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Klippel-Trenaunay syndrome with small vessel pulmonary arterial hypertension

¹ Departement of Internal Medicine, University Hospital of Zurich, Switzerland
² Hirslanden Clinic, Zurich, Switzerland
³ Department of Radiology, University Hospital of Zurich, Switzerland

Correspondence to:
Correspondence to:
Dr S Ulrich
Departement of Internal Medicine, University Hospital of Zurich, Switzerland; silvia.ulrich{at}usz.ch

A patient with Klippel-Trenaunay syndrome and pulmonary arterial hypertension not associated with chronic thrombo-embolic pulmonary hypertension is described. It is hypothesised that pulmonary arterial hypertension is another complication associated with the Klippel-Trenaunay syndrome, possibly due to haemodynamic changes of small vessel abnormalities.

Abbreviations: CTEPH, chronic thromboembolic pulmonary hypertension; HHT, hereditary haemorrhagic telangiectasia; KTS, Klippel-Trenaunay syndrome; PAH, pulmonary arterial hypertension; PAP, pulmonary arterial pressure; PCH, pulmonary capillary haemangiomatosis; PH, pulmonary hypertension; PVOD, pulmonary veno-occlusive disease

Keywords: arteriovenous malformation; Klippel-Trenaunay syndrome; pulmonary hypertension