Since the 1973 World Symposium on Pulmonary Hypertension, advancements in the understanding of pathophysiology and pathobiology have led to a myriad of pharmacotherapies for the disease. This article journeys through the development of therapeutic approaches for pulmonary arterial hypertension.
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http://dx.doi.org/10.1016/j.ccm.2020.10.002 | DOI Listing |
Front Genet
December 2024
Department of Pediatrics, West China Second University Hospital, Chengdu, Sichuan, China.
Background: Autosomal recessive cutis laxa type 1B (ARCL1B) is an extremely rare disease characterized by severe systemic connective tissue abnormalities, including cutis laxa, aneurysm and fragility of blood vessels, birth fractures and emphysema. The severity of this disease ranges from perinatal death to manifestations compatible with survival. To date, no cases have been reported in the Chinese population.
View Article and Find Full Text PDFEClinicalMedicine
August 2024
Center for Intelligent Medicine Research, Greater Bay Area Institute of Precision Medicine (Guangzhou), State Key Laboratory of Genetic Engineering, Center for Evolutionary Biology, School of Life Sciences, Fudan University, Guangzhou, China.
Background: Pulmonary embolism causes a substantial burden of morbidity and mortality. Although there are several well-established risk factors for pulmonary embolism, a substantial proportion of cases cannot be attributed to provoked or known risk factors. Accumulating evidence has suggested an association of clonal hematopoiesis of indeterminate potential (CHIP) with the risk of arterial thromboembolism.
View Article and Find Full Text PDFFront Immunol
January 2025
Department of Biomedical Sciences, University of Sassari, Sassari, Italy.
Introduction: The critical role played by vascular dysfunction and ineffective angiogenesis in the pathophysiology of systemic sclerosis (SSc) suggests that circulating biomarkers reflecting these alterations may be useful in the clinical evaluation of this patient group. We sought to address this issue by conducting a systematic review and meta-analysis of studies investigating a such candidate biomarker, endostatin, an endogenous glycoprotein exerting anti-angiogenic effects, in SSc patients and healthy controls.
Methods: A literature search was conducted in the electronic databases Web of Science, PubMed, and Scopus from inception to 27 May 2024.
J Saudi Heart Assoc
December 2024
Department of Pediatrics, Pediatric Cardiology Division, Specialized Pediatric Hospital, Cairo University, Egypt.
Objectives: To assess the outcome of pediatric pulmonary arterial hypertension (PAH) and to identify the predictors of morbidity and mortality of this progressive disease.
Patients And Methods: This prospective observational cohort study was conducted on consecutive pediatric patients with PAH. Medical history was taken with a grading of the WHO functional class as well as the serum N-terminal pro-BNP (NT pro-BNP), 6 min' walk test (6MWT), and echocardiography at the initial assessment and at follow-up.
Background: Pulmonary arterial hypertension (PAH) is a rare but severe and life-threatening condition that primarily affects the pulmonary blood vessels and the right ventricle of the heart. The limited availability of human tissue for research ~most of which represents only end-stage disease~ has led to a reliance on preclinical animal models. However, these models often fail to capture the heterogeneity and complexity of the human condition.
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