Sudden cardiac death in congenital heart disease-a narrative review and update

Abstract

The spectrum of congenital heart disease is extremely varied, from simple septal defects all the way up to complex heterotaxy with multiple overlapping congenitally malformed regions of the heart. While surgical repair has come a long way since the first congenital cardiac surgery, a B-T-T shunt, an unmet need remains as the population continues to experience sudden cardiac arrest at a greater rate than the general population. Many advances in pacing and cardioversion have occurred to address bradyarrhythmias and tachyarrhythmias, but these carry their own adverse effect profile and limitations. This review aims to survey the field, summarize advances, and provide suggestions for future research directions.

Keywords: Tetralogy of Fallot; adult congenital heart disease; grown up congenital heart disease; pediatric cardiology; sudden cardiac arrest.

Figure 1

The normal cardiac conduction system. Reproduced with permission from “Cardiac conduction system” by Npatchett, licensed under CC BY-SA 4.0.

Figure 2

The Fontan procedure (in this case illustrated as a palliation of hypoplastic left heart syndrome). The additional surgeries required before the Fontan are also depicted. Panel A illustrates a structurally normal heart. Panel B illustrates the hypoplastic left heart. Panel C and D illustrate the two potential surgeries that can be used as the first stage palliation of HLHS. Panel E illustrate the second stage palliation. Panel F illustrates the Fontan surgery, or total cavopulmonary anastomosis. Reproduced with permission from “Surgical reconstruction of HLHS hearts” by Birla et al., licensed under CC BY 4.0.

Figure 3

The approximate position of the dual AV nodes and conduction system of L-TGA from the point of view of the atrioventricular valves, with normal atrial situs.