BB01.0
Pulmonary arterial hypertension
Hipertensão arterial pulmonar
CategoryDefinition
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
Index Terms
Pulmonary arterial hypertensionprimary pulmonary hypertensionPAH - [pulmonary arterial hypertension]Arrillaga Ayerza syndromeIdiopathic pulmonary arterial hypertensionFamilial pulmonary arterial hypertensionheritable pulmonary arterial hypertensionFamilial pulmonary arterial hypertension due to BMPR2 mutationsFamilial pulmonary arterial hypertension due to ALK1 or endoglin mutationsFamilial pulmonary arterial hypertension of unknown originPulmonary arterial hypertension induced by drugs or toxinsPulmonary arterial hypertension associated with other conditionsAPAH - [Associated pulmonary arterial hypertension]Pulmonary arterial hypertension associated with connective tissue diseasesPulmonary arterial hypertension associated with HIV infectionPulmonary arterial hypertension associated with portal hypertensionPulmonary arterial hypertension associated with congenital heart diseaseEisenmenger syndromePulmonary arterial hypertension associated with congenital systemic-to-pulmonary shuntPulmonary arterial hypertension associated with schistosomiasisPulmonary arterial hypertension associated with other aetiologiesPulmonary veno-occlusive diseasePulmonary capillary haemangiomatosis