Pulmonary hypertension: a fatal complication of neurofibromatosis type 1

Respir Care. 2011 Nov;56(11):1844-8. doi: 10.4187/respcare.01030. Epub 2011 May 20.

Abstract

We report a very rare case of severe pulmonary arterial hypertension in a patient with neurofibromatosis type 1, and discuss the pathology, pathogenesis, current pulmonary hypertension classification system, and outcomes of pulmonary arterial hypertension in patients with neurofibromatosis type 1.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Contraindications
  • Fatal Outcome
  • Female
  • Humans
  • Hypertension, Pulmonary / classification
  • Hypertension, Pulmonary / etiology*
  • Hypertension, Pulmonary / physiopathology
  • Hypertension, Pulmonary / therapy
  • Lung / diagnostic imaging
  • Lung Transplantation
  • Neurofibromatosis 1 / complications*
  • Tomography, X-Ray Computed