A Rare Association of Non-Compaction of the Ventricular Myocardium, and Non-Immune Hydrops Fetalis.

Abstract

Hydrops fetalis is a rare manifestation of severe congestive heart failure in a fetus, resulting in pathological fluid accumulation in fetal soft tissues and/or serous cavities. Non-compaction of the ventricular myocardium, frequently referred to as the left ventricular non-compaction (LVNC), is a very rare congenital cardiomyopathy. For LVNC, echocardiography is the diagnostic modality of choice. Various diagnostic criteria exist based on either echocardiography or MRI. Currently, nonimmune hydrops fetalis (NIHF) comprise almost 90% of all hydrops fetalis cases. Among cardiovascular conditions that contribute to NIHF (20%), structural malformations (especially hypoplastic left heart, endocardial cushion defect) and arrhythmias are the most frequent etiological factors. We describe an extremely preterm neonate who presented with features of non-Immune hydrops fetalis (NIHF) associated with LVNC and profound refractory systemic hypotension.