The Journal of the Oklahoma State Medical Association
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J Okla State Med Assoc · Apr 2016
Case ReportsA Rare Association of Non-Compaction of the Ventricular Myocardium, and Non-Immune Hydrops Fetalis.
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. ⋯ 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.