BB01.0 Pulmonary arterial hypertension

International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2025-01

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.

inclusions

• primary pulmonary hypertension
• Idiopathic pulmonary arterial hypertension

postcoordination

synonyms

• Pulmonary arterial hypertension
• primary pulmonary hypertension
• PAH - [pulmonary arterial hypertension]
• Arrillaga Ayerza syndrome
• 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

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