Relationship of Ventricular Morphology and Atrioventricular Valve Function to Long-Term Outcomes Following Fontan Procedures

J Am Coll Cardiol. 2020 Jul 28;76(4):419-431. doi: 10.1016/j.jacc.2020.05.059.


Background: The influence of ventricular morphology on Fontan outcomes is controversial.

Objectives: This study hypothesized that dysfunction of the single right ventricle (RV) and right atrioventricular valve regurgitation (AVVR) increases over time and adversely impacts late outcomes following a Fontan operation. A single-center retrospective study was performed.

Methods: From 1985 through 2018, 1,162 patients underwent the Fontan procedure at our center and were included in this study. Transplant and takedown free survival, ventricular, and atrioventricular valve dysfunction after Fontan were analyzed. Death or heart transplantation information was obtained from the National Death Index and the Scientific Registry of Transplant Recipients.

Results: The follow-up rate was 99%. Morphologic RV was present in 58% of patients. Transplant and takedown free survival were 91%, 75%, and 71% at 10 years, 20 years, and 25 years, respectively. Morphologic RV was an independent risk factor for transplant, takedown free survival (hazard ratio: 2.4; p = 0.008). The AVVR, which preceded ventricular dysfunction in most cases, was associated with the development of ventricular dysfunction after Fontan (odds ratio: 4.3; 95% confidence interval: 2.7 to 6.7; p < 0.001). Furthermore, AVVR and ventricular dysfunction progressed over time after Fontan, especially in the RV (AVVR: p < 0.0001, ventricular dysfunction: p < 0.0001).

Conclusions: Morphologic RV is negatively associated with the long-term survival following the Fontan, possibly due to a tendency toward progressive AVVR and deterioration of the single ventricle function. Additional volume overload caused by AVVR may be one of the main factors accelerating the dysfunction of the single RV, implying that early valve intervention may be warranted.

Keywords: Fontan; atrioventricular valve regurgitation; heart transplant; long-term outcome; systemic right ventricle.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Adult
  • Female
  • Fontan Procedure* / adverse effects
  • Fontan Procedure* / methods
  • Heart Defects, Congenital / mortality
  • Heart Defects, Congenital / surgery
  • Heart Transplantation / methods
  • Heart Transplantation / statistics & numerical data*
  • Heart Ventricles* / pathology
  • Heart Ventricles* / physiopathology
  • Humans
  • Long Term Adverse Effects* / etiology
  • Long Term Adverse Effects* / mortality
  • Long Term Adverse Effects* / physiopathology
  • Male
  • Michigan
  • Outcome and Process Assessment, Health Care
  • Tricuspid Valve Insufficiency* / diagnosis
  • Tricuspid Valve Insufficiency* / physiopathology
  • Ventricular Dysfunction, Right* / etiology
  • Ventricular Dysfunction, Right* / physiopathology