double outlet right ventricle


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dou·ble out·let right ven·tri·cle

a heterogeneous category of congenital abnormalities as yet unclassified. Basically both great arteries arise in whole or in part from the right ventricle or an infundibular chamber. Ventricular septal defect is nearly always present.
References in periodicals archive ?
Holt-oram syndrome associated with double outlet right ventricle: A rare association.
Double outlet right ventricle with complete AV canal is uncommon and the VSD in this instance is usually subaortic.8 Our case shows a DORV with a complete AV canal defect a non-committed type of VSD accompanied by pulmonary stenosis and tricuspid regurgitation.
For example, in a child with double outlet right ventricle (DORV), both great arteries leave the right heart (with an obligate VSD).
Double outlet right ventricle (DORV) is a group of complex congenital heart abnormalities, in which both the pulmonary artery and the aorta arise primarily from the right ventricle.[sup][1] These two great arteries may override the ventricular septum by more than 50%, 90%, or both arteries may arise fully from the right ventricle (200%).[sup][2],[3] DORV is frequently associated with other intracardiac or extracardiac malformations.
Rotational abnormalities of the conotruncus such as arrest of the rotation in experimental animals, produces a double outlet right ventricle. The transposition of the great arteries is caused by arrested conotruncus rotation.
Legend: Complete AVSD=Complete atrio ventricular septal defect; DILV=Double Inlet Left Ventricle; DORV=Double Outlet Right Ventricle; TGA+DILV=Transposition of great arteries and double inlet left ventricle; TGA+DORV= Transposition of great arteries and double outlet Right ventricle; PUL ARESIA=Pulmonary atresia
Indications included: Pulmonary atresia with ventricular septal defect (n=11), Tetralogy of Fallot with absent Pulmonary Valve (PV) syndrome (n=2), double outlet right ventricle, transposition of great arteries and pulmonary stenosis (n=1), isolated aortic valve disease (n=1) and a pseudo-aneurysm with infective endocarditis (n=1).Conduit sizes varied between 16-22 mm.
Patients with right atrial isomerism have also frequently some anomalies like complete atrioventricular canal defect, transposition of the great arteries and double outlet right ventricle. On the other hand, patients with left atrial isomerism have defects in the intrahepatic portion of the inferior vena cava and heart block (4).
Visceroatrial situs solitus with atrioventricular alignment discordance double outlet right ventricle and superoinferior ventricles: fetal and neonatal echocardiographic findings.
Complete thoracic ectopia with double outlet right ventricle: neonatal repair.
Double outlet right ventricle (DORV) represents a continuum of congenital heart diseases that ranges from VSD with significant override of the aorta to a common arterial trunk arising completely from the right ventricle.