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Background <p>Orthotopic heart transplantation after the Fontan operation presents technical surgical challenges due to the connection of systemic veins to pulmonary arteries and well-developed systemic-to-pulmonary collateral arteries. The altered anatomy and hemodynamics necessitate extensive vascular reconstruction. We report a successful orthotopic heart transplantation with three years of ventricular assist device (VAD) support in a child who had undergone the Fontan operation.</p> Case presentation <p>A 10-year-old boy had undergone extracardiac total cavopulmonary connection (18&#xa0;mm expanded polytetrafluoroethylene conduit) at 2&#xa0;years of age for a large ventricular septal defect, straddling tricuspid valve, and mitral stenosis. Following the Fontan operation, his systemic ventricular function gradually deteriorated. At 7&#xa0;years of age, a Berlin Heart EXCOR® Pediatric VAD was implanted due to progressive heart failure, and he was listed for heart transplantation. Three years later, a heart transplant was performed.</p> <p>Cardiopulmonary bypass was established via cervical cannulation before re-sternotomy. The superior vena cava and extracardiac conduit were detached from the pulmonary artery. The pulmonary artery was reconstructed from hilum to hilum with a large bovine pericardial patch. Well-developed systemic-to-pulmonary collaterals caused excessive left atrial return; therefore, the left atrial anastomosis was performed under deep hypothermic circulatory arrest. The systemic veins were reconstructed with bicaval anastomosis, and inferior vena caval continuity was restored by leaving a short segment of the previous conduit. The procedure was completed without complications. Postoperative recovery was uneventful, and the patient was discharged on day 35.</p> Conclusions <p>This case illustrates a successful approach to orthotopic heart transplantation in a child with failing Fontan circulation supported by a VAD. Reconstruction of the pulmonary artery using a large pericardial patch and restoration of bicaval continuity were key to overcoming complex anatomical challenges.</p>

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Orthotopic heart transplantation for Fontan failure: experience and treatment strategy—a case report

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2026年1月7日

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  • Sakura Horie,
  • Fumiaki Shikata,
  • Yasutaka Hirata,
  • Shigeto Tsuji,
  • Sadayuki Moriyama,
  • Masahiko Ando,
  • Ryo Inuzuka,
  • Hideyuki Kato,
  • Minoru Ono

摘要

Background

Orthotopic heart transplantation after the Fontan operation presents technical surgical challenges due to the connection of systemic veins to pulmonary arteries and well-developed systemic-to-pulmonary collateral arteries. The altered anatomy and hemodynamics necessitate extensive vascular reconstruction. We report a successful orthotopic heart transplantation with three years of ventricular assist device (VAD) support in a child who had undergone the Fontan operation.

Case presentation

A 10-year-old boy had undergone extracardiac total cavopulmonary connection (18 mm expanded polytetrafluoroethylene conduit) at 2 years of age for a large ventricular septal defect, straddling tricuspid valve, and mitral stenosis. Following the Fontan operation, his systemic ventricular function gradually deteriorated. At 7 years of age, a Berlin Heart EXCOR® Pediatric VAD was implanted due to progressive heart failure, and he was listed for heart transplantation. Three years later, a heart transplant was performed.

Cardiopulmonary bypass was established via cervical cannulation before re-sternotomy. The superior vena cava and extracardiac conduit were detached from the pulmonary artery. The pulmonary artery was reconstructed from hilum to hilum with a large bovine pericardial patch. Well-developed systemic-to-pulmonary collaterals caused excessive left atrial return; therefore, the left atrial anastomosis was performed under deep hypothermic circulatory arrest. The systemic veins were reconstructed with bicaval anastomosis, and inferior vena caval continuity was restored by leaving a short segment of the previous conduit. The procedure was completed without complications. Postoperative recovery was uneventful, and the patient was discharged on day 35.

Conclusions

This case illustrates a successful approach to orthotopic heart transplantation in a child with failing Fontan circulation supported by a VAD. Reconstruction of the pulmonary artery using a large pericardial patch and restoration of bicaval continuity were key to overcoming complex anatomical challenges.