**19. Arryhtmias, pacemakers and defibrillators**

Adults do occasionally present with PDA- the large ones are rarely still operable and the small ones can be occluded by the interventional cardiologist. Surgery in PDA with pulmonary hypertension is high-risk as they are fragile and prone to catastrophic tears during surgery-

Coarctation is not uncommon in presentation as an adult, either as a primary coarctation or re-coarctation. Surgery is high-risk, particularly with tight coarctation with multiple collater‐ als. Entry into the chest is associated with significant bleeding and very often, the coarct segment has to be excised and replaced with an interposition graft. The aortic tissue is usually friable and is prone to tear. A bypass graft from the left subclavian artery to the descending aorta may then be preferable. Recoarctation is better dealt with balloon plasty and stenting.

Tetralogy of Fallot may present in adulthood. They are amenable to corrective surgery provided criteria are met such as adequacy of pulmonary arteries and there are no significant collaterals. They may need preoperative embolization of collaterals and intraoperative RV-PA conduit as they may not tolerate free pulmonary regurgitation in the event of transannular patch. The children who underwent repair as children may present in adulthood with RV dysfunction due to free PR. They require redo sternotomy and insertion of RV-PA conduit. A subset of these patients presents as adults with aortic regurgitation and/or ascending aortic

Patients may present de novo with RVOT obstruction at various levels. These are amenable to

Children who underwent Senning's or Mustard's procedure from transposition of great arteries (TGA) present in adulthood with residual defects such as baffle leaks or with cardiac failure as the RV in the systemic position is prone to failure. These patients require heart transplant or conversion to arterial switch following PA banding to retrain the LV. These are

Children with single ventricle physiology present in adulthood requiring Fontan procedure. Some have had atriopulmonary Fontan and require conversion to an extracardiac Fontan if

Ebstein's anomaly, cor triatriatum sinister (membrane in the left atrium obstructing pulmonary veins), ALCAPA and TAPVC with unrestricted ASD are conditions with which patients may present as adults for surgery. Congenital corrected transposition of great arteries, with VSD

Children who have undergone arterial switch as infants present as adults with coronary issues, neo-pulmonarysupravalvular stenosis, and neoaortic regurgitation. These patients require stenting of their coronary artery obstructions, coronary artery bypass grafting, patch plasty of

supravalvular obstruction and replacement of the aortic valve.

they may be calcified. Surgery is more complex- requiring cardiopulmonary bypass.

Adults with coarctation may be managed with covered stents.

264 Principles and Practice of Cardiothoracic Surgery

surgery or intervention based on various criteria.

high-risk procedures.

the former surgery fails.

and PS may also present late.

aneurysm and require AVR with or without ascending aorta replacement.

The congenital population is prone to postoperative complete heart block and even nodal arrhythmias are not tolerated by the population with single ventricle physiology. These children will require to be under the care of the electrophysiologist. They will need lifelong pacemaker changes and lead changes. Arrhythmias are common such as atrial fibrillation and those related to accessory bundle pathways. Children with dilated RV/LV are prone to malignant ventricular arrhythmias such as ventricular tachycardia and ventricular fibrillation. These require monitoring and insertion of AICD (automatic internal cardioverter-defibrilla‐ tor). Patients with chronic atrial fibrillation may benefit from the Maze procedure (antiar‐ rhythmic surgery). This procedure may involve either right atrium or left atrium or both.
