Pediatric/Adult Congenital Heart Disease -> Pediatric Cardiology D-PO04 - Poster Session IV (ID 15) Poster

D-PO04-036 - Long-term Post-fontan Survival In Patients With And Without Pre-fontan Pacemaker In A Multicenter Registry (ID 1185)

 D. Cortez: Nothing relevant to disclose.


Background: Bradyarrhythmias are common in single ventricle (SV) patients undergoing staged palliation. The effect of pre-Fontan pacemaker (PM) implantation on outcomes is valuable for clinical decision-making.
Objective: To evaluate long-term transplant-free survival in SV patients with pre-Fontan PM implantation.
Methods: We queried the PCCC, a US-based registry of CHD interventions, for Fontan operations, with outcome data by linkage to national death/transplant registries. SV patients with PM prior to Fontan were matched to controls without PM by age at initial surgery, sex, surgical era, and type of SV.
Results: Of 1,603 patients undergoing Fontan, we identified 39 with prior PM and 154 controls. Primary arrhythmia was atrioventricular block (AVB) in 59% (congenital 26%, early postoperative 15%, other acquired 18%), sinus node dysfunction in 23% (without overt risk factors), and miscellaneous in 18%. Embryologic risk factors (L-looping, left atrial isomerism type heterotaxy) were common in congenital AVB (80%) and other acquired AVB (71%) but not early postoperative AVB (17%) nor controls (15%). Transplant-free survival at 20 years after Fontan (Figure) was lower in the PM group (63% vs 87%, p=0.0461), especially when conditioned on survival to hospital discharge (65% vs 94%, p=0.0003), with progressive attrition over time.
Conclusion: Transplant-free survival after Fontan is substantially lower with PM implantation prior to Fontan. With early identification of high-risk patients, it may be possible to use cardiac resynchronization or transplant to improve outcomes.