Surgical and Catheter-Based Reinterventions Are Common in Long-Term Survivors of the Fontan Operation

Circ Cardiovasc Interv. 2017 Sep;10(9):e004924. doi: 10.1161/CIRCINTERVENTIONS.116.004924.

Abstract

Background: There are limited follow-up studies examining surgical and catheter-based reinterventions in long-term survivors of the Fontan operation.

Methods and results: All 773 patients who underwent Fontan at our institution between 1992 and 2009 were retrospectively reviewed. Current information regarding post-Fontan intervention was available for 70%. By 20 years after Fontan, 65% of patients had experienced either surgical or transcatheter intervention. The median time to first reintervention was 9.8 years. Freedom from reoperation was 69% at 15 years and 63% at 20 years. The most common operations were pacemaker placement and Fontan revision. Risk factors for pacemaker placement included systemic left ventricle (hazard ratio [HR], 2.2; P=0.006) and lateral tunnel Fontan (HR, 4.3; P=0.001). Freedom from interventional catheterization was 53% at 15 years and 50% at 20 years. The most common procedures performed were fenestration closure and pulmonary artery intervention. Catheter intervention for anatomic indications was associated with Fontan after 2002 (HR, 2.1; P=0.007), Norwood operation (HR, 2.3; P=0.001), and longer cardiopulmonary bypass time (HR, 1.1 per 10 minutes; P=0.001). Catheter intervention for physiological indications was associated with prolonged post-Fontan pleural drainage (HR, 4.0; P<0.001) and hypoplastic left heart syndrome (HR, 2.0; P=0.01).

Conclusions: In this study of Fontan survivors, two thirds of patients required surgical or catheter-based reintervention by 20 years. Families should be counseled that the Fontan is typically not the final stage of single-ventricle palliation.

Keywords: Fontan procedure; catheterization; cyanosis; hypoplastic left heart syndrome; mortality; tricuspid atresia.

MeSH terms

  • Adolescent
  • Adult
  • Cardiac Catheterization* / adverse effects
  • Cardiac Catheterization* / mortality
  • Child
  • Child, Preschool
  • Disease-Free Survival
  • Female
  • Fontan Procedure / adverse effects*
  • Fontan Procedure / mortality
  • Hospitals, Pediatric
  • Humans
  • Hypoplastic Left Heart Syndrome / diagnosis
  • Hypoplastic Left Heart Syndrome / mortality
  • Hypoplastic Left Heart Syndrome / physiopathology
  • Hypoplastic Left Heart Syndrome / surgery*
  • Infant
  • Kaplan-Meier Estimate
  • Male
  • Palliative Care
  • Philadelphia
  • Postoperative Complications / diagnostic imaging
  • Postoperative Complications / etiology
  • Postoperative Complications / mortality
  • Postoperative Complications / therapy*
  • Proportional Hazards Models
  • Reoperation* / adverse effects
  • Reoperation* / mortality
  • Retrospective Studies
  • Risk Factors
  • Survivors*
  • Time Factors
  • Treatment Outcome
  • Young Adult