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Case Report
Klippel-Trenaunay syndrome with small vessel pulmonary arterial hypertension
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  1. S Ulrich1,
  2. M Fischler1,
  3. B Walder2,
  4. T Pfammatter3,
  5. R Speich1
  1. 1Departement of Internal Medicine, University Hospital of Zurich, Switzerland
  2. 2Hirslanden Clinic, Zurich, Switzerland
  3. 3Department of Radiology, University Hospital of Zurich, Switzerland
  1. Correspondence to:
    Dr S Ulrich
    Departement of Internal Medicine, University Hospital of Zurich, Switzerland; silvia.ulrichusz.ch

Abstract

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.

  • 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
  • arteriovenous malformation
  • Klippel-Trenaunay syndrome
  • pulmonary hypertension

https://doi.org/10.1136/thx.2004.023184

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    Footnotes

    • Competing interests: none declared