Department of Cardiovascular Surgery, The Cardiovascular Institute, Tokyo, Japan.
Eur J Cardiothorac Surg. 2011 Aug;40(2):514-6. doi: 10.1016/j.ejcts.2010.12.018. Epub 2011 Jan 26.
A 29-year-old man was hospitalized because of heart failure causing dilated cardiomyopathy (DCM). On admission, he had elevated creatinine kinase levels (hyper CKemia) (4283IUl⁻) and false enlargement of bilateral calves. By a muscular biopsy, he was diagnosed as Fukuyama-type muscular dystrophy. Although neuromuscular diseases are often related to cardiomyopathy, reports showing a relation between cardiomyopathy and Fukuyama-type muscular dystrophy have been rare. Our group performed the partial left venticulectomy of the posterior wall and approximation of the papillary muscle, mitral valve annuloplasty, and tricuspid valve annuloplasty for DCM in the patient with Fukuyama-type muscular dystrophy, after obtaining informed consent from the patient and his family. At the 1-year follow-up examination, the neuromuscular symptoms had not progressed, and the left ventricular function was improved (left ventricular end-diastolic dimension (LVDd) 77-66 mm, left ventricular end-systolic dimension (LVDs) 73-59 mm, and ejection fraction (EF) 26-30%). This is the first case report of a left ventriculoplasty in a patient with Fukuyama-type muscular dystrophy.
一位 29 岁男性因心力衰竭导致扩张型心肌病（DCM）入院。入院时，他的肌酸激酶水平升高（高 CK 血症）（4283IU/L），双侧小腿假性增大。通过肌肉活检，他被诊断为 Fukuyama 型肌营养不良症。尽管神经肌肉疾病通常与心肌病有关，但报道显示心肌病与 Fukuyama 型肌营养不良症之间存在关联的情况很少见。我们的团队在获得患者及其家属的知情同意后，为该 Fukuyama 型肌营养不良症患者进行了 DCM 的部分左心室后壁切除术和乳头肌靠拢术、二尖瓣瓣环成形术和三尖瓣瓣环成形术。在 1 年的随访检查中，神经肌肉症状没有进展，左心室功能得到改善（左心室舒张末期内径（LVDd）77-66mm，左心室收缩末期内径（LVDs）73-59mm，射血分数（EF）26-30%）。这是首例 Fukuyama 型肌营养不良症患者进行左心室成形术的病例报告。
