Absence of the left ventricular myocardium in a newborn

Abstract

SummaryAbsence of myocardial fibers in the right ventricle is the essence of so-called Uhl's anomaly, which should be distinguished from the fatty replacement producing arrhythmogenic right ventricular dysplasia of the adolescent and young adult. In this report, we describe a newborn with nearly complete absence of the myocardium of the left ventricle. The infant died on the seventh day because of myocardial incompetence of the left ventricle, which was unable to open the aortic valve.

Unguarded mitral orifice, mirror-imaged atrial arrangement, and discordant atrioventricular connections

“…A single case of Uhl's disease of the left ventricle has been described, again with normal atrioventricular and arterial valves. 8 Our cases, therefore, are the first reported cases of unguarded mitral orifice with absence of the leaflets and tension appratus of the mitral valve. Interestingly, both cases had discordant atrioventricular connection, with double outlet right ventricle in the setting of mirror-imaged atrial arrangement It is possible to find similar, but far from the same, anatomical features in the rare setting of Ebstein's malformation of the morphologically mitral valve.…”