Neonatal Non-compacted Cardiomyopathy: Predictors of Poor Outcome

Abstract

Non-compacted cardiomyopathy (NCM) is a heterogenous myocardial disorder. Although much has been published in recent years, little is known about NCM in the neonatal period. The objective of this study is to characterize the involvement of newborns affected with NCM and to identify risk factors associated with increased mortality. This is a retrospective study including all neonates diagnosed with NCM between 2006 and 2018. Diagnosis was based on echocardiographic findings. Data were collected regarding prenatal history, gestational age and weight at birth, gender, age at diagnosis, left or biventricular involvement and associated malformations, medical and surgical treatments, and evolution. Fourteen patients were included. The median follow-up duration was 34 months (range 1–87 months). The left ventricular apex and lateral wall were involved in all cases (100%). Thirteen patients (92.8%) had other associated heart malformations. Six patients (42.8%) died during the follow-up period. Patients who had biventricular involvement and poor ventricular function presented a higher risk of death. The main cause of death was ventricular dysfunction (5/6 [83.3%]). During follow-up, eight patients (57.1%) underwent surgery for their cardiac malformations, without higher mortality. NCM must be included in the differential diagnosis of neonatal cardiomyopathy. The higher mortality observed in our series is related not only to the high association with congenital heart disease, but also to a greater presence of early and severe left ventricular dysfunction. We did not find that patients who underwent surgery with cardiopulmonary bypass had worse outcomes.