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.
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All types of PH develop via an early, common vasculopathy driven by disproportionate pulmonary vasoconstriction and dramatic remodeling of the endothelium and vessel wall.
“Pathologically, all types of PH develop via an early, common vasculopathy driven by disproportionate pulmonary vasoconstriction and dramatic remodeling of the endothelium and vessel wall[5,26,27].”
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Flagged first, then by citations
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2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension
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Self-citation2014 ESC Guidelines on the diagnosis and management of acute pulmonary embolism
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ESC Guidelines for the management of grown-up congenital heart disease (new version 2010)
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Pharmacologic Therapy for Pulmonary Arterial Hypertension in Adults
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Autophagic protein LC3B confers resistance against hypoxia-induced pulmonary hypertension.
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Cardiovascular complications and risk of death in sickle-cell disease
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Hemodynamic Predictors of Mortality in Adults with Sickle Cell Disease
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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
- bronze
- doi.org
- Licence
- 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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Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
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