妊娠後期に僧帽弁腱索断裂によるうっ血性心不全を発症したMarfan症候群の1例

Abstract

We report a rare case of Marfan syndrome associated with congestive heart failure due to ruptured chordae tendineae in late pregnancy. A 29-year-old woman, who was 32 weeks pregnant, was admitted to our hospital with complaints of dyspnea on exertion. Although she had had severe myopia, she was never found to have a cardiac murmur. On physical examination, arachnodactyly was revealed, and both the wrist sign and the thumb sign were positive. A chest radiography showed bilateral pleural effusion, cardiomegaly, and scoliosis. Echocardiography showed mitral valve prolapse (MVP) with ruptured chordae tendineae and severe mitral regurgitation. The patient was diagnosed as having Marfan syndrome with congestive heart failure due to ruptured chordae tendineae. She was treated with diuretics and a daily continuous intravenous drip of vasodilator (ISDN, 3.6 mg/h), dopamine (5.0μg/kg/min) and dobutamine (10.0 μg/kg/min). Due to the appearance of pulmonary edema on the 5th hospital day, positive end-expiratory pressure (PEEP) and an extracorporeal ultrafiltration method (ECUM) were performed. Although an emergency cesarean section was performed on the 6th hospital day, congestive heart failure and low-grade fever persisted. On the 27th hospital day, mitral valve replacement (MVR) was performed. Subsequently, the patient's congestive heart failure gradually improved. Histological analysis of the mitral valve extracted on MVR revealed muciform changes and infiltration of inflammatory cells in the mitral valve leaflets. These findings are consistent with changes of mitral valve leaflet related to the Marfan syndrome having both mitral valve prolapse and infectious endocarditis.