Pulmonary Hypertension - WHO Classification
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Pulmonary Hypertension - WHO Classification

Group 1: Pulmonary arterial hypertension

 • Idiopathic (F>M); Genetic (BMPR2, ALK1, ENG, SMAD9, CAV1, KCNK3); 

 • Drug/toxin (e.g., anorexigens, rapeseed oil, dasatanib, cocaine, amphetamines, SSRIs); 

 • CTD (MCTD, scleroderma, SLE); 

 • HIV; portopulmonary HTN; congenital heart diseases; schistosomiasis 

 • Subgroups: 1’ PVOD and/or pulmonary capillary hemangiomatosis; 1’’ persistent pulm HTN of newborn

Group 2: L heart disease - Post-capillary (PCWP > 15)

 • HFrEF; HFpEF; Valvular disease; Congenital/acquired left heart inflow/outflow tract obstruction and congenital cardiomyopathies

Group 3: Lung disease/hypoxia

 • COPD; ILD; OSA; Alveolar hypoventilation disorders; Developmental lung disease, among others

Group 4: CTEPH - Chronic thromboembolic (occurs after ~4% of PEs) 

Group 5: Miscellaneous

 • Chronic hemolytic anemia; Sickle cell disease; Myeloproliferative; Splenectomy; Sarcoidosis; Metabolic, etc



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