Pacing and Defibrillators in Complex Congenital Heart Disease

Abstract

Device therapy in the complex congenital heart disease (CHD) population is a challenging field. There is a myriad of devices available, but none designed specifically for the CHD patient group, and a scarcity of prospective studies to guide best practice. Baseline cardiac anatomy, prior surgical and interventional procedures, existing tachyarrhythmias and the requirement for future intervention all play a substantial role in decision making. For both pacing systems and implantable cardioverter defibrillators, numerous factors impact on the merits of system location (endovascular versus non-endovascular), lead positioning, device selection and device programming. For those with Fontan circulation and following the atrial switch procedure there are also very specific considerations regarding access and potential complications. This review discusses the published guidelines, device indications and the best available evidence for guidance of device implantation in the complex CHD population.

Management of People With a Fontan Circulation: a Cardiac Society of Australia and New Zealand Position statement

“…Beta blockers may have some protective effect against VT (Class IIb recommendation) [196,197]. 9.1.6 Defibrillator Implantation Indications for an ICD in people with a Fontan circulation include cardiac arrest survival and spontaneous sustained VT, after a careful work-up has failed to identify a clearly reversible cause [220]. ICD therapy may be reasonable in adults with a single or systemic right ventricular ejection fraction <35% (Class IIb recommendation), particularly in the presence of risk factors such as complex ventricular arrhythmias, unexplained syncope, New York Heart Association functional Class II or III symptoms, QRS duration !140 ms, or severe systemic AV valve regurgitation [196].…”

Acute and Long-Term Outcomes of Transvenous Cardiac Pacing Device Implantation in Patients With Congenital Heart Disease

“…While cardiac surgery (if possible, minimally invasive surgery using an epicardial lead system) is a viable option for CHD patients with intracardiac shunt or SVC syndrome, an alternative option of closing cardiac shunts and stent implantation for stenotic veins before or during transvenous CIED implantation tends to reduce transvenous approach-related complications. 26 Recent advances in lead extraction techniques have contributed to the increasing number of transvenous lead implantations performed in patients with CHD. 27 , 28 The relatively young age at CIED implantation of patients with CHD compared with non-CHD patients requires long-term follow-up after implantation.…”

Heart failure in congenital heart disease: management options and clinical challenges

“…In patients with complex congenital conditions anatomical obstacles may hamper implantation. This may be related to previous surgeries such as atrial switch operations or Fontan procedures, to vascular access either congenital or due to previous endovascular procedures and to options for endo-or epicardial lead placement [100]. In patients with single ventricle physiology after the modified Fontan procedure, the role of CRT in significant ventricular dysfunction is not well studied.…”