Double outlet right ventricle: an unusual variant with overriding of both great arteries, absent outlet septum and mitral-to-aortic-to-pulmonary valve continuity.
Double outlet right ventricle constitutes a spectrum of anomalies unified by the fact that both great arteries for their major part arise from the right ventricle. The cases are subcategorized on the basis of the relationship of the great arteries, the precise position of the ventricular septal defect and the muscular morphology of the outflow tracts. The spectrum extends into cases of double outlet left ventricle, while the "in between" cases have been catalogued as double outlet both ventricles. Two hearts are described with overriding of both great arteries, which on morphometric outlet septum and mitral-to-aortic-to-pulmonary valve continuity. To the best of our knowledge, this is the first report of this anatomical arrangement in double outlet right ventricle. Two cases with a similar arrangement have been documented as double outlet left ventricle and double outlet both ventricles. Our two cases further illustrate that the use of the segmental sequential approach to congenital heart disease caters for the most unusual specimens.
D001021:Aortic Valve / Q000002:abnormalities* / Q000473:pathology; D006325:Heart Atria / Q000473:pathology; D006345:Heart Septal Defects, Ventricular / Q000151:congenital* / Q000473:pathology; D006346:Heart Septum / Q000473:pathology; D006352:Heart Ventricles / Q000473:pathology; D006801:Humans; D007223:Infant; D008297:Male; D008943:Mitral Valve / Q000002:abnormalities* / Q000473:pathology; D011664:Pulmonary Valve / Q000002:abnormalities* / Q000473:pathology; D014188:Transposition of Great Vessels / Q000473:pathology*