Background And Objective: The definition of pre-capillary pulmonary hypertension (PH) has been modified, with lowering of the mean pulmonary arterial pressure (mPAP) threshold from 25 to 20 mmHg and addition of a mandatory criterion of pulmonary vascular resistance (PVR) ≥ 2 Wood units (WU). Our objectives were: 1/ to estimate the proportion of patients reclassified as having pre-capillary PH when using the new 2022 ESC/ERS hemodynamic criteria (i.e. mPAP 21-24 mmHg and PVR ≥ 2 WU), and to describe their clinical characteristics and outcome; and 2/ to study the relationship between PVR and survival in patients with mPAP > 20 mmHg.
Methods: We retrospectively analyzed consecutive SSc patients included in our National Reference Center for a first right-heart catheterization between 2003 and 2018. The association between survival and PVR was studied using smoothing splines.
Results: We included 126 SSc patients with mPAP > 20 mmHg. Among them, 16 (13%) had a baseline mPAP value between 21 and 24 mmHg and PVR ≥ 2 mmHg and were reclassified as pre-capillary PH; 10 of which (62%) raised their mPAP ≥ 25 mmHg during follow-up. In patients with mPAP > 20 mmHg, we observed a linear relation between PVR and mortality for values < 6 WU.
Conclusion: A significant proportion of SSc patients is reclassified as having pre-capillary PH with the new 2022 ESC/ERS hemodynamic definition. Lowering the PVR threshold from 3 to 2 WU captures patients at risk of raising their mPAP > 25 mmHg, with a possibly less severe disease.
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http://dx.doi.org/10.1186/s12931-022-02205-4 | DOI Listing |
Eur Respir J
November 2024
Division of Pulmonology, Department of Internal Medicine, Medical University of Graz, Graz, Austria.
Background: Exercise pulmonary hypertension (exercise PH) was defined by a mean pulmonary arterial pressure (mPAP)/cardiac output (CO) slope >3 mmHg·L·min between rest and exercise in the 2022 ESC/ERS PH guidelines. However, large, multi-center studies on the prognostic relevance of exercise hemodynamics and its added value to resting hemodynamics are missing.
Patients And Methods: The PEX-NET (Pulmonary Hemodynamics during Exercise Network) registry enrolled patients who underwent clinically indicated right heart catheterizations both at rest and ergometer exercise from 23 PH-centers worldwide.
Zhonghua Yi Xue Za Zhi
November 2024
Department of Pulmonary and Critical Care Medicine, Beijing Anzhen Hospital Affiliated to Capital Medical University, Beijing100029, China.
In the 2022 European Society of Cardiology/European Respiratory Society (ESC/ERS) guidelines, the hemodynamic definition of pulmonary hypertension (PH) was revised to mean pulmonary arterial pressure (mPAP)>20 mmHg (1 mmHg=0.133 kPa) at rest. This study aimed to evaluate the impact of the revised hemodynamic definition on the diagnosis of PH.
View Article and Find Full Text PDFHeart Lung
November 2024
Department of İnternal Medicine, Fethi Sekin Sehir Hastanesi, Elazıg, Turkey.
Background: Pulmonary arterial hypertension (PAH) is characterized by increased pulmonary artery pressure with significant morbidity and mortality. Inflammatory processes are crucial in PAH pathogenesis, with inflammatory cells and mediators present early in disease progression. IL-34 involvement in inflammatory pathways suggests that IL-34 could be an important player in the progression of PAH, influencing both pulmonary pressures and vascular changes.
View Article and Find Full Text PDFJ Heart Lung Transplant
October 2024
Sheffield Pulmonary Vascular Disease Unit, Royal Hallamshire Hospital, Sheffield, UK; Division of Clinical Medicine, School of Medicine and Population Health, University of Sheffield, Sheffield, UK. Electronic address:
Background: There are limited data assessing the spectrum of systemic sclerosis-associated pulmonary hypertension (PH).
Methods: Data for 912 systemic sclerosis patients assessed between 2000 and 2020 were retrieved from the Assessing the Spectrum of Pulmonary hypertension Identified at a REferral centre (ASPIRE) registry and classified based on 2022 European Society of Cardiology/European Respiratory Society (ESC/ERS) guidelines and multimodality investigations.
Results: Reduction in pulmonary vascular resistance (PVR) diagnostic threshold to >2WU resulted in a 19% increase in precapillary PH diagnoses.
Eur Respir J
October 2024
Center for Congenital Heart Diseases, Paediatric and Congenital Cardiology, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
Paediatric pulmonary arterial hypertension (PAH) shares common features with adult disease, but is associated with several additional disorders and challenges that require unique approaches. This article discusses recent advances, ongoing challenges and distinct approaches for caring for infants and children with PAH, as presented by the paediatric task force of the 7th World Symposium on Pulmonary Hypertension. We provide updates on diagnosing, classifying, risk-stratifying and treating paediatric pulmonary hypertension (PH) and identify critical knowledge gaps.
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