Pulmonary valve replacement (PVR) is increasingly used to treat the chronic volume overload from pulmonary regurgitation in tetralogy of fallot patients. The procedure can be performed by a trans catheter technique or surgically, using one of the many available bio-prosthetic valves. However, despite numerous investigations on timing, indications, techniques and results of PVR, large gaps in knowledge persist on how best to manage these patients.

A 22-year-old boy from hanumangarh, Rajasthan, diagnosed as tetralogy of fallot at the age of 18 months and operated at all India institute of medical sciences for the same. Repair was successful, he was discharged after 10 days of hospital stays. He was asymptomatic and doing well till 6 months ago when he developed palpitation (increased heart rate) and uneasiness. He was evaluated by private doctor and diagnosed to have supraventricular tachycardia with severe pulmonary valve regurgitation and advised for automatic implantable cardioverter-defibrillator device(AICD).
During last six months he had three episode of supraventricular tachycardia and use of direct current (D.C.) cardioversion in last episode. This patient came to Rukmani Birla hospital last month during when he was on high dose of antiarrhythmic drugs. He was evaluated at RBH. Echocardiogram was suggestive of severe leak in pulmonary valve and it was causing right ventricular volume overload, moderate pulmonary artery hypertension and right ventricular dysfunction. ECG showed broad QRS complex. Case was discussed between CTVS and cardiology team and plan of pulmonary valve replacement was made. NCCT chest was done to check the adhesion between heart and sternum and it was suggestive of dense adhesion between sternum and heart and high risk of cardiac injury during sternotomy.
Challenges in this case was;

  • It was a redo case where anatomy was distorted and dense adhesion were present. (*we prepared the femoral vessels for emergency cardiopulmonary bypass prior to sternotomy, if any cardiac injury occurs during sternotomy)
  • Position of pulmonary valve is very important in pulmonary artery otherwise it will have compressed by sternum during closure.
  • Management of pulmonary artery hypertension and right ventricular dysfunction.

We operated this boy on 23nd September and pulmonary valve replacement done with all precaution (proper positioning of valve and management of pulmonary artery hypertension and right ventricular dysfunction in post-operative period). For pulmonary valve replacement bio prosthetic valve was used and to reconstruct pulmonary artery, bovine pericardium was used. Surgery was uneventful and patient was extubated on the same day. He was kept on blood thinner (oral anticoagulant) {for first three months}to avoid clot formation and discharged on day 10. Now he is doing well in follow up without any need of high dose of antiarrhythmic drugs.

In cases of post TOF repair with arrhythmias first we have to rule out surgical cause of arrhythmia, in our patient he had severe pulmonary valve regurgitation and right ventricular volume overload with recurrent episode of arrhythmia, so we planned for pulmonary valve replacement in this patient. AICD is not the first line treatment. With timely intervention we can avoid early development of right ventricular dysfunction and failure in these patient.Automatic implantable cardioverter-defibrillator (AICD) is a device designed to monitor the. heartbeat. This device can deliver an electrical impulse or shock to the heart when it senses a life- threatening change in the heart’s rhythm.