Ventricular Morphology and Outcomes After Fontan Procedure

Quick Takes

  • RV morphology appears to confer lower transplant and takedown-free survival in patients after Fontan as compared with LV morphology.
  • Progressive atrioventricular valve regurgitation may contribute to RV dysfunction.
  • The authors suggest that early valve intervention may be warranted in patients with RV morphology after the Fontan procedure.

Study Questions:

What is the impact of ventricular morphology and atrioventricular valve function on late outcomes following the Fontan procedure?


A retrospective review was performed at a single center for patients undergoing the Fontan procedure between 1985 and 2018. Transplant and takedown-free survival, ventricular, and atrioventricular valve dysfunction were analyzed. Data regarding death or heart transplantation were obtained from the National Death Index and the Scientific Registry of Transplant Patients.


A total of 1,162 patients underwent the Fontan procedure during the study period, with transplant and takedown-free survival of 91%, 75%, and 71% at 10, 20, and 25 years, respectively. Morphologic right ventricle (RV) was an independent risk factor for transplant and takedown-free survival (hazard ratio, 2.4; p = 0.008). Atrioventricular valve regurgitation (AVVR) preceded ventricular dysfunction in most cases and was associated with the development of ventricular dysfunction after Fontan (odds ratio, 4.3; 95% confidence interval, 2.7-6.7; p < 0.001). AVVR and ventricular dysfunction appeared progressive after Fontan, particularly patients with RV morphology (AVVR: p < 0.0001, ventricular dysfunction: p < 0.0001).


The authors concluded that morphologic RV is negatively associated with long-term survival following the Fontan. There is a tendency for progressive AVVR and ventricular dysfunction, particularly in patients with systemic RVs.


Although it is intuitive that patients with single ventricle heart disease with left ventricle (LV) morphology would do better in the long-run than patients with an RV, there has been little evidence to suggest that this is the case. This single-center study, performed at a referral center for hypoplastic left heart syndrome and other complex single ventricle lesions, suggests that a morphologic RV is an independent risk factor for death and need for Fontan takedown, transplant, or death. Patients with single RVs appeared to have a greater degree of progressive AVVR, which may have contributed to ventricular dysfunction and subsequent poor outcomes. Of note, the center approaches the single ventricle palliation with a hemi-Fontan procedure at the second stage, followed by a lateral tunnel Fontan for the third stage, as compared with many centers who perform a bidirectional Glenn procedure followed by an extracardiac conduit Fontan. Studies such as this one also provide a look at current long-term survival of our Fontan population, with this study showing 71% survival at 25 years, which is in line with previous reported data.

Clinical Topics: Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Heart Failure and Cardiomyopathies, Invasive Cardiovascular Angiography and Intervention, Prevention, Cardiac Surgery and Arrhythmias, Cardiac Surgery and CHD and Pediatrics, Cardiac Surgery and Heart Failure, Congenital Heart Disease, CHD and Pediatrics and Arrhythmias, CHD and Pediatrics and Interventions, Acute Heart Failure, Heart Transplant, Interventions and Structural Heart Disease

Keywords: Cardiac Surgical Procedures, Fontan Procedure, Heart Defects, Congenital, Heart Failure, Heart Transplantation, Heart Valves, Hypoplastic Left Heart Syndrome, Secondary Prevention, Ventricular Dysfunction

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