Open Access

REVIEW

Contemporary Management of Failing Modified Fontan after the Total Cavopulmonary Connection

Honghao Fu^#, Zhangwei Wang^#, Shoujun Li^*

Department of Cardiovascular Surgery, Chinese Academy of Medical Sciences and Peking Union Medical College, National Center for Cardiovascular Diseases, Fuwai Hospital, Beijing, 100037, China

* Corresponding Author: Shoujun Li. Email:
# These authors contributed equally to this work

(This article belongs to the Special Issue: Novel Methods and Techniques for the Management of Congenital Heart Disease)

Congenital Heart Disease 2025, 20(3), 287-303. https://doi.org/10.32604/chd.2025.067619

Received 08 May 2025; Accepted 27 June 2025; Issue published 11 July 2025

Abstract

Congenital heart disease (CHD) stands as the most common cardiovascular disorder among children, exerting a profound impact on the growth, development, and quality of life of the affected pediatric population. The modified Fontan procedure, the total cavopulmonary connection (TCPC), has become a pivotal palliative or definitive surgical method for treating complex CHD cases, including single ventricle and tricuspid valve atresia. Through staged surgical processes, this technique directly diverts vena cava blood into the pulmonary artery, thus improving the patient’s oxygenation status. Despite the initial success of the Fontan circulation in providing a means for survival in patients with complex CHD, a significant proportion of patients will eventually experience Fontan failure. Fontan failure is a complex syndrome characterized by a constellation of symptoms and signs, including heart failure, arrhythmia, protein-losing enteropathy, and plastic bronchitis. Understanding the contemporary management of failing modified Fontan after TCPC is crucial for optimizing patient outcomes, as the number of adult patients with Fontan circulation continues to grow due to improved surgical techniques and postoperative care.

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