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[Short-term results of the Fontan operation in patients with Down syndrome]
1Department of Cardiovascular Surgery, Shakaihoken Chukyo Hospital, Nagoya, Japan.
Insights
The Fontan operation, a complex heart surgery, can be a viable option for select patients with Down syndrome and complex congenital heart defects when other surgical repairs are not possible. Careful patient selection is crucial for successful outcomes in this population.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Down Syndrome Genetics
Context:
- The Fontan operation is a palliative surgical procedure for complex single-ventricle congenital heart defects.
- Limited data exists on the Fontan operation's efficacy and safety in patients with Down syndrome.
- Down syndrome is frequently associated with congenital heart defects, including atrioventricular septal defects.
Purpose:
- To evaluate the outcomes of the Fontan operation in patients with Down syndrome.
- To assess the feasibility and safety of this procedure in a specific pediatric population.
- To provide guidance on the indications for the Fontan operation in Down syndrome patients with complex heart conditions.
Summary:
- A retrospective review of 79 Fontan operations between 1995 and 2003 identified 3 patients with Down syndrome.
- All 3 patients had complete atrioventricular septal defects and single-ventricle physiology.
- Two patients survived with good short-term outcomes; one patient died due to chylothorax and respiratory infection.
Impact:
- The Fontan operation may be a suitable option for carefully selected Down syndrome patients with unreconstructible single-ventricle physiology.
- This study highlights the need for individualized treatment strategies in pediatric cardiac surgery.
- Further research is warranted to optimize Fontan procedure outcomes in this patient group.
Abstract:
Despite an extensive experience with Fontan operation, there is a paucity of information to guide the indication of the procedure in patients with Down syndrome. Of 79 patients who had undergone a Fontan operation in our hospital between 1995 and 2003, 3 had Down syndrome. All 3 patients had complete atrioventricular septal defect with single ventricular physiology. Two patients survived, and 1 died of chylothorax and respiratory infection. The 2 survivors have done well in the short-term without complications. We consider that in appropriately selected patients with Down syndrome in whom biventricular repair is precluded, the Fontan operation is the choice of the procedures.