Fifteen-year-old John is the fourth of six children of Dhanam and Vadivel, from Thanjavur district in Tamil Nadu. Studying in the 11th standard, a routine school health check-up revealed a murmur in his heart. The doctor referred John to Miot Hospital in Chennai for further evaluation.
At Miot Hospital, an echo was conducted which showed that John was suffering from a complex congenital heart defect; a severe form of Ebstein’s Anomaly with a large ASD; a hole in the upper chamber of the heart, wherein the Septal and Posterior leaflets of the Tricuspid Valve are displaced towards the apex of the right ventricle. Ebstein’s Anomaly occurs in less than 1% of congenital heart defects, making John’s case a rather unique and complex situation. His right ventricle was marked by atrialised and his tricuspid wall was dysplastic and displaced.
Open heart surgery was the only way to save John and his parents were counselled for surgery. Vadivel is a farmer earning Rs 4000 per month and was thus not in a position to muster money for the surgery. With help from the government and Genesis Foundation, wherein we encourage our supporters to make donation for children suffering in India from CHD, John’s operation was financially supported, and he was discharged in a stable condition. He underwent a cone repair of the tricuspid wall with plication with atrialised RV and ASD closure, and RA reduction. In three months, John will be able to resume school and fulfil his dream of becoming a chartered account in the future.
Following are the details of his surgery, corresponding to Fig 1:
A. Shows the abnormally displaced valve and dilated right atrial chamber
B. The abnormal valve is detached from its position
C. The entire apparatus is rotated clockwise so that it forms a cone (note the multiple sutures taken to join the cut edges).
D. The valve in its new shape has been reattached. Also, at the 3 o’clock position you can see how the redundant chamber wall has been bunched up to make the chamber small and more functional.