Fontan circulation over 30 years. What should we learn from those patients?

Asian Cardiovasc Thorac Ann. 2016 Oct;24(8):765-771. doi: 10.1177/0218492316667771. Epub 2016 Aug 24.

Abstract

Background: This study aimed to evaluate Fontan circulation which was observed over 30 years.

Methods: Forty patients who underwent a Fontan operation between 1974 and 1986 and survived (group S, n = 20) or died in the late period (group LD, n = 20) were evaluated. The median age at operation was 10 years (range 2-32 years). The diagnoses were tricuspid atresia in 21, single ventricle in 9, and others in 10. The Fontan procedure was a right atrium-pulmonary artery graft in 2, atriopulmonary connection in 28, and right atrium-right ventricle anastomosis in 10 (Björk in 4, graft in 6).

Results: Causes of late death were congestive heart failure in 6, sudden death in 4, arrhythmia in 4, and others in 6. Sixteen patients underwent reoperation 23.3 ± 6.5 years after Fontan. Cardiac catheterization was performed at 1 month and 12 years. There were no significant differences in central venous pressure, ventricular ejection fraction, or pulmonary vascular resistance between the two groups. However, changes in ventricular end-diastolic volume suggested volume and pressure overload in group LD. In group S, the latest chest radiographs showed cardiothoracic ratio 51.3% ± 7.6%, oxygen saturation 94% ± 2%, brain natriuretic peptide 153 ± 111 pg ċ mL-1, and Holter monitoring revealed sinus rhythm in 10 patients. New York Heart Association class was I in 12 patients, II in 6, and III in 2.

Conclusions: For long-term Fontan survival, timely total cavopulmonary connection conversion and medication to decrease ventricular volume and pressure load might be important.

Keywords: Fontan procedure; Heart defects; Heart ventricles; Protein-losing enteropathies; Pulmonary artery; Treatment outcome; congenital.

MeSH terms

  • Adolescent
  • Adult
  • Cardiovascular Agents / therapeutic use
  • Cause of Death
  • Child
  • Child, Preschool
  • Female
  • Fontan Procedure* / adverse effects
  • Fontan Procedure* / mortality
  • Heart Defects, Congenital / diagnosis
  • Heart Defects, Congenital / mortality
  • Heart Defects, Congenital / physiopathology
  • Heart Defects, Congenital / surgery*
  • Hemodynamics
  • Humans
  • Kaplan-Meier Estimate
  • Male
  • Postoperative Complications / etiology
  • Postoperative Complications / mortality
  • Postoperative Complications / surgery
  • Reoperation
  • Retrospective Studies
  • Risk Factors
  • Time Factors
  • Tomography, X-Ray Computed
  • Treatment Outcome
  • Young Adult

Substances

  • Cardiovascular Agents