ABSTRACT
Non-compaction of the ventricular myocardium(NVM), also known as cavernous cardiomyopathy, is dominated by left ventricular non-compaction(LVNC)in most patients.The incidence of NVM in childhood cardiomyopathy is second only to dilated cardiomyopathy and hypertrophic cardiomyopathy, and the pathological changes are prominent trabeculae and deep intertrabecular clefts.Most pediatric patients have serious complications, such as heart failure, serious arrhythmias, and thrombus.Therefore, it is particularly important to study the pathogenesis of LVNC in children.NVM may be caused by the cessation of myocardial compaction in early embryonic development, so the genetic pathogenesis of NVM is particularly important.This review aimed to elucidate the genetic pathogenesis of LVNC in children, and provide more ideas for the pathogenesis and genetic therapy of LVNC in the future.
ABSTRACT
Non-compaction of the ventricular myocardium(NVM), also known as cavernous cardiomyopathy, is dominated by left ventricular non-compaction(LVNC)in most patients.The incidence of NVM in childhood cardiomyopathy is second only to dilated cardiomyopathy and hypertrophic cardiomyopathy, and the pathological changes are prominent trabeculae and deep intertrabecular clefts.Most pediatric patients have serious complications, such as heart failure, serious arrhythmias, and thrombus.Therefore, it is particularly important to study the pathogenesis of LVNC in children.NVM may be caused by the cessation of myocardial compaction in early embryonic development, so the genetic pathogenesis of NVM is particularly important.This review aimed to elucidate the genetic pathogenesis of LVNC in children, and provide more ideas for the pathogenesis and genetic therapy of LVNC in the future.
ABSTRACT
Background: Non compaction cardiomyopathy is a rare disorder caused by the arrest of myocardial compaction during embryogenesis, leading to a non compacted endocardial layer with marked hypertrabeculation and deep recesses. Aim: To report the clinical and echocardiographic characteristics of a series of 15 adult patients with non-compaction cardiomyopathy. Patients and Methods: We included a total of 15 patients aged 52 ± 17 years (40 percent males) diagnosed at our echocardiography laboratory between January 2001 and July 2010. Results: Theform of presentation was heart failure in 53 percent of subjects, syncope in 20 percento, ventricular arrhythmias in 13 percento and stroke in 7 percent>. Left ventricular end-diastolic diameter was 66 ±11 mm and estimated ejection fraction was 27 ± 10 percent>. Apical and/or mid-ventricular segments of the left ventricle were involved in all the cases. Pulmonary hypertension was present in 40 percento. The average follow-up was 19 months and no patient died during this period. Sixty seven percent ofthe patients had manifestations of heart failure, 27 percento presented sustained ventricular arrhythmias and 20 percent> had atrial fibrillation orflutter, whereas 13 percento had cerebral embolic events. An automated internal cardioverter defibrillator was implanted in 47 percento of patients. Conclusions: Non-compaction cardiomyopathy is associated with high cardiovascular morbidity. The diagnosis is made in advanced stages of the disease, with significant dilation and ventricular dysfunction.