documented sinus rhythm. He was catheterised and the pressures were OK for Fontan completion. However, in the ward, we documented slow heart rate and Holter monitoring showed intermittent A-V dissociation. Surgeons opined that the Fontan completion is still possible with DDD pacemaker insertion simultaneously. This was news to us. Is it done? Is it acceptable? Please let us know about your opinion and experience.

STUBBORN PA

We had a 6-year-old who had undergone a PA banding for multiple VSDs at an outside hospital few years back. The same hospital did a PA de-banding and closed a large upper muscular VSD at the same setting about one year back. When this child came to us, we were surprised to find few sizeable VSDs and a significant gradient across the MPA segment. The operative notes at outside hospital had clearly stated PA de-banding. But, our echo could still pick up a constricted segment in the MPA prior to bifurcation. On cath, we found this narrow segment, which had cicatrised and did not re-open following de-banding. As for the residual VSDs, our expert surgical team was confident that all of them can be dealt with. Hence, we decided for a resection of cicatrised MPA segment and closure of VSDs. How common is this cicatrisation at PA band level? Although we perform good number of PA bands, we at NH had never witnessed this complication. Are such lesions innate to few patients or is it widely seen? Please let us know your experiences on this.

It is time to congratulate Dr Shwetha Nathani, our fellow for the last two years for having successfully completing the Fellowship FNB exam in Pediatric Cardiology. All the good luck for her bright future.
It is time to mourn another departure. Our beloved echo technician, Mrs. Harini is moving to Chennai city for good. We all miss her expertise and keen acumen. Good luck wherever you go.

Pen in your criticisms at comments box or to drkiranvs@gmail.com Shall get back shortly.

Regards

Kiran

Oops!
Five months that I have updated anything in the blog!!
Sincere apologies.
I know lot of readers have stopped checking the blog. I know I have been unfaithful!!
There were times of busy deadlines, extracurricular works, depressions, blocks, fights, heart-breaks and so on. But none of them can explain the actual reason for the absence of updates: It is pure laziness on my part.
Also, lack of responses from the readers was negative impetus.
One of my teachers suggested me: do it for yourself and be happy if few more get benefitted.
But, showtime spirit is not in favour of it!!
I figured out few ways I can be more consistent. First was restructuring the blog.
I shall continue this blog with pure Pediatric Cardiology material. Shall try to make it more dense and focussed. It should serve for those into this subject or have serious interest in the field.
That actually takes away a substantial amount of readership, mainly – paediatricians. To compensate, I had to think of few ways out:

1. Proposal to start an exclusive interactive blog on Pediatric cardiology for Pediatricians. In this, we would touch common cardiac problems of children which are of much interest and frequency to paediatricians. This blog which is shaping up at present, tries to make an interactive forum with readers, inviting questions from them and giving answers, putting up practical tips on diagnosis and management. I shall give the info shortly.
2. Got a proposal to start one book-club blog! This would be pure non-academics. It aims at getting the readers of blog to write on what interesting non-academic work they have read. It can be a present reading or even a book that had a large impact on them in the past. Purely literature and anything other than politics and religion!!

Working on these ideas have got me an excuse not to write for the present blog. But, I could not avoid the temptation of keeping this blog alive. Hence, I aim to continue this with as much precise info as possible.

I have cut away the segments on articles, reviews and pearls. So, what remains in the blog is purely interesting learning discussions. I shall try to update them as and when possible.

With this prelude, let me continue to the main segment: Interesting learning scenarios:

PRESSURE AND POWER
It is often said that the left ventricle in Ebsteins anomaly gets jeopardised due to innate structural reasons and lateral pressure from atrialised RV. Can the low pressure atrialised RV exert sufficient power to deform the mightier LV? Is the phase of cardiac cycle in which the events occur responsible for such behaviour? If there is space compromise, wouldn’t the low pressure chamber suffer more than the high pressure one? What is the take of readership on this? Please let me know.

DEVICE REGURGITATION
What is the incidence of aortic valve regurgitation following VSD device closure? I would presume it is rare. The scepticism on perimembranous VSD devices are always to do with conduction blocks than aortic valve. Also, it is largely in the case selection. We are always concerned about the distance between VSD and the valves. If a perimembranous VSD device has resulted in mild+ AR, how should it be tackled? Should we just follow it as any other AR, watching for progress and intervening at the right time? Or, should we be aggressive because we have a prosthetic material impinging on the valve and deforming it? If the readers have come across such instances, please enumerate your experiences for others. Otherwise, what is the logical conclusion? We wanted to wait, but surgeons advised us to get the device out. What would your say be?

WHATS IN A NAME?
We had an interesting observation. It was a 5-year-old asymptomatic child with bilateral SVC. However, the LSVC showed flow away from the heart and RSVC was into the heart, with a proper H-communication. The pulmonary veins were normal and all of them were draining effortlessly into LA. We ruled out TAPVC, PAPVC and levo-atrio-cardianal vein. So, what explains the reversal of flow in LSVC. On careful review echo, we could trace the reversal till the H-communication (innominate vein), but the flow from the other end of LSVC was normal till the innominate vein! So, it is as if the innominate vein receives something from heart, apart from its own flow from head and neck. We traced the LSVC into the heart. It used to get blurred by the time we reached the RA. Finally we could figure out that the opening of coronary sinus into the RA was atretic! The cardiac venous flow came into coronary sinus and had a parallel vent in the form of LSVC. Thus, coronary venous flow ascended via LSVC, reached the incidental H-communication, went into the RSVC to eventually enter the RA, unobstructed! Our surgeons claimed that such lesions do exist and they have seen them earlier also. They also claimed that such a lesion has a name. We could not get this eponym. If anyone knows what this combo is called, can you please enlighten us? We did nothing for this patient, as the kid was totally symptom-free. Should we do something? Please let us know your opinion.

EDP IN DISTAL BLOCKS
How pertinent is the EDP in the presence of distal obstruction? We had a 4-year-old with moderate ASD and pulmonic stenosis. One of our consultants felt that the RV not dilated upto the expectation and advised cath study. In the cath, mean gradient across the pulmonic valve was 50mmHg, but the RV EDP was 18mmHg. I was not sure if this EDP reading should be given importance when there was a distal obstruction. Finally, the advice was to get a BPV done and re-assess the ASD. What is the yield of cath pressure data in the presence of such combination lesions? Please let us know your ideas on this.

STEALING BY FISTULA
We do come across few cases of coronary-cameral fistula in young children. We generally go by the volumes and make a decision whether it should be intervened or not based on the chamber dilatation. Do these fistulae cause coronary steal? How do we know it in younger children? Is thallium study useful when there is no wall motion abnormality on echo? I have rarely seen anyone deciding these lesions giving weight to potential steal. Please let us know your take on this.

Let us meet again with another five learning scenarios in short time. Before parting: welcome to our new NH Pediatric Cardiology fellows: Dr Arun Bableshwar, Dr Rahul Saraf, Dr Devprasath and Dr Kshitij Sheth. Hope you will have exciting learning and formative phase as pediatric cardiologists at NH. Also, good luck to Dr Shwetha Nathani – for her fellowship exit exam this month end at Escorts, New Delhi. We wish her the same success as she had for gold-medal winning case-presentation performances at various centres across India.

As usual, please put down any suggestions to drkiranvs@gmail.com or in the comments box

Regards

Kiran