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[Castaneda-de Leval's modified Fontan operation in complex cyanotic defects with subsequent percutaneous transluminal
B Schubel1, V Gliech, H Warnke
1Klinik für Chirurgie, Bereich Medizin (Charité) der Humboldt-Universität zu Berlin.
Insights
A five-year-old boy with complex congenital heart disease experienced severe protein loss after Fontan surgery. This was successfully treated by embolizing abnormal aortopulmonary collaterals, offering a new treatment strategy.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Interventional Cardiology
Background:
- Double-inlet left ventricle (DILV) with malposed aorta, ventricular septal defect (VSD), atrial septal defect (ASD), and pulmonary stenosis presents a complex congenital heart defect.
- The Fontan procedure is a palliative surgery for single-ventricle physiology, but complications like protein-losing enteropathy (PLE) can occur.
Observation:
- A 5-year-old boy with DILV and associated defects developed severe effusions and protein loss syndrome post-modified Fontan surgery.
- Diagnostic evaluation revealed aortopulmonary collaterals as the underlying cause of the protein loss and effusions.
Findings:
- Percutaneous transluminal embolization was employed in multiple sessions to treat the identified aortopulmonary collaterals.
- Successful embolization led to the resolution of severe effusions and the protein loss syndrome.
Implications:
- This case highlights aortopulmonary collaterals as a treatable cause of protein loss syndrome after Fontan surgery.
- Percutaneous embolization offers a minimally invasive and effective therapeutic option for managing this specific complication.
- Further research into the incidence and management of aortopulmonary collaterals post-Fontan surgery is warranted.
Abstract:
The case of a five year old boy with a double-inlet-left-ventricle and the aorta in 1-malposition, large VSD, ASD and pulmonary stenosis is reported, that showed after modified Fontan surgery severe effusions and a severe protein loss syndrome. Aortopulmonary collaterals were detected as the cause of this and successfully treated in several sessions by percutaneous transluminal embolization.