Vascular malformations as underlying cause of chronic thromboembolism and pulmonary hypertension.
We 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.
Department of Plastic, Reconstructive and Hand surgery, Academic Medical Centre, Amsterdam, The Netherlands. ceoduber@gmail.com
Charlene E U
CE
Oduber
Victor E A
VE
Gerdes
Chantal M A M
CM
van der Horst
Paul
P
Bresser
D000328:Adult; D000368:Aged; D002908:Chronic Disease; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology*; D007715:Klippel-Trenaunay-Weber Syndrome / Q000150:complications; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D013923:Thromboembolism / Q000209:etiology*; D054079:Vascular Malformations / Q000150:complications* / Q000175:diagnosis