大人になった先天性心疾患

DOI: 10.3179/jjmu. JJMU.R.51
◇ REVIEW ARTICLE（第 13 回教育セッション）
（循環器）◇ 大人になった先天性心疾患
富松宏文
抄　録
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．
Adult congenital heart disease
Hirofumi TOMIMATSU
Abstract
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）.
Keywords
adult congenital heart disease, echocardiography, segmental approach, named operation, palliative operation
1．はじめに
循環器疾患の診療の場において心エコー検査は聴
診器と同じくらいに身近なものであり，かつ非侵襲
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
東京女子医科大学循環器小児科
Department of Pediatric Cardiology, Tokyo Women’s Medical University, 8︲1 Kawada, Shinjukuku, Tokyo 162︲8666, Japan
Received on February 9, 2015; Accepted on February 26, 2015 J-STAGE. Advanced published. date: April 20, 2015
Jpn J Med Ultrasonics

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