Extensive pulmonary arterial thrombi in situ in association with atrial septal defect

A 48-year-old woman with cyanosis was referred for investigation of atrial septal defect (ASD). Blood gas analysis on admission revealed moderate hypoxemia, and a pressure study during right heart catheterization revealed pulmonary hypertension (PH). Spiral computed tomography (CT) scan disclosed extensive thrombi in dilated large symmetrical pulmonary arteries with clear lung fields, and large strand-like thrombi on the inner surface of the pulmonary arterial wall along the vascular curvature were visualized by virtual CT angioscopic imaging. The thrombi were eventually considered to be not thromboemboli but thrombi in situ, because no segmental or larger defects were detected in the lung perfusion scan, although it showed cardiovascular imprints and an inhomogeneously decreased perfusion pattern. Pulmonary thrombi in situ are an uncommon manifestation in patients with ASD, and have not been described from the evidence of both CT and lung perfusion scans. The findings indicate that pulmonary thrombi in situ are not associated with occlusion of the large pulmonary arteries and the resultant development of PH. The patient was conservatively treated with medication, and the pulmonary thrombi did not show significant change with anticoagulant therapy. She died suddenly at the age of 51 years.