BB01.0

ICD-11 MMS 2026-01

Pulmonary arterial hypertension

Description

Pulmonary arterial hypertension is a clinical condition characterised by the presence of pre-capillary pulmonary hypertension in the absence of other causes of pre-capillary pulmonary hypertension, such as due to lung diseases, chronic thromboembolic pulmonary hypertension, or other rare diseases. It includes different forms that share a similar clinical picture and virtually identical pathological changes of the lung microcirculation.

Index terms

  • Pulmonary arterial hypertension
  • Arrillaga Ayerza syndrome
  • PAH - [pulmonary arterial hypertension]
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  • primary pulmonary hypertension
  • Idiopathic pulmonary arterial hypertension
  • Familial pulmonary arterial hypertension
  • heritable pulmonary arterial hypertension
  • Familial pulmonary arterial hypertension due to BMPR2 mutations
  • Familial pulmonary arterial hypertension due to ALK1 or endoglin mutations
  • Familial pulmonary arterial hypertension of unknown origin
  • Pulmonary arterial hypertension induced by drugs or toxins
  • Pulmonary arterial hypertension associated with other conditions
  • APAH - [Associated pulmonary arterial hypertension]
  • Pulmonary arterial hypertension associated with connective tissue diseases
  • Pulmonary arterial hypertension associated with HIV infection
  • Pulmonary arterial hypertension associated with portal hypertension
  • Pulmonary arterial hypertension associated with congenital heart disease
  • Eisenmenger syndrome
  • Pulmonary arterial hypertension associated with congenital systemic-to-pulmonary shunt
  • Pulmonary arterial hypertension associated with schistosomiasis
  • Pulmonary arterial hypertension associated with other aetiologies
  • Pulmonary veno-occlusive disease
  • Pulmonary capillary haemangiomatosis

Inclusions

  • primary pulmonary hypertension
  • Idiopathic pulmonary arterial hypertension

Exclusions from above levels

  • 01 – Certain infectious or parasitic diseases
  • 05 – Endocrine, nutritional or metabolic diseases
  • 18 – Complications of pregnancy, childbirth and the puerperium
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  • 19 – Certain conditions originating in the perinatal period
  • 20 – Congenital malformations, deformations and chromosomal abnormalities
  • 22 – Injury, poisoning or certain other consequences of external causes

Sibling codes

  • BB01.1 – Pulmonary hypertension due to left heart disease
  • BB01.2 – Pulmonary hypertension due to lung disease or hypoxia
  • BB01.3 – Chronic thromboembolic pulmonary hypertension
  • BB01.4 – Pulmonary hypertension with multifactorial mechanism
  • BB01.5 – Cor pulmonale
  • BB01.Z – Pulmonary hypertension, unspecified

Foundation entity

Pulmonary arterial hypertension – http://id.who.int/icd/entity/1931148955

Allowed postcoordination axes

Part of: BB01: Pulmonary hypertension › 11: Diseases of the circulatory system