Pulmonary arterial hypertension (PAH) is characterized by vasoconstriction and cell proliferation in the pulmonary vasculature. Guideline-driven interventions with infused prostacyclin treatment are the mainstay for patients with advanced symptoms. Infused prostacyclin therapy is complex. It is critical to manage prostacyclin therapy with precision because boluses or interruptions can be fatal. Education of patients and inpatient staff nurses is necessary to prevent negative outcomes. Nurses are an essential part of the multidisciplinary team caring for patients with PAH. The diagnostic evaluation and treatment of PAH are reviewed here, and challenges associated with the care of patients on prostacyclin therapy are discussed.
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http://dx.doi.org/10.1097/NAN.0000000000000190 | DOI Listing |
The main treatment of patients with chronic thromboembolic pulmonary hypertension (CTEPH) is radical surgery, pulmonary thromboendarterectomy (PEA). However, about 40% of patients with CTEPH are inoperable due to distal pulmonary vascular lesions or the severity of hemodynamic disorders. Almost 30% of patients with CTEPH experience persistent or recurrent pulmonary hypertension after surgery, that requires a drug treatment with PAH-specific drugs.
View Article and Find Full Text PDFPostepy Biochem
December 2024
Department of Biochemistry and Nutrition, Centre for Preclinical Research and Technology, Medical University of Warsaw, Warsaw, Poland.
Prostaglandins are hormones found in almost all mammalian tissues. As signaling molecules, they play a key role in the regulation of many physiological processes, including hair growth cycle. The article describes the history of the discovery of prostaglandins, including the work of Professor Ryszard Gryglewski – the discoverer of prostacyclin.
View Article and Find Full Text PDFJ Manag Care Spec Pharm
January 2025
PRIME Education, New York City, NY.
Pulmonary hypertension associated with interstitial lung disease (PH-ILD) is a complex condition in which 2 consequential diseases interact and increase negative outcomes. Although the pathophysiologic mechanisms of PH-ILD are not yet well understood, the pronounced effect on functional status, supplemental oxygen requirements, health care resource utilization, and mortality that frequently accompany this diagnosis are well documented. A critical feature that complicates pathophysiologic understanding of PH-ILD is that progression of the pulmonary vascular disease does not always appear to be driven by the underlying lung disease.
View Article and Find Full Text PDFBMJ Open
December 2024
Clinical Research Department, Ignacio Chávez National Heart Institute, Mexico City, Mexico
Objectives: To investigate clinical characteristics, symptom profile, testing practices, treatment patterns and quality of life (QoL) among patients with pulmonary arterial hypertension (PAH) in Latin America.
Design: Data from the Adelphi Real World PAH Disease Specific Programme, a cross-sectional survey with retrospective data collection.
Setting: University/teaching hospital, regional centres, private practices and government institutions in Argentina, Brazil, Colombia and Mexico.
Front Med (Lausanne)
December 2024
Department of Cardiology, The University-Town Hospital of Chongqing Medical University, Chongqing, China.
Purpose: To systematically evaluate the clinical efficacy and safety of targeted drugs in patients with pulmonary arterial hypertension (PAH) with cardiac function grades III-IV, and conduct a meta-analysis.
Methods: Two researchers independently searched the PubMed, EMBASE, and Cochrane Library databases for relevant studies, with the search period extending from the establishment of the databases to March 2024. Meta-analysis was performed using statistical software Review Manager 5.
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