The article on left ventricular noncompaction cardiomyopathy by Paterick et al. is superb, particularly the anatomic and echocardiographic images. My colleagues and I reported three fetal cases of this disorder as spongiform cardiomyopathy in Obstetrics and Gynecology , a journal that is not reviewed in PubMed and probably not read by most cardiologists. I would like to bring to the attention of the readers of JASE a few additional characteristics of left ventricular noncompaction cardiomyopathy echocardiographic findings in fetuses that illustrate the pathophysiology.
Two cases were familial, and all three patients were female, ruling out the Barth syndrome. (The mitochondria were normal.) All three patients in our report were autopsied. They all had severe hydrops, but the ejection velocity was only mildly diminished. There was no left ventricular enlargement in two patients, but the atria were enlarged in all three, indicating restrictive physiology rather than dilated cardiomyopathy. The right ventricle was also involved by noncompaction in all three patients, suggesting that the word left could be eliminated from the term left ventricular noncompaction cardiomyopathy . All three patients had intertrabecular recesses that were lined with endocardium, ruling out sinusoids such as occur in pulmonary atresia. Endocardial fibroelastosis was extensive with patchy fibrosis.