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Published on: December 11, 2017
Severe coronary sinus dilatation after a modification of the classic Fontan operation: successful resolution with a
A L Mock1, C D Fraser, W J Dreyer
1Department of Pediatrics, Baylor College of Medicine, Texas Children's Hospital, Houston, TX 77030, USA.
Insights
Two patients with single ventricle and left superior vena cava experienced severe dilation after Fontan modification. Revision surgery improved their functional capacity, offering a potential solution for complex congenital heart disease.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Congenital Heart Disease
Background:
- Functional single ventricle physiology presents unique surgical challenges.
- The Fontan procedure is a palliative treatment for complex single-ventricle defects.
- Anatomic variations like left superior vena cava require specific surgical considerations.
Observation:
- Two patients with single ventricle and left superior vena cava (LSVC) underwent Fontan modification incorporating the coronary sinus.
- Both patients developed significant right atrial and coronary sinus dilation post-Fontan.
- This dilation suggests potential complications related to altered venous drainage pathways.
Findings:
- Revision of the Fontan pathway using an intraatrial lateral tunnel and extracardiac conduit was performed.
- Post-revision, both patients demonstrated improved functional capacity.
- The surgical revision effectively addressed the complications arising from the initial Fontan modification.
Implications:
- Intraatrial lateral tunnel and extracardiac conduit revisions may be effective for managing Fontan-associated complications.
- Understanding venous drainage patterns in single-ventricle physiology is crucial for surgical planning.
- Further research is needed to optimize Fontan pathway modifications in patients with complex venous anomalies.
Abstract:
Two patients with functional single ventricle and a left superior vena cava, which drained to the coronary sinus, are presented. Each had undergone a Fontan modification that incorporated the coronary sinus into the Fontan pathway. Each developed severe right atrial and coronary sinus dilatation. Revision of a Fontan with an intraatrial lateral tunnel and an extracardiac conduit resulted in improved functional capacity of the patients.
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