pulmonary hypertension

 

Pulmonary Hypertension Classification & Treatment Overview

Group	Cause	Common Etiologies	Treatment
Group 1	Pulmonary Arterial Hypertension (PAH)	Idiopathic, connective tissue disease, congenital heart disease, drugs (e.g., fenfluramine)	Advanced therapies: endothelin receptor antagonists (e.g., ambrisentan), PDE-5 inhibitors (e.g., sildenafil), prostacyclin analogs (e.g., epoprostenol)
Group 2	PH due to Left Heart Disease	HFrEF, HFpEF, valvular disease (e.g., AS, MR)	Treat underlying heart failure and valvular disease. No role for PAH therapies (e.g., sildenafil) unless in trial setting.
Group 3	PH due to Lung Disease or Hypoxia	COPD, interstitial lung disease, sleep apnea	Optimize lung disease, oxygen therapy
Group 4	Chronic Thromboembolic PH (CTEPH)	Chronic PE	Anticoagulation, possible pulmonary thromboendarterectomy (PTE), riociguat
Group 5	PH with unclear/multifactorial mechanisms	Sarcoid, sickle cell, metabolic disorders	Treat underlying disease, supportive care