State-of-the-Art Paper
Updated Clinical Classification of Pulmonary Hypertension

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In 1998, a clinical classification of pulmonary hypertension (PH) was established, categorizing PH into groups which share similar pathological and hemodynamic characteristics and therapeutic approaches. During the 5th World Symposium held in Nice, France, in 2013, the consensus was reached to maintain the general scheme of previous clinical classifications. However, modifications and updates especially for Group 1 patients (pulmonary arterial hypertension [PAH]) were proposed. The main change was to withdraw persistent pulmonary hypertension of the newborn (PPHN) from Group 1 because this entity carries more differences than similarities with other PAH subgroups. In the current classification, PPHN is now designated number 1. Pulmonary hypertension associated with chronic hemolytic anemia has been moved from Group 1 PAH to Group 5, unclear/multifactorial mechanism. In addition, it was decided to add specific items related to pediatric pulmonary hypertension in order to create a comprehensive, common classification for both adults and children. Therefore, congenital or acquired left-heart inflow/outflow obstructive lesions and congenital cardiomyopathies have been added to Group 2, and segmental pulmonary hypertension has been added to Group 5. Last, there were no changes for Groups 2, 3, and 4.

Key Words

chronic thromboembolic pulmonary hypertension
PH due to chronic lung diseases
PH due to left heart disease
pulmonary arterial hypertension
pulmonary hypertension

Abbreviations and Acronyms

CHD
congenital heart disease
HAART
highly active antiretroviral therapy
HIV
human immunodeficiency virus
IFN
interferon
PAH
pulmonary arterial hypertension
PAP
pulmonary arterial pressure
PH
pulmonary hypertension
POPH
portopulmonary hypertension
PPHN
persistent pulmonary hypertension of the newborn
PVR
pulmonary vascular resistance
SCD
sickle cell disease
Sch-PAH
schistosomiasis-associated PAH
TGF
tumor growth factor
TKI
tyrosine kinase inhibitor

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Dr. Simonneau has served on advisory boards of Eli Lilly, Actelion, Pfizer, Bayer-Schering, GlaxoSmithKline, and Novartis; has received payment for lectures by Eli Lilly, Pfizer, Bayer-Schering, and GlaxoSmithKline; and his institution has received grant support from Actelion, Pfizer, Bayer-Schering, GlaxoSmithKline, and Novartis. Dr. Gatzoulis has served on advisory boards of Actelion UK and Global, Pfizer UK, and GlaxoSmithKline; and has received unrestricted educational grants from Actelion and Pfizer UK. Dr. Adatia is a member of Critical Events Committee for the tadalafil study in pediatrics with Eli Lilly. Dr. Celermajer is a member of the speakers' bureau, serves on the advisory board of, and receives travel and research support from Actelion. Dr. Denton has received payment for consultancy and speaker's fees from Actelion, Pfizer, GlaxoSmithKline, Digna, Sanofi Aventis, Boehringer Ingelheim, Roche, CSL Behring, and Genzyme; has received grant funding from Encysive, Actelion, Novartis, and Genzyme; and has served as clinical trial investigator and steering committee member for Pfizer, Actelion, Sanofi-Aventis, MedImmune, Digna, United Therapeutics, Novartis, and Celgene. Dr. Denton has received consultancy and speaker fees from Actelion, Pfizer, GlaxoSmithKline, Digna, Sanofi-Aventis, Boehringer Ingelheim, Roche, CSL Behring, and Genzyme. Dr. Ghofrani has received support from Actelion, Bayer, GlaxoSmithKline, Merck, Novartis, and Pfizer. Dr. Gomez Sanchez has received honoraria for consultations and speaking at conferences from Actelion, Bayer, GlaxoSmithKline, Novartis, Pfizer, United Therapeutics, and Ferrer Pharma. Dr. Landzberg has received research grants from Actelion, Myogen, and the NHLBI; and is on the steering committee for Actelion. Dr. Machado has received institutional grant support (without salary support) from Actelion and United Therapeutics; and has served on advisory boards of Gilead and United Therapeutics. Dr. Olschewski has received consultancy and lecture fees from Actelion, Bayer, Lilly, Gilead, GlaxoSmithKline, Pfizer, and Unither; and consultancy fees from NebuTec. Dr. Robbins has received honoraria from United Therapeutics, Gilead, and Actelion for attending advisory board meetings; has received honoraria from Actelion, Gilead, United Therapeutics, and Bayer; and he has been the primary investigator on industry-sponsored studies from Actelion, Gilead, United Therapeutics, GeNO, Novartis, and Aires in which payment was made to Vanderbilt University. Dr. Souza has received consultancy/lecture fees from Bayer. All other authors report that they have no relationships relevant to the contents of this paper to disclose.