Double inlet left ventricle (DILV) describes a congenital cardiac anomaly in which both atrioventricular valves are associated with a single ventricle which demonstrates left ventricular morphology.
This uncommon entity constituents 1% of all congenital cardiac anomalies, and is one of the more common variants of a univentricular heart 1.
Features include a single, dominant ventricle with an elliptical shape, smooth septal endocardium lacking associated papillary muscles, defining left ventricular morphology 3. Atrioventricular valves may lack sufficient anatomical features to define their morphology in mitral/tricuspid valves, and are commonly stenotic or hypoplastic. A rudimentary outflow chamber (right ventricle) may be identified at the cardiac base. The location of the septum may be left/anterior, defining the L-loop orientation, or right/anterior defining the less common D-loop orientation. Further classification is based upon the relation of the great arteries 1:
- type I
- normal arterial relations
- type II
- rightward/anterior aortic location
- type III
- leftward/anterior aortic location
- type IV
- leftward/posterior aortic location
- 1. Paul Khairy, Nancy Poirier, Lise-Andrée Mercier. Univentricular Heart. (2007) Circulation. 115 (6): 800-12. doi:10.1161/CIRCULATIONAHA.105.592378 - Pubmed
- 2. Bellsham-Revell H, Masani N. EDUCATIONAL SERIES IN CONGENITAL HEART DISEASE: The sequential segmental approach to assessment. (2019) Echo research and practice. 6 (1): R1-R8. doi:10.1530/ERP-17-0039 - Pubmed
- 3. Wyman W. Lai, Luc L. Mertens, Meryl S. Cohen, Tal Geva. Echocardiography in Pediatric and Congenital Heart Disease. (2016) ISBN: 9780470674642