Updated Clinical Classification of Pulmonary Hypertension

• Published in: Journal of the American College of Cardiology Vol. 54; no. 1; pp. S43 - S54
• Main Authors: Simonneau, Gérald, Robbins, Ivan M., Beghetti, Maurice, Channick, Richard N., Delcroix, Marion, Denton, Christopher P., Elliott, C. Gregory, Gaine, Sean P., Gladwin, Mark T., Jing, Zhi-Cheng, Krowka, Michael J., Langleben, David, Nakanishi, Norifumi, Souza, Rogério
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 30.06.2009; Elsevier Limited
• Subjects: Algorithms; Anticoagulants - therapeutic use; Antihypertensive Agents - administration & dosage; Calcium Channel Blockers - therapeutic use; Cardiology; Cardiovascular; Cardiovascular disease; Classification; clinical classification; Clinical trials; Comorbidity; Defects; Drug therapy; Endothelin Receptor Antagonists; Epoprostenol - administration & dosage; Evidence-Based Medicine; Family medical history; Genetic counseling; Humans; Hypertension, Pulmonary - classification; Hypertension, Pulmonary - drug therapy; Hypertension, Pulmonary - epidemiology; Internal Medicine; Mutation; Prognosis; pulmonary arterial hypertension; Pulmonary hypertension; Randomized Controlled Trials as Topic; Scleroderma, Systemic - epidemiology; Surgery; Treatment Outcome; United States > US; California; ESRD; BMPR2; OR; pulmonary hypertension; PPH; IPAH; PVR; POPH; HIV; PCH; SCD; PAH; PH; clinical classification; TRV; CTEPH; pulmonary arterial hypertension; CHD; PAP; PVOD; odds ratio; pulmonary arterial pressure; primary pulmonary hypertension; sickle cell disease; congenital heart disease; bone morphogenetic protein receptor type 2; tricuspid regurgitation jet velocity; end-stage renal disease; idiopathic pulmonary arterial hypertension; chronic thromboembolic pulmonary hypertension; human immunodeficiency virus; pulmonary veno-occlusive disease; pulmonary vascular resistance; pulmonary capillary hemangiomatosis; portopulmonary hypertension
• ISSN: 0735-1097; 1558-3597; 1558-3597
• DOI: 10.1016/j.jacc.2009.04.012

The aim of a clinical classification of pulmonary hypertension (PH) is to group together different manifestations of disease sharing similarities in pathophysiologic mechanisms, clinical presentation, and therapeutic approaches. In 2003, during the 3rd World Symposium on Pulmonary Hypertension, the clinical classification of PH initially adopted in 1998 during the 2nd World Symposium was slightly modified. During the 4th World Symposium held in 2008, it was decided to maintain the general architecture and philosophy of the previous clinical classifications. The modifications adopted during this meeting principally concern Group 1, pulmonary arterial hypertension (PAH). This subgroup includes patients with PAH with a family history or patients with idiopathic PAH with germline mutations (e.g., bone morphogenetic protein receptor-2, activin receptor-like kinase type 1, and endoglin). In the new classification, schistosomiasis and chronic hemolytic anemia appear as separate entities in the subgroup of PAH associated with identified diseases. Finally, it was decided to place pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis in a separate group, distinct from but very close to Group 1 (now called Group 1′). Thus, Group 1 of PAH is now more homogeneous.