Rare cause of chronic recurrent pulmonary emboli and pulmonary artery hypertension

A 41-year-old man with known Klippel-Trenaunay syndrome was admitted to hospital because of progressive dyspnea on exertion. Examination on admission revealed the typical signs of Klippel-Trenaunay syndrome, predominantly of the left leg. D-dimers were significantly increased. Contrast-enhanced computed tomography of the chest revealed multiple small pulmonary arterial emboli in subsegmental arteries on both lungs. Pulmonary arterial digital subtraction angiography revealed extensive peripherally localized perfusion defects. Pulmonary artery pressure measurement demonstrated bilateral pulmonary artery hypertension. Phlebography of the left lower leg showed marked varices in the calf. There was also a persisting sciatic vein. The recurrent peripheral pulmonary emboli with pulmonary arterial hypertension was a complication of a Klippel-Trenaunay syndrome. Another rare entity of this syndrome was a persisting sciatic vein. Heparinization was initiated, followed by oral anticoagulation. As the patient had not been anticoagulated before, implantation of an inferior vena cava filter was not deemed appropriate. Recurrent peripheral pulmonary emboli leading to chronic pulmonary artery hypertension is a rare but typical complication of Klippel-Trenaunay syndrome. Early recognition of this syndrome and any complications as a separate entity, as well as initiation of therapeutic measures, like anticoagulation or early pulmonary thrombendarterectomy for chronic pulmonary artery emboli, are of prognostic importance.