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Outcome of congenital diaphragmatic hernia with indication for Fontan procedure
Nobuko Shiono1, Noboru Inamura, Shigehiro Takahashi
1Department of Pediatric Cardiology, Osaka Medical Center and Research Institute for Maternal and Child Health, Osaka, Japan.
Insights
Patients with congenital diaphragmatic hernia (CDH) and cardiovascular malformation (CVM) rarely need the Fontan procedure. Those with left ventricular outflow tract obstruction (LVOTO) have a poor prognosis, highlighting a critical surgical consideration.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Defects
- Surgical Outcomes
Background:
- Congenital diaphragmatic hernia (CDH) can present with complex cardiovascular malformations (CVM).
- The Fontan procedure is a palliative option for specific univentricular heart conditions.
Purpose of the Study:
- To investigate the outcomes of CDH patients with CVM who are candidates for the Fontan procedure.
- To identify factors influencing survival in this high-risk population.
Main Methods:
- Retrospective analysis of 76 CDH patients with CVM from a national survey.
- Comparison of outcomes between patients with and without indication for the Fontan procedure.
- Evaluation of the impact of left ventricular outflow tract obstruction (LVOTO) on survival.
Main Results:
- Only 2.6% of CDH patients (16/614) with CVM were candidates for the Fontan procedure; none underwent it.
- Absence of LVOTO significantly improved 90-day survival (71.4% vs. 0%, P=0.0007).
- Excluding genetic abnormalities, 90-day survival was better in neonates without Fontan indication (62.5% vs. 28.6%, P=0.0271).
Conclusions:
- Fontan procedure candidacy is uncommon in CDH patients with CVM.
- Left ventricular outflow tract obstruction (LVOTO) is a critical negative prognostic factor in this cohort.
- These findings underscore the complexity and poor outcomes associated with specific CVMs in CDH.
Background:
The aim of this study was to clarify the outcome of patients with cardiovascular malformation (CVM) among those with congenital diaphragmatic hernia (CDH) who are indicated for the Fontan procedure.
Methods:
The subjects included 76 CDH patients with CVM recruited from a national survey of 614 CDH patients. The outcomes were evaluated between two groups divided according to indication for the Fontan procedure. Patients with functional univentricular disease were considered to be candidates for the Fontan procedure.
Results:
Sixteen (21.1%) of the 76 patients were candidates for the Fontan procedure, accounting for 2.6% of all 614 patients with CDH. None of these patients, however, underwent the Fontan procedure. Among the 16 patients, the absence of obstruction of the left ventricular outflow tract (LVOTO) was significantly associated with better 90 day survival (71.4%, 5/7, for those without LVOTO vs 0.0%, 0/9, for those with LVOTO, P = 0.0007). After excluding 22 patients with chromosomal and/or genetic abnormalities or syndromes, the 90 day survival rate was significantly better in neonates without than with indication for the Fontan procedure (62.5%, 25/40 vs 28.6%, 4/14, P = 0.0271).
Conclusions:
Patients with indications for the Fontan procedure are rare, and the outcome of patients with LVOTO among those with CDH is especially poor.
