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A case with heterotaxy reaching the Fontan procedure after unifocalizations
International Journal of Cardiology
|June 15, 2007
Summary
This case study details a single patient with complex congenital heart disease, including major aortopulmonary collateral arteries and heterotaxy. The patient successfully underwent staged Fontan completion with manageable complications, showing favorable pulmonary arterial pressure post-procedure.
Area of Science:
- Congenital Heart Surgery
- Pediatric Cardiology
- Cardiovascular Physiology
Background:
- Heterotaxy and major aortopulmonary collateral arteries present complex challenges in single-ventricle palliation.
- Staged surgical approaches, including unifocalization and Fontan completion, are critical for managing these conditions.
Observation:
- The patient underwent staged unifocalization and Fontan completion.
- Complications included left pulmonary artery thrombosis after the bidirectional Glenn procedure and progressive atrioventricular valve regurgitation requiring two repairs.
- Pulmonary arterial pressure was measured at 11 mm Hg one year post-Fontan.
Findings:
- Successful staged surgical palliation was achieved in a patient with major aortopulmonary collateral arteries and heterotaxy.
- Early complications, such as pulmonary artery thrombosis and atrioventricular valve regurgitation, were effectively managed.
- Favorable pulmonary arterial pressure was maintained one year after Fontan completion.
Implications:
- This case highlights the feasibility of staged Fontan completion in complex single-ventricle physiology.
- Effective management of intraoperative and postoperative complications is crucial for long-term outcomes.
- The findings suggest that successful palliation can lead to acceptable hemodynamic status in select patients.
