World health organization group 5 pulmonary hypertension

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20 Scopus citations


World Health Organization (WHO) group 5 pulmonary hypertension (PH) entails a heterogeneous group of disorders that may cause PH by unclear and/or multiple mechanisms. In particular, group 5 includes PH caused by hematologic disorders, systemic diseases, metabolic disorders, chronic renal failure, and disorders leading to pulmonary vascular occlusion or compression. This article discusses common pathogenic mechanisms leading to group 5 PH, followed by a detailed overview of epidemiology, pathogenesis, and disease-specific management of the individual group 5 conditions. Off-label use of vasomodulatory therapies, typically indicated for pulmonary arterial hypertension (WHO group 1PH), in group 5 conditions is also discussed.

Original languageEnglish
Pages (from-to)753-778
Number of pages26
JournalClinics in Chest Medicine
Issue number4
StatePublished - Dec 1 2013


  • Fibrosing mediastinitis
  • Lymphangioleiomyomatosis
  • Metabolic disorders
  • Myeloproliferative disorders
  • Pulmonary Langerhans cell histiocytosis
  • Renal failure
  • Sarcoidosis
  • Splenectomy


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