The New World Symposium on Pulmonary Hypertension Guidelines

Ruopp, N.; Farber, Harrison W.

doi:10.1161/circulationaha.119.040292

Cited by 11 publications

(6 citation statements)

References 5 publications

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“…This is a change from the original mPAP > 25 mmHg established in the 1970s by the WHO. A revised definition of PH was proposed in 2018 by a task force at the sixth World Symposium on Pulmonary Hypertension [ 1 ]. These new criteria are aimed at increasing the sensitivity of diagnosing PH as recent data suggest that even an mPAP > 20 mmHg carries an increased risk of mortality, although these data may not adjust for confounders such as increased age, BMI, and comorbidities of LHD or lung disease [ 1 ].…”

Section: Physiologymentioning

confidence: 99%

“…A revised definition of PH was proposed in 2018 by a task force at the sixth World Symposium on Pulmonary Hypertension [ 1 ]. These new criteria are aimed at increasing the sensitivity of diagnosing PH as recent data suggest that even an mPAP > 20 mmHg carries an increased risk of mortality, although these data may not adjust for confounders such as increased age, BMI, and comorbidities of LHD or lung disease [ 1 ]. Another reason for the change may be that many PAH cases still go undiagnosed for two or more years from the onset of symptoms to the time of diagnosis.…”

Section: Physiologymentioning

confidence: 99%

“…Another reason for the change may be that many PAH cases still go undiagnosed for two or more years from the onset of symptoms to the time of diagnosis. The change in definition comes with some controversy as the majority of the current data on the safety and efficacy of pharmacologic therapy have been collected on patients with a mPAP > 25 mm Hg and not in the population with mPAP > 20 and mPAP < 25, which was previously considered a borderline elevation [ 1 ]. Current and future studies will now need to include this population to ensure that this subgroup also shows the same degree of benefit with guideline-directed therapy.…”

Section: Physiologymentioning

confidence: 99%

“…Over time, PH leads to pulmonary vascular remodeling and increased pulmonary vascular resistance which can cause right ventricular failure and the systemic consequences of right heart failure, eventually culminating in death. Elevated mean pulmonary artery pressure (mPAP) > 20 mmHg measured on resting right-heart catheterization is the defining characteristic in all categories of PH [ 1 ]. The most common etiology of PH originates from left-heart disease (LHD) with the next most common one being parenchymal lung disease.…”

Section: Introductionmentioning

confidence: 99%

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Strategizing Drug Therapies in Pulmonary Hypertension for Improved Outcomes

et al. 2022

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Pulmonary hypertension (PH) is characterized by a resting mean pulmonary artery pressure (PAP) of 20 mmHg or more and is a disease of multiple etiologies. Of the various types of PH, pulmonary arterial hypertension (PAH) is characterized by elevated resistance in the pulmonary arterial tree. It is a rare but deadly disease characterized by vascular remodeling of the distal pulmonary arteries. This paper focuses on PAH diagnosis and management including current and future treatment options. Over the last 15 years, our understanding of this progressive disease has expanded from the concept of vasoconstrictive/vasodilatory mismatch in the pulmonary arterioles to now a better appreciation of the role of genetic determinants, numerous cell signaling pathways, cell proliferation and apoptosis, fibrosis, thrombosis, and metabolic abnormalities. While knowledge of its pathophysiology has expanded, the majority of the treatments available today still modulate the same three vasodilatory pathways that have been targeted for over 30 years (endothelin, nitric oxide, and prostacyclin). While modifying these pathways may help improve symptoms and quality of life, none of these directly modify the underlying disease pathogenesis. However, there are now studies ongoing with new drugs that can prevent or reverse these underlying causes of PAH. This review discusses the evidence base for the current treatment algorithms for PAH, as well as discusses novel therapies in development.

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Section: Physiologymentioning

confidence: 99%

Section: Physiologymentioning

confidence: 99%

Section: Physiologymentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Strategizing Drug Therapies in Pulmonary Hypertension for Improved Outcomes

et al. 2022

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“…It is characterized by elevated pressure in the pulmonary artery, with a mean pulmonary arterial pressure (PAP) at rest of ≥20 mm Hg, and manifests clinically with dyspnea (shortness of breath), fatigue, and presyncope/syncope (lightheadedness/fainting). [ 1 , 2 , 3 , 4 ]…”

Section: Introductionmentioning

confidence: 99%

The Role of Bone Morphogenetic Protein Receptor Type 2 (BMPR2) and the Prospects of Utilizing Induced Pluripotent Stem Cells (iPSCs) in Pulmonary Arterial Hypertension Disease Modeling

Devendran

Kar

Bailey

et al. 2022

Cells

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Pulmonary arterial hypertension (PAH) is a progressive disease characterized by increased pulmonary vascular resistance (PVR), causing right ventricular hypertrophy and ultimately death from right heart failure. Heterozygous mutations in the bone morphogenetic protein receptor type 2 (BMPR2) are linked to approximately 80% of hereditary, and 20% of idiopathic PAH cases, respectively. While patients carrying a BMPR2 gene mutation are more prone to develop PAH than non-carriers, only 20% will develop the disease, whereas the majority will remain asymptomatic. PAH is characterized by extreme vascular remodeling that causes pulmonary arterial endothelial cell (PAEC) dysfunction, impaired apoptosis, and uncontrolled proliferation of the pulmonary arterial smooth muscle cells (PASMCs). To date, progress in understanding the pathophysiology of PAH has been hampered by limited access to human tissue samples and inadequacy of animal models to accurately mimic the pathogenesis of human disease. Along with the advent of induced pluripotent stem cell (iPSC) technology, there has been an increasing interest in using this tool to develop patient-specific cellular models that precisely replicate the pathogenesis of PAH. In this review, we summarize the currently available approaches in iPSC-based PAH disease modeling and explore how this technology could be harnessed for drug discovery and to widen our understanding of the pathophysiology of PAH.

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