Abstract
We present a case of isolated left ventricular noncompaction (LVNC), a severe congenital cardiomyopathy, which presented in the neonatal period as fetal hydrops. To our knowledge, this is the first child with LVNC presenting with hydrops fetalis to survive infancy. Once considered a uniformly fatal and extremely rare form of cardiomyopathy, LVNC has recently been shown to be more common than previously reported, with a varying range of clinical severity. Although long-term morbidity and mortality are not clearly known, recent work suggests better survivability than once reported.
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The authors acknowledge Dr. E. Lovett for his assistance with diagnosis and management.
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246_2009_9465_MOESM2_ESM.mov
Parasternal long axis view demonstrating impaired contractility and deep intertrabecular recesses continuous with the left ventricular cavity. (MOV 1052 kb)
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Richards, A., Mao, C.Y. & Dobson, N.R. Left Ventricular Noncompaction: A Rare Cause of Hydrops Fetalis. Pediatr Cardiol 30, 985–988 (2009). https://doi.org/10.1007/s00246-009-9465-7
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DOI: https://doi.org/10.1007/s00246-009-9465-7