成人先天性心臓外科という新たな胸部外科領域―周術期管理と手術の実際

Adult Congenital Heart Surgery as a Novel Specialty in Thoracic Surgery; Perioperative Patient Management and Surgical Procedures

成人先天性心疾患（adult congenital heart disease：ACHD）は，先天性心疾患の手術成績の向上に伴い急激に患者数が増多している疾患群であるが，再手術治療介入を要する例は珍しくない．その一方で先天性心疾患手術の歴史はまだ浅く，ACHDの手術に関するエビデンスが十分に確立しているとはいえない．本稿では解剖および心機能が複雑なACHDに対して，手術介入を前提として手術および周術期管理の要点について論じ，遠隔期生命予後や生活の質を向上するための手術およびACHDハートチームのあり方について論じる．

Recently, number of adult congenital heart disease （ACHD） patients are rapidly increasing, but statistical evidences in adult congenital heart surgery are not sufficient because of its short history in addition to the complicated pathophysiology of ACHD. Therefore, a “heart team” including surgeons, pediatricians, and cardiologists are required, and blood flow imaging and 4-dimensional （4D） imaging plays an essential role in perioperative patient management.

Enhanced 3-dimensional computed tomography （3D-CT） scan is useful to determine cardiopulmonary bypass strategies in reoperation, and 4D flow magnetic resonance imaging （MRI） is useful in assessment of complicated hemodynamics. Diastolic function of systemic ventricle is very important in right side heart valve surgery or univentricular heart surgery, echocardiography is essential to examine the valve structure, and electrophysiological mapping is useful in concomitant arrhythmia surgery.

This article describes the indications, procedures, and perioperative managements of surgery in the right ventricular outflow tract, tricuspid valve, aortic root, Fontan circulation, and systemic right ventricle.