Craiova Cardiology Center - EuroEchoLab, Craiova, Romania.
Craiova Cardiology Center - Cardiology Department, Craiova, Romania.
J Med Case Rep. 2021 Jun 8;15(1):316. doi: 10.1186/s13256-021-02862-x.
Left ventricular noncompaction is a rare cardiomyopathy characterized by a thin, compacted epicardial layer and a noncompacted endocardial layer, with trabeculations and recesses that communicate with the left ventricular cavity. In the advanced stage of the disease, the classical triad of heart failure, ventricular arrhythmia, and systemic embolization is common. Segments involved are the apex and mid inferior and lateral walls. The right ventricular apex may be affected as well.
A 29-year-old Caucasian male was hospitalized with dyspnea and fatigue at minimal exertion during the last months before admission. He also described a history of edema of the legs and abdominal pain in the last weeks. Physical examination revealed dyspnea, pulmonary rales, cardiomegaly, hepatomegaly, and splenomegaly. Electrocardiography showed sinus rhythm with nonspecific repolarization changes. Twenty-four-hour Holter monitoring identified ventricular tachycardia episodes with right bundle branch block morphology. Transthoracic echocardiography at admission revealed dilated left ventricle with trabeculations located predominantly at the apex but also in the apical and mid portion of lateral and inferior wall; end-systolic ratio of noncompacted to compacted layers > 2; moderate mitral regurgitation; and reduced left ventricular ejection fraction. Between apical trabeculations, multiple thrombi were found. The right ventricle had normal morphology and function. Speckle-tracking echocardiography also revealed systolic left ventricle dysfunction and solid body rotation. Abdominal echocardiography showed hepatomegaly and splenomegaly. Abdominal computed tomography was suggestive for hepatic and renal infarctions. Laboratory tests revealed high levels of N-terminal pro-brain natriuretic peptide and liver enzymes. Cardiac magnetic resonance evaluation at 1 month after discharge confirmed the diagnosis. The patient received anticoagulants, antiarrhythmics, and heart failure treatment. After 2 months, before device implantation, he presented clinical improvement, and echocardiographic evaluation did not detect thrombi in the left ventricle. Coronary angiography was within normal range. A cardioverter defibrillator was implanted for prevention of sudden cardiac death.
Left ventricular noncompaction is rare cardiomyopathy, but it should always be considered as a possible diagnosis in a patient hospitalized with heart failure, ventricular arrhythmias, and systemic embolic events. Echocardiography and cardiac magnetic resonance are essential imaging tools for diagnosis and follow-up.
左室心肌致密化不全是一种罕见的心肌病，其特征为心外膜层薄且致密，心内膜层非致密，有小梁和隐窝与左心室腔相通。在疾病的晚期，心力衰竭、室性心律失常和全身栓塞这一经典三联征较为常见。受累节段为心尖部及中下、外侧壁。右室心尖部也可能受累。
一名 29 岁白人男性，在入院前的最后几个月，于轻微活动时出现呼吸困难和疲劳，还自述在最近几周出现腿部水肿和腹痛。体格检查发现呼吸困难、肺部啰音、心脏扩大、肝大、脾大。心电图显示窦性心律，伴有非特异性复极改变。24 小时动态心电图监测发现右束支传导阻滞形态的室性心动过速发作。入院时的经胸超声心动图显示左心室扩张，心尖部有小梁，但也见于外侧壁和下壁的心尖部和中部；非致密化层与致密化层的收缩末期比值＞2；中度二尖瓣反流；左心室射血分数降低。在心尖部小梁之间发现多个血栓。右心室形态和功能正常。斑点追踪超声心动图也显示左心室收缩功能障碍和实体旋转。腹部超声心动图显示肝大、脾大。腹部 CT 提示肝、肾梗死。实验室检查显示 N 末端脑利钠肽前体和肝酶水平升高。心脏磁共振评估在出院后 1 个月时确诊。患者接受抗凝、抗心律失常和心力衰竭治疗。2 个月后，在植入除颤器之前，患者出现临床改善，且超声心动图评估未发现左心室血栓。冠状动脉造影正常。植入心脏除颤器以预防心源性猝死。
左室心肌致密化不全是一种罕见的心肌病，但对于因心力衰竭、室性心律失常和全身栓塞事件而住院的患者，应始终考虑将其作为可能的诊断。超声心动图和心脏磁共振是诊断和随访的重要影像学工具。
