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[Fontan procedure for asplenia syndrome]
1Department of Cardiovascular Surgery, Kanagawa Children's Medical Center, Yokohama, Japan.
Kyobu Geka. the Japanese Journal of Thoracic Surgery
|April 19, 2003
Summary
Recent Fontan operation modifications improve survival for patients with single ventricle asplenia syndrome. Surgical techniques and fenestration use contribute to better outcomes in this complex congenital heart defect population.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Surgery
- Single Ventricle Physiology
Context:
- The Fontan operation is a palliative procedure for complex single-ventricle congenital heart disease.
- Historically, patients with asplenia syndrome undergoing Fontan palliation faced high mortality rates.
- Recent advancements in surgical techniques may offer improved outcomes.
Purpose:
- To evaluate the impact of recent surgical modifications on Fontan operation outcomes in patients with asplenia syndrome.
- To assess survival rates and complications in this specific patient cohort.
Summary:
- A retrospective review included 11 patients with asplenia syndrome who underwent Fontan operations between 1996 and 2002.
- Surgical modifications included extracardiac tube-graft, lateral tunnel, and intra-atrial/intra-extra atrial grafts, with fenestration used in 27% of cases.
- No early deaths occurred; two late hospital deaths (18%) were reported due to tachyarrhythmia and Fontan circulation failure. No late deaths or complications were observed during a mean 3-year follow-up.
Impact:
- The Fontan operation can now be performed with good survival in patients with asplenia syndrome.
- Improved outcomes are attributed to Fontan staging, appropriate surgical modifications, management of associated anomalies, and the use of fenestration.