僧帽弁形成術および左室形成術にてカテコラミン依存性心不全から離脱し得た肢帯型筋ジストロフィー心筋症の1例

A Case of Cardiomyopathy Based on Limb-girdle Muscular Dystrophy in which Mitral Valvoplasty Together with Left Ventriculectomy was Relieved from Catecholamine-dependent Heart Failure

要旨　患者は53歳，女性．肢帯型筋ジストロフィーに伴う拡張型心筋症を背景に心不全の急性増悪にて入院加療を行うも，カテコラミン離脱が困難であった．長期経過中の左心機能低下はほぼ不変であったが，左室後壁基部の瘤状変化に伴う僧帽弁tetheringによる僧帽弁逆流の進行が主要な増悪要因と考え，僧帽弁形成術および後壁切除による左室形成術を施行した．術後はカテコラミン離脱に成功し，β遮断薬をはじめとする薬物療法の強化も可能となった．

筋ジストロフィーに関連する拡張型心筋症様心筋病変において，左室後壁瘤の切除による左室形成術が僧帽弁逆流の減弱への一助となった1例であり，その治療的意義を強調する．

　A fifty-three-year-old woman with dilated cardiomyopathy caused by limb-girdle type muscular dystrophy suffered from refractory congestive heart failure(HF)and was treated with an intravenous catecholamine infusion. Assessing her long-term course indicated by echocardiographic and clinical parameters, an aneurysmal formation of the posterior left ventricular(LV)wall leading to advanced mitral valve(MV)regurgitation caused by MV tethering in addition to impaired LV contraction was found and factors of HF exacerbation. Based on the above understanding, partial left ventriculectomy using posterior wall excision and mitral annuloplasty were designated. The operation succeeded in the catecholamine secession together with intensified medical treatment for HF.

　This is a representative case of muscular dystrophy/cardiomyopathy in which ventriculectomy of the aneurysmal portion and mitral annuloplasty was able to reduce MV regurgitation derived from MV tethering, leading to relief of exacerbated HF.