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Patent Ductus Arteriosus with Severe Pulmonary Hypertension

Imaging findings

Chest radiograph of a 39-year-old shows a markedly enlarged pulmonary artery, larger than the aorta and possibly calcified, with shunt vascularity throughout both lungs; the lateral confirms very large branch pulmonary arteries, with the main pulmonary artery segment and right ventricular outflow tract filling the retrosternal clear space. CT shows massive pulmonary arterial enlargement without bronchial arterial hypertrophy, a communication between the aorta and the pulmonary artery with a gradient of brighter systemic contrast entering the pulmonary artery and associated flow artifact, and central chronic-appearing in situ thrombus. The right heart is not dilated, while the left atrium and pulmonary veins are enlarged, and contrast is preferentially denser in the left pulmonary artery. There is no ascites to suggest right heart failure.

Key takeaways

Pulmonary hypertension in a young patient should prompt consideration of two main causes: a congenital shunt or a genetic mutation. Large, central, chronic-appearing thrombus in this setting is commonly misinterpreted as chronic thromboembolic disease, but in the absence of bronchial arterial hypertrophy it more likely represents in situ thrombus. This was an undiagnosed patent ductus arteriosus finally declaring itself in adulthood, with the right heart compensated enough not to have failed; flow remained left to right from systemic to pulmonary artery, so Eisenmenger syndrome had not yet developed. A single-phase CT can yield substantial physiologic information from contrast-density gradients alone.

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