Tachyarrhythmia in patients with congenital heart disease: inevitable destiny?

Abstract

The prevalence of patients with congenital heart disease (CHD) has increased over the last century. As a result, the number of CHD patients presenting with late, postoperative tachyarrhythmias has increased as well. The aim of this review is to discuss the present knowledge on the mechanisms underlying both atrial and ventricular tachyarrhythmia in patients with CHD and the advantages and disadvantages of the currently available invasive treatment modalities.

Keywords: Atrial tachyarrhythmia; Cardiac surgery; Catheter ablation; Congenital heart defects; Ventricular tachycardia.

Fig. 1

Anatomy of the cavotricuspid isthmus. Postmortem human heart with a superolateral view of the right atrium (turned inside out) with a bicaval incision. The cavotricuspid isthmus, which is regarded as the zone of slow conduction, is encircled. The isthmus is bordered anteriorly by the TA and posteriorly by the orifice of the ICV. CS coronary sinus, CTI cavotricuspid isthmus, ICV inferior vena cava, TA tricuspid annulus

Fig. 2

ECG characteristics of regular atrial tachycardias. Left panel: typical atrial flutter consisting of flutter waves with 1) flat descending part, 2) steep descending transition, 3) sharp upstroke and 4) a minor overshoot. Right panel: intra-atrial reentrant tachycardia; the four characteristics of the typical flutter waves are missing

Fig. 3

Anatomy of heart with CHD. Postmortem human heart of a 4-year-old child with a large atrial septal defect. The heart is shown from a lateral view through a right atriotomy incision into the right atrium. In adult patients with congenital heart defects, the intra atrial reentry tachycardia is frequently observed around the right atriotomy scar (red marked area), but also around the atrial septal defect (yellow marked area). ASD atrial septal defect, CS coronary sinus, ICV inferior caval vein, RAA right atrial appendage, RV right ventricle, SCV superior caval vein

Fig. 4

Electroanatomical mapping of IART. Three-dimensional electroanatomical mapping of the right atrium in a 15-year-old patient, 12 years after completion of the Fontan correction, who was referred for ablative therapy of an incessant atrial tachycardia. The colour-coded right atrial activation map shows a figure-of-eight reentry around 2 areas of scar tissue. The tachycardia was eliminated by constructing a linear lesion between 2 areas of scar tissue

Fig. 5

Electroanatomical mapping of focal atrial tachycardia. A 17-year -old patient with patent foramen ovale presented with paroxysmal episodes of regular atrial tachycardia. During an invasive electrophysiological study with 3D activation mapping, the atrial tachycardia (cycle length 348 ms) had a focal origin at the left atrial free wall. The map shows expansion from one circumscribed area in the anterior-posterior view (AP) to the remainder of the atrium in the posterior-anterior view (PA). After construction of a circular lesion around the earliest activated area, the tachycardia terminated