富松 宏文

Hirofumi TOMIMATSU

東京女子医科大学循環器小児科

Department of Pediatric Cardiology, Tokyo Women’s Medical University

キーワード : adult congenital heart disease, echocardiography, segmental approach, named operation, palliative operation

循環器疾患の診療において心エコー検査は非侵襲的に多くの情報を得ることができるものであり，欠くことのできない検査である．一方，先天性心疾患（congenital heart disease: CHD）に対する心臓手術成績は飛躍的に向上し多くのCHD患者が成人期に達するようになり成人CHD患者（adult congenital heart disease: ACHD）は既に40万人に達している．これら多くのACHDが，生活習慣病や妊娠および心臓機能障害などに直面する機会も増加しつつある．しかし，ACHDの内科的診療に携わる可能性のある小児循環器科医は少ない．したがって，ACHDの心エコー検査の知識は内科医・検査技師にとっても必要なものである．ACHDでは複雑な心形態を示すことが多いうえに，音響窓の制限が多いため心エコー検査だけではその心形態を正確に評価することが困難なことがある．しかし，区分診断法を用いてその基本形態を評価するとともに，その状態ごとに問題点を整理把握して検査を進めることにより病態を把握することは可能である．ACHDの心エコー検査では原疾患の形態的特徴に加え，自然歴，手術術式，術後歴（合併症，残遺症，続発症）などの知識を整理しておくことが重要である．

Echocardiography is an essential examination in the diagnosis and treatment of cardiovascular disease as it noninvasively yields much information. In Japan, there has been an estimated increase of 9,000 adults with congenital heart disease every year from 1997 to 2007. In 2007, there were estimated to be about 409,101 adults with congenital heart disease (ACHD) in Japan. An increasing number of those with ACHD face lifestyle-related diseases, pregnancy-related problems, and cardiac dysfunction. However, there are few pediatric cardiologists engaged in the medical treatment of those with ACHD. Therefore, knowledge of echocardiography of ACHD is necessary for physicians and laboratory technicians. Although adults with congenital heart disease share the basic cardiac anatomical anomalies and physiology with the pediatric population, cardiac structures grow and evolve with the patients. Structural changes occur following surgical palliation and repair. Even without intervention during infancy and childhood, progression into adulthood can bring with it changes in ventricular mass, calcification or dysplasia of valves, and myocardial fibrosis. Many of these patients face further operations and interventions. They require lifelong medical care. Echocardiography is an essential tool for the diagnosis and follow-up of adults with congenital heart disease. In adults, it is often difficult to obtain much information with echocardiography, because adults with congenital heart disease have limited acoustic windows. However, it is possible to evaluate the primary form of ACHD by using a segmental approach, where it is possible to grasp the pathological state by identifying the issues involved in each of its states as the examination proceeds. In echocardiography of adults with congenital heart disease, it is important to organize the knowledge of the morphological characteristics of congenital heart disease, natural history, surgical methods, and postoperative history (complications, residuae, sequelae).