Alizadeh-Sani Zahra, Madadi Shabnam, Sadeghpour Anita, Khajali Zahra, Golnari Pedram, Kiavar Majid
Shaheed Rajaei Cardiovascular, Medical and Research Center, Tehran University of Medical Sciences, Tehran, Iran.
J Tehran Heart Cent. 2011 Nov;6(4):214-6. Epub 2011 Nov 30.
Left ventricular non-compaction cardiomyopathy is a rare congenital cardiomyopathy that affects both children and adults. Since the clinical manifestations are not sufficient to establish diagnosis, echocardiography is the diagnostic tool that makes it possible to document ventricular non-compaction and establish prognostic factors. We report a 47-year-old woman with a history of dilated cardiomyopathy with unknown etiology. Echocardiography showed mild left ventricular enlargement with severe systolic dysfunction (EF = 20-25%). According to cardiac magnetic resonance imaging findings non-compaction left ventricle with hypertrophic cardiomyopathy was considered, and right ventricular septal biopsy was recommended. Right ventricular endomyocardial biopsy showed moderate hypertrophy of cardiac myocytes with foci of myocytolysis and moderate interstitial fibrosis. No evidence of infiltrative deposition was seen.
左心室致密化不全心肌病是一种罕见的先天性心肌病，可累及儿童和成人。由于临床表现不足以确诊，超声心动图是能够记录心室致密化不全并确定预后因素的诊断工具。我们报告一名47岁女性，有病因不明的扩张型心肌病病史。超声心动图显示左心室轻度扩大，伴有严重收缩功能障碍（射血分数=20%-25%）。根据心脏磁共振成像结果，考虑为肥厚型心肌病合并左心室致密化不全，并建议进行右心室间隔活检。右心室心内膜活检显示心肌细胞中度肥大，伴有心肌溶解灶和中度间质纤维化。未见浸润性沉积的证据。
