Pulmonary Arterial Hypertension: The Clinical Syndrome
Open accessAbstract
Pulmonary arterial hypertension is a progressive disorder in which endothelial dysfunction and vascular remodeling obstruct small pulmonary arteries, resulting in increased pulmonary vascular resistance and pulmonary pressures. This leads to reduced cardiac output, right heart failure, and ultimately death. In this review, we attempt to answer some important questions commonly asked by patients diagnosed with pulmonary arterial hypertension pertaining to the disease, and aim to provide an explanation in terms of classification, diagnosis, pathophysiology, genetic causes, demographics, and prognostic factors. Furthermore, important molecular pathways that are central to the pathogenesis of pulmonary arterial hypertension are reviewed, including nitric oxide, prostacyclin, endothelin-1, reactive oxygen species, and endothelial and smooth muscle proliferation.
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Pulmonary arterial hypertension is caused by progressive smooth muscle and intimal proliferation and in situ thrombosis, ultimately obliterating the pulmonary arterioles and increasing pulmonary vascular resistance.
“Pulmonary arterial hypertension is caused by progressive smooth muscle and intimal proliferation and in situ thrombosis, ultimately obliterating the pulmonary arterioles and increasing pulmonary vascular resistance (PVR) (184).”
From Pathophysiology of Sickle Cell Disease (2019) · cites this paper as [184]
Many patients with PH share a common link to the metabolic syndrome.
“Despite the diversity of patients who develop PH, many share a common link to the metabolic syndrome[4,12–14].”
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References 126 · Cited by 402
- Authors
- Lai, Y.-C., Potoka, K. C., Champion, H. C., Mora, A. L., & Gladwin, M. T.
- Affiliations
- University of Pittsburgh (US)
- Venue
- Circulation Research
- Published
- 2014-06-20
- Volume
- 115
- Issue
- 1
- Pages
- 115-130
- ISSN
- 0009-7330 (ISSN-L)
- 1524-4571
- PMC
- PMC4096686
- OpenAlex
- W2061715434
- Cited by
- 402
- References
- 126
- Citations by year
- 2026: 29 · 2025: 30 · 2024: 32 · 2023: 37 · 2022: 36 · 2021: 40 · 2020: 46 · 2019: 44 · 2018: 35 · 2017: 31 · 2016: 24 · 2015: 14 · 2014: 3
- FWCI
- 19.26
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Circulation Research (publishedVersion)
- PubMed (publishedVersion)
- Europe PMC (PubMed Central) (acceptedVersion)
- PubMed Central (acceptedVersion)
- Funders
- National Heart, Lung, and Blood Institute (R01HL096973, P01HL103455, R01HL098032)
- Eunice Kennedy Shriver National Institute of Child Health and Human Development (K12HD052892)
- Topics
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Pulmonary Hypertension Research and Treatments
- Health Sciences › Medicine › Genetics › Vascular Anomalies and Treatments
- Health Sciences › Medicine › Pathology and Forensic Medicine › Systemic Sclerosis and Related Diseases
- Keywords
- pulmonary arterial hypertension, endothelial dysfunction, vascular remodeling, pulmonary vascular resistance, right heart failure, disease classification, diagnosis, pathophysiology, genetic factors, demographics, prognostic factors, nitric oxide, prostacyclin, endothelin-1, reactive oxygen species, endothelial cell proliferation, smooth muscle cell proliferation, molecular pathways
- MeSH terms
- Female, Hemodynamics, Humans, Hypertension, Pulmonary, Male, Mutation, Prognosis, Sex Factors, Signal Transduction, Positron-Emission Tomography, Bone Morphogenetic Protein Receptors, Type II, Familial Primary Pulmonary Hypertension
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