Scleroderma is an uncommon autoimmune disease of unknown cause that may affect any organ system in the body. Patients with scleroderma are prone to developing pulmonary complications, including pulmonary arterial hypertension (PAH), that are the leading cause of death in this population. This article describes scleroderma-related PAH and its diagnosis and management.
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http://dx.doi.org/10.1097/01.JAA.0000522129.93995.13 | DOI Listing |
Int J Cardiol Congenit Heart Dis
March 2025
Department of Obstetrics, Chelsea and Westminster Hospital NHS Foundation Trust, London, UK.
Pulmonary hypertension (PH) is a term used to describe a complex heterogenous group of conditions defined by a mean pulmonary arterial pressure of more than 20 mmHg at rest on right-heart catheterization. PH in pregnancy is associated with high rates of maternal morbidity and mortality and poor fetal outcomes. Currently, pregnancy in these women is classified as modified WHO class IV (pregnancy contraindicated).
View Article and Find Full Text PDFInt J Cardiol Congenit Heart Dis
March 2025
Royal Brompton Hospital, Part of GSTT Foundation Trust, UK.
Int J Cardiol Congenit Heart Dis
March 2025
From the Cardiopulmonary Department, Instituto Nacional de Cardiologia Ignacio Chavez, Mexico City, Mexico.
Pulmonary arterial hypertension (PAH) has been classically described as a disease in young adults, predominantly females with no comorbidities. However, in recent registries, the epidemiology has changed to older patients with comorbidities such as obesity, diabetes, systemic hypertension, and coronary heart disease. Nevertheless, there is not enough inclusion of these patients in clinical trials.
View Article and Find Full Text PDFCirc Rep
March 2025
Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center Osaka Japan.
Background: Despite the development of effective pulmonary vasodilators, the prognosis for patients with pulmonary hypertension (PH) remains poor, particularly in medication-refractory patients. Catheter-based pulmonary artery denervation (PADN) is an emerging therapeutic strategy targeting the sympathetic nervous system in various types of PH. However, data on its safety and efficacy in refractory patients with PH who truly require non-pharmacotherapy are lacking.
View Article and Find Full Text PDFCirc Rep
March 2025
Department of Vascular Physiology, National Cerebral and Cardiovascular Center Research Institute Osaka Japan.
Background: Because Regnase-1, encoded by , suppresses the development of pulmonary arterial hypertension (PAH) by controlling pro-inflammatory cytokines, we aimed to identify variants in patients with PAH.
Methods And Results: We analyzed whole-genome sequence data of patients with PAH to search for disease-associated variants. The Regnase-1 p.
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