Left-Ventricular Noncompaction in a Pediatric Population: Predictors of Survival

Abstract

Left-ventricular noncompaction (LVNC) is an echocardiographic finding of increasing frequency in pediatrics; however, predictors of outcomes have been difficult to identify. We conducted a retrospective review of pediatric patients at the Morgan Stanley Children's Hospital of New York from January of 1993 to September of 2009 to identify predictors of the primary outcome of death or heart transplantation. LVNC was identified in 50 patients, 34 of them < 1 year of age. Death or transplantation occurred in 26 patients, with a median survival of 1.17 years after presentation. Patients surviving 1 year after presentation had 75% conditional survival, and patients surviving 2 years after presentation had 92% conditional survival. Hemodynamic instability, poor ventricular function, and LV dilatation were each independent predictors of poor outcome. Of the 21 patients who presented with hemodynamic instability, 17 died or underwent transplantation at a median of 0.08 years after presentation. In conclusion, LVNC is recognized more in younger patients; however, age is not a predictor of outcome. Patients who present with hemodynamic instability and poor ventricular function have decreased transplant-free survival, and most poor outcomes occur within the first year after presentation. Therefore, early listing for transplant may lead to better outcomes in this population.

The Impact of Concomitant Left Ventricular Non-compaction with Congenital Heart Disease on Perioperative Outcomes

“…Previous studies have shown that LVNC appears to be more commonly associated with right-sided disorders including Ebstein's anomaly and tricuspid atresia. Less frequently it has been reported among patients with aortic coarctation, tetralogy of Fallot, and bicuspid aortic valves, as well as septal defects [7,9,11,12]. The present study expands upon these previous findings by noting that the distribution of CHD lesions was diverse and included many congenital heart defects that have not been routinely associated with LVNC, such as coarctation of the aorta, double inlet left ventricle, and ventricular septal defects.…”

“…Criteria for diagnosis varies in the literature, but most agree, to make the diagnosis, there must be prominent trabeculations and inter-trabecular recesses that communicate with the left ventricular cavity [2,8]. Myocardial abnormalities consistent with LVNC cardiomyopathy have been reported in association with some forms of structural CHD [7,[9][10][11][12][13][14].…”

Cardiomyopathy Phenotypes and Outcomes for Children With Left Ventricular Myocardial Noncompaction: Results From the Pediatric Cardiomyopathy Registry

“…A more recent retrospective review of 50 children with LVNC found 23 (46%) also had DCM. 28 Death or transplant occurred in 16 (59%) of the children with LVNC and DCM; half of the entire group of 50 children died within 1.2 years of presentation. Time to death or transplant was not reported for the LVNC with DCM subgroup.…”

Pediatric Cardiomyopathies: causes, epidemiology, Clinical course, Preventive Strategies and Therapies

“…Although the cause of this arrest is unknown, LV noncompaction has been associated with other congenital heart defects. Zuckerman et al found 13 out of 58 (22%) patients with LV noncompaction had another form of congenital heart defect, most commonly ventricular septal defects (seven out of 13), followed by secundum atrial septal defects (three out of 13) [94]. Other abnormalities associated with LV noncompaction include Ebstein’s anomaly, hypoplastic right ventricle and hypoplastic left heart syndrome [81].…”