ReviewInternational journal of cardiology. Congenital heart disease2024
Recent developments in connective tissue disease associated pulmonary arterial hypertension.
Review in International journal of cardiology. Congenital heart disease, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 6 papers.
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Who cites it
6 citing papers in PubMed.
- The New Era of Pulmonary Hypertension: The Dawn of Disease Modification & Therapeutic Modalities.Journal of cardiovascular development and disease · 2026Review
- Advancing the science and care of pulmonary hypertension, globally.International journal of cardiology. Congenital heart disease · 2026Article
- Vitamin D Attenuates Inflammation and Mitochondrial Dysfunction in Experimental Models Relevant to Connective Tissue Disease-Associated Pulmonary Arterial Hypertension.Food science & nutrition · 2026Article
- Connective Tissue Disease-Associated Pulmonary Arterial Hypertension: Current Therapeutic Strategies and Future Prospects.Biomolecules · 2026Review
- A Lung Ultrasound-Integrated Clinical Model for Predicting Pulmonary Arterial Hypertension in Patients with Connective Tissue Disease-Associated Interstitial Lung Disease.Diagnostics (Basel, Switzerland) · 2026Article
- Risk stratification and multidisciplinary management strategies for pregnancy complicated by pulmonary arterial hypertension: a narrative review.Frontiers in endocrinology · 2026Review
Corrections and comments
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Authors and funding
3 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Connective tissue disease associated pulmonary arterial hypertension (CTD-PAH) has benefited from the major treatment advances that have occurred within pulmonary hypertension over the past three decades. Inclusion of CTD-PAH cases in pivotal clinical trials led to regulatory approval and drug availability. This has improved outcomes but there are additional challenges for management. First, the multifaceted co-morbidity related to the associated CTD needs treatment alongside PAH and may impact on diagnosis and evaluation of treatment response. Secondary, cardiac involvement, interstitial lung disease and predisposition to thromboembolism in CTD may lead to compound phenotypes where PH has multiple mechanisms as well as precapillary pulmonary vasculopathy of PAH. In general, especially for systemic sclerosis, CTD-PAH has worse long-term survival than idiopathic or familial PAH. However, CTD also present an opportunity for screening and early detection and treatment for associated PAH, and this may in the future be a major advantage over idiopathic disease where presentation inevitable only occurs at symptomatic stages and diagnosis may be delayed. This article reviews and summarises some of the recent developments in investigation and management of CTD-PAH.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.