Implantable cardioverter-defibrillators in congenital heart disease

Abstract

Implantable cardioverter-defibrillators (ICD) have an important role in reducing sudden cardiac death in patients with congenital heart disease (CHD); however, the benefit of ICDs needs to be weighed up against both short-term and long-term adverse effects, which are difficult to evaluate in the heterogeneous CHD population. A tailored approach, taking into account risk stratification and patient-specific factors, is needed to select the most appropriate strategy. This review discusses primary and secondary ICD indications, implantation approaches and long-term follow-up. Recent publications have shed light on the concerns of system longevity, lead extractions, inappropriate shocks and impact on the quality of life. All of these factors require consideration prior to commitment to this long-term treatment strategy.

Keywords: Congenital heart disease; Implantable cardioverter-defibrillator; Sudden cardiac death; Ventricular tachycardia.

Fig. 1

Distribution of congenital heart disease (CHD) implantable cardioverter-defibrillator population by CHD lesion. Figures are adapted from the two largest population studies. The 1304 patients reported by Jordan et al with atrial septal defect (ASD) alone are excluded. No patients with VSD alone were reported by Berul et al [3, 13]

Fig. 2

a Posteroanterior and b lateral radiographs demonstrating transvenous pacing system in an 18 kg child with long QT (LQT) syndrome. Generator has been placed abdominally with lead tunnelled to left subclavian vein (cross). Note redundant loop to accommodate growth (star). c Posteroanterior and d lateral radiographs demonstrating a more complex hybrid system in a 15 kg child with LQT type 3. The first ICD implanted was non-transvenous, with intrapericardial coil (asterisk) and epicardial sense and pacing leads (white arrows). Subsequent failure of the epicardial pacing leads with R‑wave undersensing and rising defibrillation threshold necessitated upgrade of the system with subcutanous coil (scc) and transvenous atrial (a) and ventricular (v) pace and sense leads (c and d courtesy of Jasveer Mangat, Great Ormond Street Hospital, London)

Fig. 3

Diagram of transvenous implantable cardioverter-defibrillator implanted following Senning repair (patient aged 18 years) for transposition of the great arteries (a). Posteroanterior (b) and lateral (c) radiograph projections. Note ventricular coil (V Coil) placed in the posterior LV via the venous baffle. A atrial sense/pace lead, SVC superior vena cava, RA right atrium. Ao Aorta, PA pulmonary artery, LA left atrium, RV right ventricle and LV left ventricle

Fig. 4

Patient with severe scoliosis, pulmonary atresia, ventricular septal defect and major aortopulmonary collateral arteries. a Computed tomography and b Balanced steady state free precession (b‑SSFP) magnetic resonance image. c  Anteroposterior and d  lateral radiographs demonstrating subcutaneous implantable cardioverter-defibrillator with excellent shock vector (subcutaneous coil has been placed to the right of the sternum, RV right ventricle, LV left ventricle, SP spine, ST sternum)

Fig. 5

Diagram of subcutaneous implantable cardioverter-defibrillator and transvenous atrial pacemaker in patient with lateral tunnel Fontan (a). Posteroanterior (b) and lateral (c) radiographs demonstrating single chamber pacemaker (white cross) with lead to systemic venous portion of the right atrium and subcutaneous implantable cardioverter-defibrillator (white star). Figure adapted with permission from Chubb et al [7]

Fig. 6

Cardiac magnetic resonance imaging in a patient with congenital aortic stenosis (status post-Ross procedure) and magnetic resonance-conditional implantable cardioverter-defibrillator, demonstrating typical results for balance steady state free precession (b‑SSFP) cine imaging. Lead position is indicated by white arrows and the ring artefact related to the generator is seen at the top left of panels (c) and (d). a four chamber view, b short axis, c three chamber view, d right ventricular outflow tract view. RV right ventricle, LV left ventricle, Ao aorta, LA left atrium, PA pulmonary artery