Double outlet right ventricle (with VSD and PAH)

Case contributed by Dr Praveen Jha


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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Case information

rID: 18770
Published: 20th Jul 2012
Last edited: 16th Jul 2018
System: Cardiac
Inclusion in quiz mode: Included

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