Double Outlet Right Ventricle: A Rare Finding in a 15-Month-Old Female With Failure to Thrive

Abstract

Double outlet right ventricle (DORV) is a rare congenital heart defect where both the aorta and pulmonary artery originate from the right ventricle, often accompanied by additional cardiac anomalies to mitigate circulatory imbalance, though such compensations usually fail. We report a 15-month-old infant with recurrent respiratory infections and poor weight gain, referred for computed tomography angiography. Physical examination showed a small, non-syndromic infant with pallor, tachypnea, irritability, and finger clubbing. Initial imaging revealed cardiomegaly and lung infiltrates; echocardiography and computed tomography angiography confirmed additional intracardiac defects of double superior vena cavae, a hypoplastic aortic arch, hypertrophic right ventricular wall, and a patent ductus arteriosus. Corrective surgery was delayed due to respiratory complications. This case emphasizes the critical need to consider cardiac pathology in pediatric patients with recurrent respiratory symptoms, as untreated DORV can lead to high mortality.

Keywords: cardiac computed tomography; case report; congenital heart defect; double outlet right ventricle; echocardiography.

FIGURE 1

An AP frontal radiograph showing an enlarged cardiac shadow, cardiothoracic ration of 0.7 and widened superior mediastinum, patchy rounded opacities in the para‐cardiac area bilaterally. The rest of the bony ribcage as normal, no defects seen.

FIGURE 2

(a, b) shows computed tomography angiography images coronal reformat and axial slices respectively showing the globally enlarged heart with cardiothoracic ratio of 0.76, with a Ventral Septal Defect (VSD) noted in the coronal image, as well as the increased thickness of the right ventricular wall of 1.61 cm in the axial image. LV, left ventricle; RV, right ventricle.

FIGURE 3

(a, b) shows chest computed tomography angiography coronal reformats at different sections showing the aorta (Ao) arising entirely from the right ventricle (RV), the main pulmonary trunk (MPA) arising from both the right ventricle and the left ventricle (LV) due to a large mid‐septum ventricular septal defect (VSD) which measured 9.49 mm giving the double outlet right ventricle diagnosis. We also see that the MPA is twice the size of the aorta with the MPA: Ao of 2.4:1.

FIGURE 4

Computed tomography angiography axial slice at the level of the aortic arch showing severely hypoplastic mid and distal arch as compared to the ascending and descending thoracic aorta that measured 1 cm. There is also a left sided superior vena cava in addition to the normally placed right sided superior vena cava (SVC).

FIGURE 5

Computed tomography angiography axial slice at the level of the great vessels showing the enlarged main pulmonary trunk (MPA), double superior vena cavae (SVC) and a patent ductus arteriosus between the MPA and the Descending thoracic aorta.