PH Classification - Group 1 IPAH — EECC MCQ
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Correct answer: B — Group 1 PAH (idiopathic pulmonary arterial hypertension) after exclusion of other causes
This patient has pre-capillary PH (mPAP >20, PAWP ≤15, PVR >2 WU) with no evidence of left heart disease (normal PAWP), lung disease (normal PFTs), or CTEPH (normal V/Q scan). After excluding secondary causes (CTD, HIV, drugs, portal hypertension, CHD), this is classified as idiopathic PAH (Group 1). The 2022 ESC/ERS PH Guidelines classify PH into 5 groups: (1) PAH, (2) PH due to left heart disease, (3) PH due to lung disease/hypoxia, (4) CTEPH, (5) PH with unclear/multifactorial mechanisms. Treatment for Group 1 PAH follows the ESC risk stratification algorithm with initial combination oral therapy and potential escalation to parenteral prostacyclins.
Reference: ESC/ERS (2022): Guidelines for Pulmonary Hypertension