Pacemaker treatment after Fontan surgery—A Swedish national study

Abstract

Objective Fontan surgery is performed in children with univentricular heart defects. Previous data regarding permanent pacemaker implantation frequency and indications in Fontan patients are limited and conflicting. We examined the prevalence of and risk factors for pacemaker treatment in a consecutive national cohort of patients after Fontan surgery in Sweden. Methods We retrospectively reviewed all Swedish patients who underwent Fontan surgery from 1982 to 2017 (n = 599). Results After a mean follow‐up of 12.2 years, 13% (78/599) of the patients with Fontan circulation had received pacemakers. Patients operated with the extracardiac conduit (EC) type of total cavopulmonary connection had a significantly lower prevalence of pacemaker implantation (6%) than patients with lateral tunnel (LT; 17%). Mortality did not differ between patients with (8%) and without pacemaker (5%). The most common pacemaker indication was sinus node dysfunction (SND) (64%). Pacemaker implantation due to SND was less common among patients with EC. Pacemaker implantation was significantly more common in patients with mitral atresia (MA; 44%), double outlet right ventricle (DORV; 24%) and double inlet left ventricle (DILV; 20%). In contrast, patients with pulmonary atresia with intact ventricular septum and hypoplastic left heart syndrome were significantly less likely to receive a pacemaker (3% and 6%, respectively). Conclusions Thirteen percent of Fontan patients received a permanent pacemaker, most frequently due to SND. EC was associated with a significantly lower prevalence of pacemaker than LT. Permanent pacemaker was more common in patients with MA, DORV, and DILV.

Salvage of an epicardial lead in a pacemaker‐dependent patient with Fontan palliation using an IS‐1 extender

“…Extensive and multiple surgeries involving placement of intra‐atrial baffles contribute to sinus node dysfunction and the need for atrial pacing 1–3 . Patients with intracardiac Fontan and double‐inlet left ventricles (such as the patient presented in this case report) are more likely to need a permanent pacemaker 2 . Other characteristics associated with higher risk for need of pacemaker implantation are mitral atresia, double outlet right ventricle and heterotaxy syndrome 2 …”

“…90% of patients with Fontan palliation who have survived to age ≥ 16 will survive at 30 years and 80% at 40 years of age ( N = 683) 6 . Patients with Fontan palliation commonly require pacemaker implantation (6–13% of cases 2,6 ), and in some cohorts pacemaker insertion or revision is the most common reason for reoperation (up to 20% of cases) 8 . The most common indication for pacemaker implantation is sinus node dysfunction (64% of cases) 2 .…”

“…Patients with Fontan palliation commonly require pacemaker implantation (6–13% of cases 2,6 ), and in some cohorts pacemaker insertion or revision is the most common reason for reoperation (up to 20% of cases) 8 . The most common indication for pacemaker implantation is sinus node dysfunction (64% of cases) 2 . Extensive and multiple surgeries involving placement of intra‐atrial baffles contribute to sinus node dysfunction and the need for atrial pacing 1–3 .…”

“…Delivering atrial pacemaker leads in patients with Fontan palliation is challenging since, with modern Fontan procedures, the venous circulation is directed to the pulmonary arteries with a conduit, bypassing the atrium. Atrial leads can be delivered epicardially with surgical approaches or, in select cases of intracardiac conduits, transvenously with complex interventions that involve puncture of the Fontan conduit 2–5 . Case reports are available that describe transvenous lead implantation in patients with extracardiac Fontan conduits 4,5,9,10 .…”

“…Sinus node dysfunction and the need for atrial pacing is common in patients with Fontan palliation 1–3 . Pacemaker implantation in patients with Fontan palliation is challenging as, with modern Fontan procedures, the venous circulation is directed to the pulmonary arteries with a conduit, bypassing the systemic atrium 2–5 . This anatomy makes placement of a pacing lead via the typical venous routes nearly impossible.…”