[5] Infants may exhibit severe cyanosis and right heart failure at birth, though these conditions may get better as pulmonary vascular resistance decreases.

[6] Although the precise cause of Uhl's anomaly is unknown, there have been reports of primary nondevelopment of myocytes, selective apoptosis, and cardiomyocyte overexpression of vascular endothelial growth factor.

[6] Global dyskinesia of a thin-walled right ventricle, absence of myocardium at the right ventricular free wall, lack of fibrofatty infiltration, normal tricuspid valve attachment to the right atrioventricular junction, and normal left ventricular myocardium are all indicative of cardiac magnetic resonance imaging findings.

[6] Histopathologically, nonfunctioning fibroelastic tissue replaces the myocardial layer, giving the right ventricular free wall a parchment-like appearance.

[12] When the pulmonary vascular resistance has become high and the newborn has severe cyanosis soon after birth, a brief intravenous prostaglandin E infusion is recommended.