Pediatric Cardiac Services

Achievements

Since February 2012 to March 2020 we have performed around 3048 cases of cardiac surgical operations in children with heart disease. The range of operations included all varieties of complex cardiac surgery in neonates and infants. Among them, 777 infants were weighing below 5 kg and 409 neonates were weighing below 3 kg.

The following are the recent data of the various surgeries performed in our unit, classified in appropriate subheadings :

1. Common & Routine Surgeries :

Atrial septal defect (ASD) 258 cases, Ventricular septal defect (VSD) 801 cases, Multiple ventricular septal defects (Multiple VSD) 88cases, Tetralogy of Fallot (TOF) 588 cases, Coarctation of aorta repair (CoA) 81 cases,  Aorto-pulmonary window (AP window)  14 cases. The smallest weight baby operated in the TOF category is 4.5 kg; in the VSD category is 2.0 kg and in the CoA repair category is 1.4 kg. 

2. Major & Complex Cardiac Surgical Procedures :

Total anomalous pulmonary venous connection (TAPVC) 216 cases, Arterial switch operation for transposition of great arteries (TGA) 138 cases, combined repair of the aortic arch and ventricular septal defect 52 cases, Anomalous Left Coronary Artery from Pulmonary Artery (ALCAPA) 18 cases,  Complete AV canal defect (CAVCD) 38 cases,  Mitral valve repair (MV repair) 52 cases, Truncus Arteriosus repair with RV to PA conduit 9 cases,  Fontan repair (final stage of single ventricle palliation- TCPC) 24 cases,   Senning operation 9 cases,  Double switch operation for c-TGA 14 cases, Unifocalization for TOF with Pulmonary atresia and MAPCAS 18 cases. The smallest weight baby operated in the complex surgery category like TGA/TAPVC is 2.0 kg. 

Our experience with arch repair in neonates and infants :

Our unique achievement is successful outcomes in arch repair operations in neonates and infants with the Stanford University technique of “Innominate artery cannulation using selective cerebral perfusion” (52 cases). In this technique, we are able to perform the repair of the aortic arch without stopping blood flow to the brain (circulatory arrest). This data was presented as a poster in the Asian Cardiovascular Surgeons Association meeting (ASCVTS) Hongkong in May 2015 and was awarded the best poster award in the conference.  Also, the same data was published in the “Indian Journal of Thoracic and Cardiovascular Surgery” (IJTCVS 2015; vol 31, No. 2) titled as “Single-stage repair of the aortic arch and associated cardiac defects with antegrade cerebral perfusion using direct innominate artery cannulation in neonates and infants”.

Our Innovation in the repair of tetralogy of Fallot (TOF) in children :

A) Valve and annulus sparing repair in Tetralogy of Fallot (TOF) :

We have pioneered a new innovation in TOF (Tetralogy of Fallot) repair of preserving the native pulmonary valve of the baby/kid and avoiding cutting across the pulmonary artery annulus. By doing this we are able to prevent leaking of the pulmonary valve. This will preserve the right ventricle function in a better way in the long term. This approach of the repair was aggressively pursued in 305 cases between March 2013 to March 2020.  In the recent past, we have improvised the surgical technique of valve-sparing by using a new concept of delamination of the valve. This has shown very good outcomes in the patients. The entire spectrum of cases of valve-sparing repair of TOF has been analyzed by us recently. The outcomes have been excellent and we are in the process of publishing this experience in a reputed journal.

B) TOF repair using core matrix patch for RVOT reconstruction : 

During TOF repair, if the pulmonary artery annulus is cut across we have pioneered a technique of creating a new pulmonary valve using a material of biological scaffold called the core matrix patch. This valve will prevent pulmonary valve leaking after surgery. The early outcomes of this group of patients have been encouraging. We have done this repair in about 66 patients.  This work has been published in the prestigious international journal called the “World Journal of Paediatric and Congenital Heart Surgery” (WJPCHS) titled as “ Preliminary experience with the use of an extracellular matrix to augment the native pulmonary valve during repair of tetralogy of Fallot” in the March 2017 issue of the Journal.  This data was also presented as an oral presentation by Dr Anil Kumar Dharmapuram in the international conference of the World Society of Paediatric and Congenital Heart Surgery (WSPCHS) in Abu Dhabi during October 2016. 

C) TOF  repair using PTFE valve for RVOT reconstruction :

In recent years, we have innovated another superior technique of RVOT reconstruction in TOF repair if the patient’s pulmonary valve is small and abnormal.  We have been creating a new valve in the right ventricular outflow tract using a synthetic thin membrane called a 0.1 mm polytetrafluoroethylene (PTFE) membrane. We have used this technique in 73 patients till now who required a trans-annular patch in TOF repair. The results of this technique have been excellent and the valve function looks very good during follow up of these patients. Also, in cases of TOF with absent pulmonary valve syndrome we have been doing the reconstruction of the RVOT using a two leaflet valve of the same material. The entire spectrum of cases of TOF repair with the 0.1 mm PTFE membrane is being analyzed for publication in a reputed journal.

Our experience in repairing multiple holes in the hearts of small babies :

Many children including infants have multiple holes in the heart called multiple VSDs. Between March 2012 to March 2020 we did a primary repair of multiple VSD in 88 children including infants weighing as small as 2.7 kg. We have pioneered a new and internationally popular technique of repairing these multiple holes called “endothelial suturing of the multiple holes”. This technique preserves the heart function better than other techniques in small babies. The outcomes of this technique have been excellent with minimal mortality.

The comprehensive surgical approach adopted to repair mitral valve disease in children including infants :

Repair of congenital mitral valve disease is a challenge to a surgeon. A combination of techniques is required to minimize the stenosis and regurgitation and avoid valve replacement which is a very difficult option in this subset. 

Between June 2013 to March 2020, 52 children including many infants underwent repair for mitral valve disease. The age ranged from 2 months to 12 years and the weight as small as 3.7 kg. Some of them had a stenotic lesion (obstructive/narrow pathway) while many of them had a regurgitant lesion (leaking valve with enlargement of the annulus of the valve). We have a pioneered a new technique of repairing the mitral valve in these small children including infants using artificial chordae with a synthetic material called Goretex chordae. This is used as a substitute to the support mechanism of the valve leaflet that is insufficient and causes the valve leak. Using this material to repair the mitral valve in small infants is really challenging and only a few centres in the world have a good experience. 

We are very proud of our outcomes with this technique of repair using the artificial Goretex chordae. We have used this technique in many patients among the number mentioned above. All of them have done well and have reduced or minimal leak of the valve during follow up in our outpatient department.  

Our experience with Unifocalization during repair of TOF with pulmonary atresia with MAPCAS : 

In our unit, 18  patients underwent a very major surgery called Unifocalization in the spectrum of patients of TOF with pulmonary atresia and MAPCAS. This is a very difficult and complex spectrum of disease wherein there is no blood going from the heart to the lungs in view of pulmonary atresia (absence of pulmonary valve and very small pulmonary artery). The blood supply to the lungs is from abnormal vessels called MAPCAS (major aortopulmonary collaterals) which arise from the aorta. To achieve complete correction and normalcy of circulation to the lungs, the MAPCAS have to be disconnected from the aorta and connected to the right ventricle of the heart (Unifocalization surgery). This is usually done in stages and after the first stage of surgery which is the Unifocalization, the intracardiac repair and repair of the VSD in the heart is done later. However, complete correction requires a valved tube from the right ventricle to the reconstructive pulmonary artery. 

Across the world, Unifocalization type of complex cardiac surgery is not done routinely in many centres. Stanford University, California, USA is the world leader in this particular operation. Our unit in KIMS Hospitals has the expertise and technology to perform this operation. This technology has been transferred significantly from Stanford University, California, the USA where Dr Anil Kumar Dharmapuram has been trained. 

Our experience with Double Switch Operations for corrected transposition of great arteries (cc TGA) : 

Corrected transposition of great arteries (CCTGA) is a complex congenital heart problem wherein the pathway of the blood coming into the heart and then going out of the heart is in the reverse position. In other words, the incoming and outgoing pathways are both reversed. Many times, this is associated with a hole in the heart (VSD). To achieve complete correction, we have to switch both the pathways and this is a very major and a surgically challenging operation called double switch operation (DSO). Across the world, this operation is not done routinely in all the centres and many centres do not have a good outcome. We have done  14 cases till now in our unit. We are very proud and happy to say that our outcomes have been good in this operation with minimal mortality. 

Our experience and innovation with a new technique called Pulmonary Root Translocation for d-TGA with VSD and LVOT obstruction :

d-Transposition of great arteries (d-TGA) with VSD and obstruction to the outflow of the major chamber of the heart (LVOT) is a very difficult surgical challenge. Across the world, the standard operation for this is called Rastelli Operation. Unfortunately, in this operation, a conduit from the right ventricle to the pulmonary artery (RV to PA) is required. This has to be changed when the baby grows and this is not a good option in the long term.  A new operation for this problem called Pulmonary Root Translocation (PRT) has been reported from the Brazilian group of surgeons.  The advantage of this operation is that the baby’s own pulmonary valve which is smallish is removed from the LV and moved to the RV ( the two major lower chambers) and hence a conduit is avoided. We realized that in a country like us this is very important because of the difficulty in procuring these conduits and the enormous expenditure involved. For this reason, we have started doing this procedure in the last few years and did 16 cases till date with satisfactory outcomes. Only a few centres in the world have the expertise to do this operation and in our country, we have pioneered this technique with a very aggressive approach. We are very proud of the outcomes of this PRT operation in our unit.   

Our experience with repair of coarctation of aorta with arch hypoplasia in neonates and infants :

In this lesson, the major tube called the aorta which comes out from the left ventricle becomes narrow in the distal portion of the arch and this condition is called coarctation of the aorta (CoA). This is associated with hypoplasia of the arch. In these babies, if there is no VSD in the heart, the repair can be done from the side (thoracotomy) without the use of cardiopulmonary bypass (CPB). We have pioneered a modification of this technique of repair called “end-to-side anastomosis” and successfully published our excellent outcomes in the Annals of Pediatric Cardiology journal during September/October 2018  titled as “Early outcomes of modification of end to side repair of coarctation of aorta with arch hypoplasia in neonates and infants”. We have done this technique in babies as small as 1.4 kg with a good outcome. 

   

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Dr. Sudeep Verma

Pediatric Cardiac Services

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Master Ali Monamed Ahmad

Pediatric Cardiac Services

Master Enitan Modupe Famoti

Pediatric Cardiac Services

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Pediatric Cardiac Services

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