CTICasesSinus Venosus ASD with PAPVR and Pulmonary Hypertension
Imaging findings
An immigrant with an undiagnosed sinus venosus atrial septal defect and partial anomalous pulmonary venous return (pulmonary veins, including an inferior vein, directed toward the right atrium) has a markedly dilated right ventricle and severe pulmonary hypertension. The pulmonary arteries show extensive mural thrombus with occlusions and classic periarterial blushing; heart-lung transplant explant showed organizing thrombi.
Key takeaways
A long-standing sinus venosus ASD with PAPVR can lead to severe pulmonary hypertension with a blown-out right ventricle. The extensive occlusive mural thrombus raised debate about whether this represented chronic thromboembolic disease superimposed on shunt-related pulmonary hypertension or unusually occlusive mural thrombus alone, since mural thrombus rarely causes occlusion; periarterial blushing is a classic sign of severe pulmonary hypertension.
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