Markovic N S, Dimkovic N, Damjanovic T, Loncar G, Dimkovic S
Cardiology Department, Zvezdara University Medical Center, Belgrade, Serbia.
Clin Nephrol. 2008 Jul;70(1):72-6. doi: 10.5414/cnp70072.
Isolated ventricular noncompaction is a rare congenital disorder characterized by the presence of numerous prominent trabeculations and deep intratrabecular recesses which communicate with the ventricular cavity. This disease has a very bad prognosis. Two cases of isolated ventricular noncompaction in patients with chronic renal failure have been described. The first case is a 65-year-old male, on regular hemodialysis for 3.5 years due to mesangioproliferative glomerulonephritis. He was symptomless regarding signs of congestive heart failure, angina pectoris, systemic embolization or arrhythmia. The second case is a patient with chronic renal failure (due to renal calculosis) admitted because of non-ST elevation acute myocardial infarction. In both cases echocardiography revealed an enlarged left ventricle, with extremely thickened walls with two layers: a thin, compacted myocardium on the epicardial side, and a thicker noncompaction endocardial layer. Ratio between noncompaction part of the wall and compaction part was 2.56 in the first and 4.94 in the second case. Blood flow from the left ventricular cavity into recesses was recorded with Color Doppler. Oral anticoagulation therapy was introduced in both of them. Holter ECG in the first patient revealed an intermittent right bandle branch block and in the second patient, premature ventricular contractions. Neurological examination findings were normal in both patients. Echocardiography of first-degree relatives was performed in the first case and it was normal in all 5 relatives. In the second case it was not performed due to technical reasons (relatives live abroad). Regular echocardiographic follow-up of all patients with chronic renal failure is necessary in order to diagnose cardiovascular comorbidities including this rare abnormality and its complications.
孤立性心室肌致密化不全是一种罕见的先天性疾病，其特征是存在大量突出的肌小梁和与心室腔相通的深陷肌小梁内隐窝。这种疾病预后很差。本文描述了两例慢性肾衰竭患者合并孤立性心室肌致密化不全的病例。第一例是一名65岁男性，因系膜增生性肾小球肾炎规律血液透析3.5年。他没有充血性心力衰竭、心绞痛、全身性栓塞或心律失常的症状。第二例是一名慢性肾衰竭患者（因肾结石病），因非ST段抬高型急性心肌梗死入院。两例患者的超声心动图均显示左心室增大，室壁极度增厚，分为两层：心外膜侧为薄的致密心肌层，心内膜侧为较厚的非致密层。第一例患者室壁非致密部分与致密部分的比例为2.56，第二例为4.94。彩色多普勒记录到左心室腔内血液流入隐窝。两例患者均采用口服抗凝治疗。第一例患者的动态心电图显示间歇性右束支传导阻滞，第二例患者出现室性早搏。两例患者的神经学检查结果均正常。第一例患者对其一级亲属进行了超声心动图检查，5名亲属结果均正常。第二例患者由于技术原因（亲属住在国外）未进行检查。对所有慢性肾衰竭患者进行定期超声心动图随访，对于诊断包括这种罕见异常及其并发症在内的心血管合并症很有必要。
