Double outlet right ventricle (with VSD and PAH)

Case contributed by Praveen Jha
Diagnosis certain


recurrent dyspnea since birth. Prior ECHO reveals pulmonary arterial hypertension and ventricular septal defect.

Patient Data

Age: 9
Gender: Male

Aorta and pulmonary artery are seen to arise from right ventricle, with a large ventricular septal defect in the membranous ventricular septum, with partial AV canal defect. Markedly dilated pulmonary artery is seen suggesting pulmonary arterial hypertension.

Case Discussion

This is a characteristic case of Double outlet right ventricle.

Surviving cases of double outlet right ventricle, usually have a concurrent VSD, and/or pulmonary stenosis, aortic coarctation or pulmonary artery hypertension.

This patient was taken for surgery, and diagnosis was confirmed. However, patient died intra-operatively probably due to severe concurrent PAH.

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