Evidence map›Paper›PMID 41501943›Full record

ArticleGeneral thoracic and cardiovascular surgery cases2026

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

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

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Article in General thoracic and cardiovascular surgery cases, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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4 · The record

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5 · Who and what money

Authors and funding

9 authors.

Sakura HorieDepartment of Cardiovascular Surgery, The University of Tokyo Hospital, 7-3-1 Hongo, Tokyo, Bunkyo-Ku, 113-8655, Japan.
Fumiaki ShikataDepartment of Cardiovascular Surgery, The University of Tokyo Hospital, 7-3-1 Hongo, Tokyo, Bunkyo-Ku, 113-8655, Japan. fumishikata@outlook.com.ORCID http://orcid.org/0000-0001-9325-1877
Yasutaka HirataDepartment of Cardiovascular Surgery, National Center for Child Health and Development, Tokyo, Japan.
Shigeto TsujiDepartment of Cardiovascular Surgery, The University of Tokyo Hospital, 7-3-1 Hongo, Tokyo, Bunkyo-Ku, 113-8655, Japan.
Sadayuki MoriyamaDepartment of Cardiovascular Surgery, The University of Tokyo Hospital, 7-3-1 Hongo, Tokyo, Bunkyo-Ku, 113-8655, Japan.
Masahiko AndoDepartment of Cardiovascular Surgery, The University of Tokyo Hospital, 7-3-1 Hongo, Tokyo, Bunkyo-Ku, 113-8655, Japan.
Ryo InuzukaDepartment of Pediatrics, The University of Tokyo Hospital, Tokyo, Japan.
Hideyuki KatoDepartment of Cardiovascular Surgery, University of Tsukuba Hospital, Ibaraki, Japan.
Minoru OnoDepartment of Cardiovascular Surgery, The University of Tokyo Hospital, 7-3-1 Hongo, Tokyo, Bunkyo-Ku, 113-8655, Japan.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundOrthotopic 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.

conclusionsThis 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.

Indexed as

Extracardiac total cavopulmonary connectionFontan circulationFontan failureOrthotopic heart transplantationPediatric heart transplantation

Identifiers

PMID41501943
PMCPMC12781744

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