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Long-term follow-up of atrioventricular valve function in Fontan patients: effect of atrioventricular valve surgery
Thierry Bove1, Eva Grootjans1, Romanie Naessens1
1Department of Cardiac Surgery, University Hospital of Ghent, Ghent, Belgium.
Insights
Fontan survivors show encouraging long-term survival, with 12.4% needing atrioventricular valve surgery. However, progressive declines in valve and ventricular function over time warrant further investigation into heart failure therapies.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Congenital Heart Disease
Background:
- The Fontan procedure is a palliative surgery for single-ventricle congenital heart defects.
- Long-term outcomes in Fontan survivors are influenced by atrioventricular valve and ventricular function.
- Understanding the relationship between these factors is crucial for optimizing patient management.
Purpose of the Study:
- To evaluate the relationship between atrioventricular valve function and ventricular function in Fontan survivors.
- To assess the impact of atrioventricular valve surgery on long-term outcomes.
- To analyze transplant-free survival rates in this population.
Main Methods:
- Analysis of transplant-free survival and need for atrioventricular valve surgery in single-ventricle patients post-Fontan completion.
- Longitudinal echocardiographic assessment of long-term valve and ventricular function.
- Evaluation of 113 patients with a median follow-up of 16.3 years.
Main Results:
- 25-year transplant-free survival was 90.4% ± 5.8%.
- Atrioventricular valve surgery was performed in 12.4% of patients.
- Progressive time-related decline in both atrioventricular valve function (HR 1.112) and ventricular function (HR 1.141) was observed.
Conclusions:
- Long-term transplant-free survival in a homogeneously treated Fontan population is encouraging.
- While atrioventricular valve surgery can result in durable function, a slow, time-related decline in valve and ventricular function is a concern.
- Further research into additional heart failure therapies is warranted for Fontan survivors.
Objectives:
The aim of this study was to evaluate the relationship between atrioventricular valve and ventricular function in Fontan survivors, including the effect of atrioventricular valve surgery.
Methods:
Analysis focused on transplant-free survival and the need for atrioventricular valve surgery in single ventricle patients after Fontan completion. Longitudinal echocardiographic examination of long-term valve and ventricular function was performed.
Results:
Fontan completion was performed in 113 patients, having a right univentricular morphology in 33.6%, a left ventricle morphology in 62.8% and ambiguous in 3.6%. Perioperative mortality was 2.7% (n = 3). Within a median follow-up of 16.3 years (interquartile range 10.6-23.6), transplant-free survival was 96.1 ± 1.9% and 90.4 ± 5.8% at 10-25 years. Twenty AV valve procedures were performed in 14 (12.4%) children, respectively, pre-Fontan (n = 10), per-Fontan (n = 8) and post-Fontan (n = 2), resulting in a cumulative incidence of AV valve surgery is 5.7 ± 2.2% and 12.3 ± 3.2% at 1-5 years. Atrio-ventricular valve function deteriorated over time [hazard ratio (HR) 1.112, 95% confidence interval (CI) 1.089-1.138, P < 0.001], without difference for valve morphology (P = 0.736) or ventricular dominance (P = 0.484). AV valve dysfunction was greater in patients requiring AV valve surgery (HR 20.383, 95% CI 6.223-36.762, P < 0.001) but showed a comparable evolution since repair to those without valve surgery (HR 1.070, 95% CI 0.987-1.160, P = 0.099). Progressive time-related ventricular dysfunction was observed (HR 1.141, 95% CI 1.097-1.182, P < 0.001), significantly less in left ventricle-dominance (HR 0.927, 95% CI 0.860-0.999, P = 0.047) but more after AV valve surgery (HR 1.103, 95% CI 1.014-1.167, P = 0.022).
Conclusions:
In a homogeneously treated Fontan population, 25-year transplant-free survival is encouraging. Atrio-ventricular valve surgery was necessary in 12.4%, resulting mostly in a durable valve function. However, a slow time-related decline of atrioventricular valve function as of ventricular function is worrisome, evoking a role for additional heart failure therapy.
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