Vertebral Tortuosity Is Associated With Increased Rate of Cardiovascular Events in Vascular Ehlers‐Danlos Syndrome
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Le résumé fourni par la source
Background Arterial tortuosity is associated with adverse events in Marfan and Loeys‐Dietz syndromes but remains understudied in Vascular Ehlers‐Danlos syndrome. Methods and Results Subjects with a pathogenic COL3A1 variant diagnosed at age <50 years were included from 2 institutions and the GenTAC Registry (National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions). Height‐adjusted vertebral artery tortuosity index (VTI‐h) using magnetic resonance or computed tomography angiography was calculated. Associations between VTI‐h and outcomes of (1) cardiovascular events (arterial dissection/rupture, aneurysm requiring intervention, stroke), or (2) hollow organ collapse/rupture at age <50 years were evaluated using receiver operator curve analysis (using outcome by age 30 years) and mixed‐effects Poisson regression for incidence rate ratios. Of 65 subjects (54% male), median VTI‐h was 12 (interquartile range, 8–16). Variants were missense in 46%, splice site in 31%, and null/gene deletion in 14%. Thirty‐two subjects (49%) had 59 events, including 28 dissections, 5 arterial ruptures, 4 aneurysms requiring intervention, 4 strokes, 11 hollow organ ruptures, and 7 pneumothoraces. Receiver operator curve analysis suggested optimal discrimination at VTI‐h ≥15.5 for cardiovascular events (sensitivity 70%, specificity 76%) and no association with noncardiovascular events (area under the curve, 0.49 [95% CI, 0.22–0.78]). By multivariable analysis, older age was associated with increased cardiovascular event rate while VTI‐h ≥15.5 was not (incidence rate ratios, 1.79 [95% CI, 0.76–4.24], P =0.185). However, VTI‐h ≥15.5 was associated with events among those with high‐risk variants <40 years (incidence rate ratios, 4.14 [95% CI, 1.13–15.10], P =0.032), suggesting effect modification by genotype and age. Conclusions Increased arterial tortuosity is associated with a higher incidence rate of cardiovascular events in Vascular Ehlers‐Danlos syndrome. Vertebral tortuosity index may be a useful biomarker for prognosis when evaluated in conjunction with genotype and age.
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Le contrôle bibliographique ouvert
DOI retrouvé dans Crossref DOI retrouvé ; titre concordant.
- Titre Crossref
- Vertebral Tortuosity Is Associated With Increased Rate of Cardiovascular Events in Vascular Ehlers‐Danlos Syndrome
- Date Crossref
- 03/10/2023
- Éditeur
- Ovid Technologies (Wolters Kluwer Health)
- Type
- journal-article
Ce recoupement confirme des métadonnées liées au DOI. Il ne confirme ni la méthode ni les conclusions de l’étude, et il ne compte pas comme une seconde source scientifique indépendante.
Où se fait cette recherche
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Baylor College of Medicine Department of Pediatrics pays non établi dans la noticeUniversité ou école supérieure
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Texas Children's Hospital pays non établi dans la noticeOrganisme public
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The University of Texas Health Science Center at Houston Department of Epidemiology pays non établi dans la noticeUniversité ou école supérieure
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Oregon Health & Science University Department of Surgery pays non établi dans la noticeUniversité ou école supérieure
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Memorial Health University Medical Center Nicholas Dodd pays non établi dans la noticeÉtablissement de santé
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Kumamoto University Department of Diagnostic Radiology pays non établi dans la noticeUniversité ou école supérieure
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CHI St. Vincent pays non établi dans la noticeÉtablissement de santé
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MedStar Heart & Vascular Institute pays non établi dans la noticeÉtablissement de santé
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Cornell University pays non établi dans la noticeUniversité ou école supérieure
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Devereux pays non établi dans la noticeOrganisation à but non lucratif
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Weill Cornell Medicine pays non établi dans la noticeUniversité ou école supérieure
Department of Pediatrics — Baylor College of Medicine, Texas Children's Hospital et Department of Epidemiology — The University of Texas Health Science Center at Houston, avec 9 autres affiliations.
Une affiliation ne permet pas de déduire la nationalité d’un auteur.