Cited by 4,191Pulmonary Hypertension Research and TreatmentsCongenital Heart Disease StudiesVascular Anomalies and Treatments

Updated clinical classification of pulmonary hypertension

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Simonneau, Gerald, Gatzoulis, Michael A., Adatia, Ian, Celermajer, David, Denton, Chris, Ghofrani, Ardeschir, Gomez Sanchez, Miguel Angel, Krishna Kumar, R. +5 more·2013·Journal of the American College of Cardiology

Abstract

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.

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  1. [4]4. Galie N., Torbicki A., Barst R. et al: Guidelines on diagnosis and treatment of pulmonary arterial hypertension. The Task Force on Diagnosis and Treatment of Pulmonary Arterial Hypertension of the European Society of Cardiology. Eur Heart J 2004; 25: 2243. Crossref PubMed Google Scholar
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Authors
Simonneau, G., Gatzoulis, M. A., Adatia, I., Celermajer, D., Denton, C., Ghofrani, A., Gomez Sanchez, M. A., Krishna Kumar, R., Landzberg, M., Machado, R. F., Olschewski, H., Robbins, I. M., & Souza, R.
Affiliations
  • University of Alberta (CA)
  • The University of Sydney (AU)
  • The Royal Free Hospital (GB)
  • Boston Children's Hospital (US)
  • University of Illinois Chicago (US)
  • Medical University of Graz (AT)
  • Vanderbilt University Medical Center (US)
  • Universidade de São Paulo (BR)
Venue
Journal of the American College of Cardiology
Publisher
Elsevier BV
Published
2013-12
Volume
62
Issue
25
Pages
D34-D41
ISSN
0735-1097 (ISSN-L)
OpenAlex
W2183644045
Cited by
4,191
References
193
Citations by year
2026: 35 · 2025: 60 · 2024: 87 · 2023: 96 · 2022: 131 · 2021: 182 · 2020: 224 · 2019: 253 · 2018: 360 · 2017: 411 · 2016: 441 · 2015: 465 · 2014: 378 · 2013: 287 · 2012: 293
FWCI
81.39
Type
journal article
Language
en
Open access
Locations
  • Journal of the American College of Cardiology (publishedVersion)
  • PubMed (publishedVersion)
Topics
  • Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Pulmonary Hypertension Research and Treatments
  • Health Sciences › Medicine › Epidemiology › Congenital Heart Disease Studies
  • Health Sciences › Medicine › Genetics › Vascular Anomalies and Treatments
Keywords
pulmonary hypertension, clinical classification, pulmonary arterial hypertension, persistent pulmonary hypertension of the newborn, chronic hemolytic anemia, pediatric pulmonary hypertension, congenital heart disease, congenital cardiomyopathy, hemodynamic characteristics
MeSH terms
Anemia, Hemolytic, Animals, Connective Tissue Diseases, Heart Defects, Congenital, Humans, Hypertension, Pulmonary

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