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. 2008 Oct;4(5):943–952. doi: 10.2147/vhrm.s2270

Table 1.

Pulmonary hypertension, Group I World Health Organization Classification (after Venice 2003)

Group Ia
Idiopathic IPAH (IPAH)
Familial PAH (FPAH)
Related to

  • Connective tissue diseases

  • HIV

  • Portal hypertensionb

  • Anorexigens

  • Congenital heart diseases

Primary pulmonary hypertension of the newborn (PPHN)
PAH with venule/capillary involvement (pulmonary veno-occlusive disease, PVOD)
Others: Glycogen storage disease, Gaucher’s, hemoglobinopathies (ie, sickle cell), hereditary hemorrhagic telagiectasia (HHT)
a

Endothelin receptor anagonists have not been studied formally in portal hypertension, PPHN, PVOD and other types of group I PAH.

b

Given the potential for liver toxicity, caution is advised when using these agents in patients with portal hypertension due to end stage liver disease.