Objective To investigate the clinical characteristics of isolated non-compaction cardiomyopathy （NCCM） in children. Methods Clinical data of 13 NCCM children were analyzed retrospectively. Results There was no manifestations of embolism presented in all the 13 NCCM children, but eight of them had obvious cardiac dysfunction, 12 had cardiac arrhythmia, of whom, four had premature ventricular beats. Typical echocardiographic changes were observed in all the 13 children, of whom, 12 were involved in the left ventricle alone, both ventricles involved in one; left ventricular enlargement was seen in nine, eight with decreased left ventricular ejection fraction, and nine bad an increased pulmonary artery systolic pressure. During 0.5--4.4-year fol- low-up, sudden death was noted in one case, two died of intractable heart failure; and three repeated hospitalization due to heart failure. Conclusion Cardiac dysfunction and arrhythmia are frequently observed in children with NCCM, and embolism is rare. Ultrasound cardiography is of great value in the diagnosis of NCCM a disease of poor prognosis.
Acta Academiae Medicinae Qingdao Universitatis
isolated non-compaction cardiomyopathy