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Long-term outcomes of atrioventricular septal defect and single ventricle: A multicenter study

Sara C Arrigoni1, Rinske IJsselhof2, Douwe Postmus3

  • 1Department of Cardiothoracic Surgery, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands.

The Journal of Thoracic and Cardiovascular Surgery
|June 8, 2021
PubMed

Insights

This study found that Fontan completion survival rates for single-ventricle patients with atrioventricular septal defects are better than previously reported. Atrioventricular valve repair is recommended for moderate-severe regurgitation to improve survival.

Area of Science:

  • Pediatric Cardiology
  • Congenital Heart Disease
  • Cardiac Surgery

Background:

  • Single-ventricle physiology presents complex challenges in congenital heart disease.
  • Unbalanced atrioventricular septal defects (AVSD) often require surgical intervention.
  • The Fontan procedure is a palliative surgery for single-ventricle defects, with outcomes influenced by associated anomalies.

Purpose of the Study:

  • To analyze survival and Fontan completion rates in patients with single-ventricle and unbalanced atrioventricular septal defects.
  • To evaluate the impact of atrioventricular valve procedures on survival and Fontan completion.
  • To identify risk factors for adverse outcomes in this patient population.

Main Methods:

  • Retrospective analysis of 151 patients with single-ventricle AVSD from Dutch and Belgian databases.
  • Comparison of outcomes between patients who underwent atrioventricular valve procedures and those who did not.
  • Assessment of survival, Fontan completion incidence, and freedom from atrioventricular valve reoperation.

Main Results:

  • Long-term survival was 71.2% at 10 years and 68.5% at 20 years.
  • Fontan completion incidence reached 77.6% by 15 years.
  • Moderate-severe or severe atrioventricular valve regurgitation significantly worsened 15-year survival (58.3%) compared to mild regurgitation (89.2%).

Conclusions:

  • Fontan completion outcomes in single-ventricle AVSD patients are favorable and exceed previous reports.
  • Atrioventricular valve procedures do not negatively impact mortality or Fontan completion rates.
  • Repair of atrioventricular valves is crucial when regurgitation is moderate-severe or severe to improve long-term survival.
Abstract

Keywords:
Fontansingle ventricle

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