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Background: The discussion of Fontan fenestration is difficult because many institutions have different strategies over time. In our institute, we performed a non-fenestrated Fontan procedure for single-ventricular physiology as our definitive strategy.

Methods: Between August 1999 and December 2007, 72 consecutive patients with single-ventricle physiology underwent extracardiac total cavopulmonary connection without fenestration as our definitive strategy.

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The conventional Damus-Kaye-Stansel procedure may cause coronary artery compression when the coronary arteries are situated between the great arteries. We have performed a modified Damus-Kaye-Stansel procedure utilizing a "flap-bridging technique," in which an inverted U-shaped flap incised from the aorta is bridged to the main pulmonary trunk, creating sufficient space between the great arteries, in an 8-month-old boy who was a Fontan candidate with congenitally corrected transposition of the great arteries. This modified approach yielded favorable outcomes without coronary events and can effectively prevent coronary obstruction in cases where the coronary arteries run between the great arteries.

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Patients With Hypoplastic Left Heart Syndrome Have a Shorter Superior Vena Cava.

Ann Thorac Surg Short Rep

September 2024

Biostatistics Unit, Department of Data Science, National Center for Child Health and Development, Tokyo, Japan.

Background: The primary treatment for hypoplastic left heart syndrome (HLHS) is the Fontan pathway, which entails performing the Glenn procedure. We hypothesized that the superior vena cava in patients with HLHS was short. As the length of the superior vena cava influences the Glenn procedure, we compared its length between patients with HLHS and those with other congenital heart diseases.

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Background: Single-ventricle cardiac defects (SVCDs) are among of the most health care resource-intensive congenital diseases. Although SVCDs are traditionally palliated using the Norwood pathway, in the last 2 decades select programs have used the hybrid strategy, which redistributes the operative and interstage risks. This study sought to characterize resource use for a cohort of patients with hybrid-palliated SVCD.

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Clinical predictors and noninvasive imaging in Fontan-associated liver disease: A systematic review and meta-analysis.

Hepatol Commun

December 2024

Division of Pediatric Cardiology, Department of Pediatrics, Congenital Heart Disease Center, Severance Cardiovascular Hospital, Yonsei University College of Medicine, Seoul, Republic of Korea.

Background: Despite the development of several imaging modalities for diagnosing Fontan-associated liver disease (FALD), there is no optimal protocol for the follow-up of FALD. We conducted a systematic review and meta-analysis to identify factors related to liver fibrosis using biopsy reports and to identify alternative noninvasive modalities that could better reflect liver histological changes in FALD.

Methods: A systematic review and meta-analysis were conducted following the PRISMA guidelines Table S2.

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