Online ISSN: 2187-2988 Print ISSN: 0911-1794
特定非営利活動法人日本小児循環器学会 Japanese Society of Pediatric Cardiology and Cardiac Surgery
Pediatric Cardiology and Cardiac Surgery 40(1): 3-8 (2024)


左心低形成症候群(HLHS)の外科治療における術式の変遷Surgical Technique Variations in the Norwood Operation for Hypoplastic Left Heart Syndrome

長野県立こども病院 心臓血管外科Department of Cardiovascular Surgery, Nagano Children’s Hospital ◇ Nagano, Japan

発行日:2024年2月29日Published: February 29, 2024

左心低形成症候群(hypoplastic left heart syndrome: HLHS)の治療成績は向上しているが依然としてハイリスク疾患である.





Although treatment for hypoplastic left heart syndrome has improved, this condition is still considered high-risk.

[Aortic reconstruction] Aortic stenosis causes postoperative heart failure by increasing afterload and thus pulmonary blood flow. Patch augmentation of the entire arch is a common reconstruction technique for creating a large flow path. In Japan, however, reconstruction with primarily autologous tissue is common.

[Pulmonary artery reconstruction] The pulmonary blood flow must be adequate. Excessive pulmonary blood flow causes cardiac volume overload, while insufficient blood flow results in hypoxemia. A right ventricular-pulmonary artery conduit provides hemodynamic stability. This is considered an option, particularly for patients with a poor preoperative condition, such as low cardiac function or tricuspid valve regurgitation. However, there are concerns about the right ventricular incision, which can lead to ventricular dysfunction, and conduit stenosis.

[Bilateral pulmonary artery banding] Many Japanese institutions perform bilateral pulmonary artery banding as the first palliative procedure. It significantly reduces the risk of low birth weight, prematurity, cerebral hemorrhage, and complications of early cardiopulmonary bypass. It is expected to improve long-term outcomes, including neurodevelopment, by postponing open chest surgery during the neonatal period.

[Extracorporeal circulation] Partial cerebral perfusion is widely used in Japan. Complete circulatory arrest by hypothermia is most commonly used in North America and Europe, though the number of such procedures has grown in recent years. Lower body perfusion is also commonly used in Japan, but it has not become the standard method in other countries. Lower body blood perfusion is expected to reduce renal failure and shorten the intensive care unit stay.

Key words: hypoplastic left heart syndrome; Norwood operation; aortic reconstruction; pulmonary reconstruction; bilateral pulmonary banding

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