ReviewFuture cardiology2025
Current landscape for connective tissue disease associated-pulmonary arterial hypertension: a focus on right ventricular adaptation and failure.
Review in Future cardiology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Authors and funding
6 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Right ventricular (RV) function is the primary determinant of survival in connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH). A "double hit" hypothesis suggests that RV suffers not only from pressure overload common to all PAH but also from direct insults related to the underlying systemic autoimmunity and inflammation. This likely drives distinct maladaptive remodeling (fibrosis, inflammation) and contributes to the poorer prognosis observed in CTD-PAH compared to idiopathic PAH (IPAH).Comprehensive, multi-modal RV assessment - integrating clinical evaluation, biomarkers, echocardiography, cardiac MRI, and right heart catheterization - is crucial for prognosis and monitoring therapy. RV size, function, and tissue characteristics are key indicators.Current management involves PAH-targeted therapies to reduce RV afterload, optimal CTD control, and supportive care. However, CTD-PAH often shows attenuated treatment responses and worse outcomes, emphasizing the need for therapies directly addressing RV maladaptation. Future research priorities include understanding specific RV injury mechanisms in CTD, refining assessment tools, and developing novel RV-directed strategies. Optimizing outcomes requires a deep understanding of RV pathobiology within the CTD context and integrated, multidisciplinary care.
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