左室緻密化障害を伴う僧帽弁閉鎖不全症に対して僧帽弁置換術および心臓再同期療法を施行した1例

Mitral Valve Replacement and Cardiac Resynchronization Therapy for Mitral Regurgitation with Left Ventricular Noncompaction：Report of a Case

はじめに　左室緻密化障害（LVNC）は，胎生期における心筋の緻密化過程が途中で停止してスポンジ状の胎児心筋が遺残した病態と考えられ，心室壁の過剰な網目状の肉柱形成と深い間隙を形態的特徴とする先天性心筋症の一つである1～8）．われわれは成人のLVNC合併僧帽弁閉鎖不全症（MR）に対して，僧帽弁置換術（MVR）および心臓再同期療法（CRT-P）を施行したので報告する．

Left ventricular noncompaction （LVNC） is a rare congenital abnormality resulting from an arrest of the normal process of myocardial compaction. LVNC has been known as a disease of infants, however, some surgical cases of LVNC in adult have been reported.

A 61-year-old man who was diagnosed as dilated cardiomyopathy due to LVNC was admitted to our hospital because of dyspnea. Echocardiography revealed severe mitral regurgitation and diffuse left ventricular hypokinesis, and the hypokinetic wall consists of a thin compacted epicardial layer and a thicker noncompacted endocardial layer. Mitral valve replacement using a mechanical valve and cardiac resynchronization therapy were performed. The patient was easily weaned from cardiopulmonary bypass with medium-dose inotropic support and scheduled intra-aortic balloon pumping （IABP）. Postoperative course was uneventful, and he was discharged in good condition on the 29th postoperative day.