AI Article Synopsis
- Pulmonary arterial hypertension (PAH) is a serious and worsening condition that affects the small blood vessels in the lungs, leading to high blood pressure, heart failure, and a high risk of death, particularly in patients classified as WHO functional class IV.
- Recent advancements in PAH treatments have improved patient outcomes significantly, especially when used in combination with tailored management strategies.
- Despite these improvements, the prognosis for WHO FC IV PAH patients remains bleak, prompting ongoing research and updated treatment recommendations aimed at better addressing their critical health needs.
Article Abstract
Pulmonary arterial hypertension (PAH) is a severe, progressive condition of the small pulmonary vessels that leads to increased pulmonary vascular resistance, right ventricular failure and death. Patients in World Health Organization functional class (WHO FC) IV are the most severely affected in terms of disease severity, symptomatic impairment, exercise capacity and haemodynamics, with a very poor prognosis and low survival rate. Recent developments in PAH-specific therapies have conferred significant prognostic improvements upon PAH patients, especially when coupled with management strategies such as goal-oriented therapy and combination treatment. Despite these important developments, the outlook for WHO FC IV PAH patients remains poor. This article examines the recommendations for WHO FC IV patients that appear in current PAH treatment guidelines and the research underpinning this guidance, and discusses possible future directions for treatment of this severely unwell patient population.
Download full-text PDF |
Source |
|---|---|
| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC9487499 | PMC |
| http://dx.doi.org/10.1183/09059180.00008010 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Congenital pulmonic and aortic stenosis in Newfoundland dogs: Results of a 14-year French cardiovascular screening program (921 dogs).
PLoS One
January 2025
Cardiology Department, Vétérinaire Clinic Boulogne Roland Garros, Boulogne Billancourt, France.
Introduction: Aortic stenosis (AS) and pulmonic stenosis (PS) are two of the most common canine congenital heart diseases (CHD), with a high relative risk for Newfoundland dogs to develop inherited subvalvular AS. For this reason, a cardiovascular screening program has been set up by the French Newfoundland kennel club in order to manage mattings and reduce AS prevalence.
Materials And Methods: The records of untreated and non-anesthetized adult Newfoundland dogs screened between 2010 and 2023 were retrospectively reviewed.
Management Strategies for Truncus Arteriosus: A Comparative Analysis of Staged vs. Primary Repair.
Pediatr Cardiol
January 2025
Department of Cardiovascular Surgery, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences and Okayama University Hospital, 2-5-1 Shikatacho, Kitaku, Okayama, Japan.
We reviewed the outcomes of truncus arteriosus repair (primary vs. staged repair incorporating bilateral pulmonary artery banding), focusing on survival, reintervention, and functional data. We analyzed 39 patients who underwent a first intervention for truncus arteriosus (staged, n = 19; primary, n = 20) between 1992 and 2022.
View Article and Find Full Text PDFAccuracy of two-compartment modelling of gas exchange with ventilation-perfusion mismatch in inhalational anesthesia.
Anesthesiology
January 2025
Department of Critical Care, Melbourne Medicine School, Faculty of Medicine, Dentistry and Health Sciences, University of Melbourne, Victoria, Australia.
Background: Multi-compartment computer models of heterogeneity in alveolar ventilation-perfusion ratios (VA/Q scatter) across the lung explain the significant alveolar-arterial (A-a) partial pressure gradients and associated alveolar dead-space fractions (VDA/VA) seen in anesthetized patients for both carbon dioxide and for anesthetic gases of different blood solubilities. However, the accuracy of a simpler two-compartment model of VA/Q scatter to do this has not been tested or compared to calculations from the traditional Riley model with "ideal", unventilated (shunt) and unperfused (deadspace) compartments.
Methods: Measurements of gas partial pressures in inspired and expired gas and arterial and mixed venous blood from 29 patients undergoing inhalational general anesthesia for cardiac surgery was used to compare the accuracy of two simple models of VA/Q scatter and lung gas exchange in predicting measured alveolar and arterial partial pressure differences, and associated alveolar dead-space calculations for the modern anesthetic gases isoflurane, sevoflurane and desflurane.
Berlin Heart EXCOR sVAD implantation technique for neonates and infants with functionally univentricular ductal-dependent systemic circulation.
Multimed Man Cardiothorac Surg
January 2025
Congenital Heart Center, Division of Cardiovascular Surgery, Department of Surgery, University of Florida, Gainesville, FL, USA.
The Berlin Heart EXCOR is a pulsatile paracorporeal ventricular assist device (VAD) for neonates, infants, children and adults with congenital or acquired severe ventricular dysfunction. Berlin Heart EXCOR VADs are routinely used as either a bridge to a cardiac transplantation, or occasionally as a bridge to ventricular recovery. Our programmatic philosophy is to bridge neonates and infants with functionally univentricular ductal-dependent systemic circulation or functionally univentricular ductal-dependent pulmonary circulation who are at high risk for staged palliation because of important cardiac risk factors with a single-ventricle VAD (sVAD) as a bridge to a cardiac transplant.
View Article and Find Full Text PDFSystemic venous collaterals between Glenn and Fontan: prevalence, therapy, and impact on outcomes.
Cardiol Young
January 2025
Department of Congenital and Pediatric Heart Surgery, German Heart Center Munich, Technische Universität München, Division of Congenital and Pediatric Heart Surgery, University Hospital of Munich, Ludwig-Maximilians-Universität München, Europäisches Kinderherzzentrum München, Munich, Germany.
Objective: This study aimed to evaluate veno-venous collaterals between bidirectional cavopulmonary shunt and total cavopulmonary connection.
Methods: Patients who underwent staged total cavopulmonary connection between 1995 and 2022 were reviewed. Veno-venous collaterals between bidirectional cavopulmonary shunt and total cavopulmonary connection were depicted using angiograms.
Want AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!