Extract

A 35-year-old male was admitted due to increasing dyspnea and chest pain. Physical examination found cyanosis, clubbed fingers, and systolic murmur at the left sternal border. Laboratory tests showed polycythemia (hemoglobin 221 g/L) and hypoxemia (PO2 50.8 mmHg). Echocardiography revealed that there was discontinuity of the pulmonary artery branches with the left pulmonary artery (LPA) originating from the main pulmonary artery and the right pulmonary artery (RPA) from the ascending aorta. RPA was severely dilated with a large echogenic mass suggestive of a large thrombus (Panels A and B). A large patent ductus arteriosus (PDA, diameter 11 mm) was present between the LPA and descending aorta (Panel C). Severe pulmonary hypertension was present in both pulmonary artery branches as suggested by a high velocity tricuspid regurgitation jet (around 6 m/s) and right-to-left shunting through the PDA (Panel D). CT angiography confirmed the echocardiographic findings, and confirmed the presence of a large thrombus in the RPA together with prominent arteriopulmonary collaterals (Panels E and F).

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