Double outlet right ventricle: A partial transposition complex

Abstract

Three anatomic variations of a partial transposition complex characterized by pulmonary and systemic arterial circuits arising entirely from the right ventricle are described. Clinical data is presented in the first, the “Eisenmenger type,” in which the heart revealed coarctation and complete transposition of the aorta, large pulmonary artery in normal position, ventricular septal defect, and patent ductus arteriosus. The clinical picture of this syndrome is characterized by slowly developing cyanosis, a parasternal systolic murmur, occasionally a diastolic murmur and signs of aortic coarctation. Survival may reach the second decade. Fluoroscopy reveals plethoric lung fields, combined ventricular enlargement, and a wide supracardiac shadow in the anteroposterior and left oblique views. Another example of this category was complicated by absence of the aortic arch. It was associated with a small separate ascending aorta, absence of the aortic arch, and descending aorta supplied by a patent ductus. All vessels arose from the right ventricle. Survival past the age of 6 years has not been recorded in this type. The second principal variation of the “double outlet” right ventricle is the Fallot type. It resembles the tetralogy of Fallot because of various degrees of pulmonary hypoplasia. Biventricular enlargement, however, suggests that one is not dealing with a true tetralogy, and angiocardiography may demonstrate the exact relationship of the septal defect to aortic and pulmonary orifices.