Double outlet right ventricle (DORV) is a congenital heart defect in which both the aorta and the pulmonary artery arise from the right ventricle, rather than the normal arrangement where the aorta arises from the left ventricle. Because the left ventricle has no great artery to pump into directly, blood from the left ventricle can only exit through a ventricular septal defect (VSD) into the right ventricle and thence into the great arteries.
DORV encompasses a spectrum of anatomy, and the clinical presentation depends critically on the location of the VSD and the relationship of the great arteries to each other. When the VSD is subaortic (beneath the aorta), oxygenated blood preferentially enters the aorta and the physiology resembles a large VSD. When the VSD is subpulmonary (beneath the pulmonary artery), oxygenated blood enters the pulmonary artery and the physiology resembles transposition of the great arteries. With pulmonary stenosis present, the physiology can resemble tetralogy of Fallot.
Surgical repair of DORV aims to create a pathway (typically an intraventricular tunnel) connecting the left ventricle to the aorta, so that oxygenated blood is directed into the systemic circulation. The technical approach and outcomes depend on the specific anatomy; in some cases the arterial switch operation or complex conduit reconstructions are required.
Adults with repaired DORV are followed up in adult congenital heart disease (ACHD) clinics. They may have residual lesions such as VSD patch leak or conduit obstruction, and are at increased risk of arrhythmia. Ongoing surveillance with echocardiography and cardiac MRI is standard.
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