Table 32013 (Nice, France) Pulmonary Arterial Hypertension Categories

1 Pulmonary arterial hypertension
1.1 Idiopathic
1.2 Heritable
  1.2.1. BMPR2
  1.2.2. ALK1, ENG, CAV1, KCNK3, Smad9
  1.2.3. Unknown
1.3 Drug- and toxin-induced
1.4 Associated with:
  1.4.1 Connective tissue disease
  1.4.2 HIV infection
  1.4.3 Portal hypertension
  1.4.4 Congenital heart disease
  1.4.5 Schistosomiasis
1’ Pulmonary veno-occlusive disease and/or pulmonary capillary hemangiomatosis
1”. Persistent pulmonary hypertension of the newborn

ALK1 = activin receptor-like kinase type 1; BMPR2 = bone morphogenetic protein receptor type 2; CAV1 = caveolin-1; ENG = endoglin; KCNK3 = potassium channel super family K member-3; Smad 9 = mothers against decapentaplegic 9.

Source: Simonneau 2013.2

From: 1, Introduction

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