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