Vascular malformations as underlying cause of chronic thromboembolism and pulmonary hypertension

SummaryWe report four patients with chronic thromboembolic pulmonary hypertension (CTEPH) presumably due to recurrent pulmonary embolism from low-flow vascular malformations, and give a review of the literature.Venous malformations, such as those observed in Klippel- Trenaunay syndrome (KTS) can be associated with hypercoagulability, thrombosis and recurrent pulmonary embolism and ultimately CTEPH. Since many physicians appear unfamiliar with these potential complications, patients may experience a delayed diagnosis of this progressive and potentially life-threatening CTEPH.