Pulmonary arterial hypertension: Navigating the pathways of progress in diagnosis, treatment, and patient care
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| Title: | Pulmonary arterial hypertension: Navigating the pathways of progress in diagnosis, treatment, and patient care |
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| Authors: | Miguel Azaredo Raposo, Daniel Inácio Cazeiro, Tatiana Guimarães, Nuno Lousada, Céline Freitas, Joana Brito, Susana Martins, Catarina Resende, Peter Dorfmüller, Rita Luís, Susana Moreira, Pedro Alves da Silva, Luís Moita, Mário Oliveira, Fausto J. Pinto, Rui Plácido |
| Source: | Revista Portuguesa de Cardiologia, Vol 43, Iss 12, Pp 699-719 (2024) |
| Publisher Information: | Elsevier BV, 2024. |
| Publication Year: | 2024 |
| Subject Terms: | Pulmonary Arterial Hypertension, Hipertensão pulmonar, Hipertensão arterial pulmonar, Diagnóstico, Hypertension, Pulmonary, Inibidores da fosfodiesterase tipo 5, 03 medical and health sciences, 0302 clinical medicine, Estimuladores da guanilato ciclase solúvel, RC666-701, Estratificação de risco, Critical Pathways, Diseases of the circulatory (Cardiovascular) system, Humans |
| Description: | Pulmonary arterial hypertension (PAH) is a form of precapillary pulmonary hypertension caused by a complex process of endothelial dysfunction and vascular remodeling. If left untreated, this progressive disease presents with symptoms of incapacitating fatigue causing marked loss of quality of life, eventually culminating in right ventricular failure and death. Patient management is complex and based on accurate diagnosis, risk stratification, and treatment initiation, with close monitoring of response and disease progression. Understanding the underlying pathophysiology has enabled the development of multiple drugs directed at different targets in the pathological chain. Vasodilator therapy has been the mainstay approach for the last few years, significantly improving quality of life, functional status, and survival. Recent advances in therapies targeting dysfunctional pathways beyond endothelial dysfunction may address the fundamental processes underlying the disease, raising the prospect of increasingly effective options for this high-risk group of patients with a historically poor prognosis. |
| Document Type: | Article |
| Language: | English |
| ISSN: | 0870-2551 |
| DOI: | 10.1016/j.repc.2024.03.004 |
| Access URL: | https://pubmed.ncbi.nlm.nih.gov/38972452 https://doaj.org/article/7624f3aa95ca4e73b556005891911fa6 |
| Rights: | CC BY NC ND |
| Accession Number: | edsair.doi.dedup.....5aadb154fb46b32d1bcc0e905f43b988 |
| Database: | OpenAIRE |
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Nájsť tento článok vo Web of Science | Abstract: | Pulmonary arterial hypertension (PAH) is a form of precapillary pulmonary hypertension caused by a complex process of endothelial dysfunction and vascular remodeling. If left untreated, this progressive disease presents with symptoms of incapacitating fatigue causing marked loss of quality of life, eventually culminating in right ventricular failure and death. Patient management is complex and based on accurate diagnosis, risk stratification, and treatment initiation, with close monitoring of response and disease progression. Understanding the underlying pathophysiology has enabled the development of multiple drugs directed at different targets in the pathological chain. Vasodilator therapy has been the mainstay approach for the last few years, significantly improving quality of life, functional status, and survival. Recent advances in therapies targeting dysfunctional pathways beyond endothelial dysfunction may address the fundamental processes underlying the disease, raising the prospect of increasingly effective options for this high-risk group of patients with a historically poor prognosis. |
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| ISSN: | 08702551 |
| DOI: | 10.1016/j.repc.2024.03.004 |