Clinical Outcomes for Children with Left Ventricular Noncompaction and Cardiomyopathy

Purpose Left ventricular noncompaction (LVNC) is a rare cardiomyopathy; its clinical presentations and prognosis in children remain debated. Although a few reports have proposed that LVNC with cardiomyopathy has a poor prognosis, the reason for this is unknown. This study compared LVNC with and without cardiomyopathy and identified the risk factors for poor prognosis of LVNC. Methods Twenty patients aged 2 on echocardiography. Combined cardiomyopathy was diagnosed on echocardiography. Patients were divided into two groups: LVNC with and without cardiomyopathy. We retrospectively reviewed the clinical course, cardiac function that was measured on echocardiography, and cardiac catheterization if surgical intervention was needed. Results Eleven of the 20 children were diagnosed with LVNC with cardiomyopathy: five with dilated cardiomyopathy (DCM), five with restrictive DCM, and one with restrictive cardiomyopathy. In nine children without cardiomyopathy, five had congenital heart disease and four had isolated LVNC. Age at diagnosis did not differ significantly. At the time of diagnosis, heart failure was present in seven children (63%) with cardiomyopathy and in one (11%) without cardiomyopathy. Ten (91%) patients with cardiomyopathy needed a left ventricular assist device (VAD); clinical outcomes were heart transplantation in six, VAD explantation in two, and continued device use in three patients. Children with LVNC without cardiomyopathy were all followed up as outpatients. In LVNC with cardiomyopathy, the mean LV ejection fraction (LVEF) on echocardiography was 36.9% at diagnosis and 33.5% before VAD implantation (p=0.6). On cardiac catheterization, the mean pulmonary capillary wedge pressure (PCWP) was 13.2 mmHg at diagnosis and 22.2 mmHg before VAD implantation (p Conclusion All children with LVNC with cardiomyopathy needed VAD support or heart transplantation. Those without cardiomyopathy were seen as outpatients and had good cardiac function. PCWP increased over time in the cases of cardiomyopathy. Hence, diastolic function could be associated with the prognosis.