ArticleJTCVS open2025
Loeys-Dietz syndrome subtypes exhibit distinct clinical behavior and aortic cellular transcriptomic profiles.
Article in JTCVS open, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers.
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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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Who cites it
2 citing papers in PubMed.
- Total Thrombus-Formation Analysis System (T-TAS) in Aortopathies: A Conceptual and Potential Framework to Spatial Heterogeneity and Regional Context.International journal of molecular sciences · 2026Review
- Bicuspid Aortic Valve in Heritable Thoracic Aortic Disease: Insights from the Montalcino Aortic Consortium.medRxiv : the preprint server for health sciences · 2025Article
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Authors and funding
8 authors.
Funding
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Abstract
Objectives: Loeys-Dietz syndrome comprises genetically discrete subtypes of varying clinical severity. This study integrates longitudinal Loeys-Dietz syndrome clinical outcomes after aortic root replacement with transcriptomic analysis of aortic smooth muscle cell dysregulation to investigate mechanisms governing this subtype-specific aortic vulnerability. Methods: Single institutional experience with aortic root replacement for nondissected aneurysm in patients with Loeys-Dietz syndrome was reviewed for midterm survival and distal aortic events (subsequent aortic intervention, aneurysm, or dissection). Single-cell RNA sequencing was performed using fresh aortic aneurysm tissue to compare smooth muscle cell phenotypes between patients with Results: A total of 62 patients with Loeys-Dietz syndrome were identified, including 59 genetically confirmed (n = 36 Conclusions: Loeys-Dietz syndrome subtypes may demonstrate variable clinical outcomes after aortic root replacement. Distinct gene dysregulation patterns suggest varying smooth muscle cell-extracellular matrix interactions may participate in clinical variation.
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