CTICasesDouble outlet right ventricle with Eisenmenger physiology
Imaging findings
Chest CT in a patient in her 40s shows a large ventricular septal defect, overriding aorta originating predominantly from the right ventricle, severe pulmonary arterial hypertension with a giant main and left pulmonary artery, right-to-left shunting, and pulmonary arterial atherosclerotic calcification. A right-sided aortic arch with an anomalous left subclavian artery is also present.
Key takeaways
Double outlet right ventricle is a congenital heart defect where both the aorta and pulmonary artery arise from the right ventricle. If left uncorrected into adulthood, the chronic high-pressure left-to-right shunt leads to irreversible pulmonary vascular remodeling, Eisenmenger physiology, severe pulmonary hypertension, and pulmonary arterial atherosclerosis.
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