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Clinical Treatment and Management of Pulmonary Arterial Hypertension

A special issue of Journal of Clinical Medicine (ISSN 2077-0383). This special issue belongs to the section "Cardiovascular Medicine".

Deadline for manuscript submissions: closed (25 February 2025) | Viewed by 981

Special Issue Editors


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Guest Editor
Department of Medicine, Cardiovascular Division, Medical School, University of Minnesota, Minneapolis, MN 55455 USA
Interests: right ventricular dysfunction; pulmonary hypertension; pulmonary arterial hypertension

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Guest Editor
Department of Pediatrics, Division of Pediatric Cardiology, University of Texas Southwestern Medical Center, Dallas, TX, USA
Interests: pediatric cardiology; pulmonary hypertension; pulmonary arterial hypertension

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Guest Editor
Department of Medicine, Division of Allergy and Critical Care Medicine, University of Pittsburgh, Pittsburgh, PA 15213, USA
Interests: exercise hemodynamics; pulmonary vascular disease; invasive cardiopulmonary; exercise testing

Special Issue Information

Dear Colleagues,

Pulmonary arterial hypertension (PAH) is a progressive and life-threatening condition characterized by high pressure in the lung arteries. Treatment of PAH aims to relieve symptoms, slow down disease progression, and improve quality of life; it often involves a combination of medication, lifestyle modifications, and in some cases, surgical interventions. Current commonly used medications include endothelin receptor antagonists, phosphodiesterase-5 inhibitors, and prostacyclin analogs, with several new therapies in the horizon. Regular monitoring and follow-up with healthcare professionals are essential for managing PAH. Without proper treatment, PAH can lead to right-sided heart failure, severe complications, and reduced life expectancy. However, with early diagnosis and appropriate management, many individuals with PAH can lead fulfilling lives. 

I strongly encourage you to submit your original research or review papers about adult or pediatric PAH to our Special Issue and be a part of our vibrant scientific community.

Dr. Sasha Z. Prisco
Dr. Megan Griffiths
Dr. Michael Risbano
Guest Editors

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Keywords

  • pulmonary arterial hypertension
  • pulmonary hypertension
  • pulmonary vascular remodeling
  • right ventricle
  • right ventricular–pulmonary artery uncoupling
  • six-minute walk distance

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Published Papers (1 paper)

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Research

15 pages, 2746 KiB  
Article
Comparison of Tricuspid Annular Plane Systolic Excursion to Pulmonary Artery Systolic Pressure Ratio Measured by Transthoracic Echocardiography and Right Heart Catheterization in Pulmonary Arterial Hypertension: Prognostic Implications
by Eva Gutiérrez-Ortiz, Carmen Jiménez López-Guarch, Juan Francisco Delgado Jiménez, María Lorena Coronel, Irene Martín de Miguel, Belen Biscotti Rodil, Juan Duarte Torres, Teresa Segura de la Cal, María Teresa Velázquez Martín, Fernando Arribas Ynsaurriaga, Alejandro Cruz-Utrilla and Pilar Escribano-Subías
J. Clin. Med. 2025, 14(6), 1968; https://doi.org/10.3390/jcm14061968 - 14 Mar 2025
Viewed by 559
Abstract
Background/Objectives: Ventricular-arterial (VA) coupling, assessed via the TAPSE/PASP ratio, is a well-established prognostic marker in pulmonary arterial hypertension (PAH). However, transthoracic echocardiography (TTE) often fails to estimate the pulmonary artery systolic pressure (PASP). This study evaluated the prognostic value of TAPSE/PASP when [...] Read more.
Background/Objectives: Ventricular-arterial (VA) coupling, assessed via the TAPSE/PASP ratio, is a well-established prognostic marker in pulmonary arterial hypertension (PAH). However, transthoracic echocardiography (TTE) often fails to estimate the pulmonary artery systolic pressure (PASP). This study evaluated the prognostic value of TAPSE/PASP when PSAP was obtained both via TEE and RHC and their correlation. Methods: A prospective registry included 90 PAH patients (April 2021–May 2024). TTE and RHC were performed according to clinical guidelines. The correlation and agreement between both techniques were assessed using Spearman’s rank correlation and a Bland–Altman analysis. The prognostic utility of TAPSE/PASP for clinical worsening (CW) (death or lung transplantation) was evaluated using Cox models, Harrell’s c-statistics, and ROC curve analysis. Results: The median interval between TTE and RHC was 1.5 days (range −3 to +43). TAPSE/PASP showed a strong correlation between both techniques (rho = 0.74, p < 0.001), though TTE slightly overestimated values due to PASP underestimation. The PASP correlation was moderate (rho = 0.56, p < 0.001). CW occurred in 17.8% of patients. According to cut-off points established based on ESC/ERS guidelines, VA coupling via TTE effectively stratified the risk of CW (HR 7.0, p = 0.076 and HR 34.8, p = 0.002 for intermediate and high risk, respectively), whereas VA coupling with PASP measured via RHC showed no association with CW. TAPSE/PASP based on TTE demonstrated superior prognostic performance (C-index = 0.81) over RHC-derived parameters (C-index = 0.58). Conclusions: The TAPSE/PASP ratio showed a strong correlation between TTE and RHC. However, while RHC remains the gold standard for hemodynamic assessments, echocardiographic measurements demonstrated superior performance in risk stratification, supporting its role as a valuable non-invasive tool in PAH. Full article
(This article belongs to the Special Issue Clinical Treatment and Management of Pulmonary Arterial Hypertension)
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