Author + information
- Received August 27, 2010
- Revision received September 15, 2010
- Accepted September 21, 2010
- Published online August 16, 2011.
In Uhl's anomaly, a thin right ventricle (RV) anterior free wall is the characteristic finding on echocardiogram and cardiac magnetic resonance imaging (A, B, Online Videos 1, 2, and 3) (1,2). We describe for the first time the abnormal physiology associated with this condition. The inferior vena cava is dilated, showing little respiratory variation, and there is atrial wave reversal in hepatic veins during inspiration (C). The pulmonary venous Doppler flow shows marked respiratory variation, with persistent antegrade flow over the cardiac cycle in inspiration (D). Forward flow in the right ventricular outflow and pulmonary circulation is dependent entirely on atrial contraction, akin to atriopulmonary Fontan circulation (E). There is free pulmonary regurgitation that is more prominent during inspiration. That is probably due to the expansion of an “atonic” infundibulum during atrial contraction with increased pre-load, despite the presence of a normal pulmonary valve (F, G), thus emphasizing the role of the infundibulum in maintaining pulmonary valve competence. LV = left ventricle; RVOT = right ventricular outflow tract; TV = tricuspid valve.
- Received August 27, 2010.
- Revision received September 15, 2010.
- Accepted September 21, 2010.
- American College of Cardiology Foundation