背景:左心室致密化非心肌病(LVNC)是一种罕见的先天性心肌病,其特征是心室的一个或多个节段的小梁形成增加。LVNC表现为非特异性症状和高度可变的临床表现,从无症状到进行性心力衰竭以及复发性或危及生命的心律失常。
方法:54岁的黑人有高血压病史,糖尿病和终末期肾病在透析后出现一天的心悸和头昏眼花。他否认有任何呼吸困难或晕厥。在检查中,血压为175/91mmHg,脉搏不规则。没有杂音,摩擦或疾驰受到赞赏。除肌酐升高和轻度贫血且甲状腺功能正常外,实验室均无明显变化。心电图(ECG)显示房颤,心室率正常。经胸超声心动图显示左心室(LV)壁厚度轻度增加,明显的小梁形成和射血分数为55-60%,伪正常左心室充盈模式,伴随着异常松弛和增加的填充压力,暗示LVNC。患者改用阿哌沙班。建议对家庭成员进行基因检测。
结论:LVNC是罕见的具有非特异性症状的先天性心肌病,应作为出现心律失常患者的可能诊断之一。超声心动图和心脏磁共振成像可用于建立诊断。
BACKGROUND: Left ventricular non-compaction cardiomyopathy (LVNC) is a rare congenital cardiomyopathy characterized by increased
trabeculation in one or more segments of the ventricle. LVNC presented with non-specific symptoms and highly variable clinical presentation ranging from asymptomatic to progressive heart failure and recurrent or life-threatening arrhythmias.
METHODS: 54-year-old Black man with a history of hypertension, diabetes and end-stage renal disease presented with one day palpitations and lightheadedness following a dialysis session. He denied any dyspnea or syncope. On examination, blood pressure was 175/91 mmHg with irregular pulse. No murmur, rubs or gallops were appreciated. Laboratory were unremarkable except increased creatinine and mild anemia with normal thyroid function test. Electrocardiogram (ECG) revealed atrial fibrillation with normal ventricular rate. Transthoracic echocardiogram revealed mildly increased left ventricular (LV) wall thickness with prominent
trabeculation and ejection fraction of 55-60 percent, a pseudo-normal LV filling pattern, with concomitant abnormal relaxation and increased filling pressure, suggestive of LVNC. The patient was switched to apixaban. Genetic testing was recommended for family members.
CONCLUSIONS: LVNC is rare congenital cardiomyopathy with non-specific symptoms and should be considered among the possible diagnosis in patients presenting with arrythmia patients. Echocardiographic and cardiac magnetic resonance imaging can be utilized to establish diagnosis.