Increased Vertebral Artery Tortuosity Index Is Associated With Adverse Outcomes in Children and Young Adults With Connective Tissue Disorders

Abstract

Background-Arterial tortuosity is described as a common and distinctive feature of Loeys-Dietz syndrome (LDS), yet reports on arterial tortuosity are based on qualitative observations and none have investigated an association between tortuosity and cardiovascular outcomes in LDS or other connective tissue disorders. Methods and Results-We performed a retrospective analysis of 90 patients Յ50 years of age with Marfan syndrome, LDS, Ehlers-Danlos syndrome, or nonspecific connective tissue disorder who underwent thoracic contrast-enhanced magnetic resonance angiography. Controls (nϭ30) underwent magnetic resonance imaging to exclude arrhythmogenic right ventricular dysplasia. Using a volume-rendered angiogram, vertebral arteries were measured along the curvature of the vessel (actual length) and linearly (straight length), and distance factor was calculated: [(actual/straight lengthϪ1)ϫ100]. Each subject's maximum distance factor was designated the Vertebral Tortuosity Index (VTI). The VTI was compared among diagnostic groups and among patients with cardiac surgery, dissection, and death. Median age at magnetic resonance imaging was 19.6 years (range 0.2 to 50.1). VTI interrater reliability was excellent (intraclass correlation coefficient ϭ0.987). The VTI was higher in Marfan syndrome (nϭ57, median 26; interquartile range 10 to 49) and LDS (nϭ13, median 58; interquartile range 18 to 92) compared with controls (median 4.5; interquartile range 3 to 6; PϽ0.001 for both). Higher VTI was associated with younger age at surgery even when controlling for root size (adjusted Pϭ0.002). Vertebral tortuosity index Ն50 was associated with earlier age at dissection and death compared with VTI Ͻ50 (Pϭ0.001 versus PϽ0.001). We found no difference in age at surgery, dissection, or death in Marfan syndrome compared with LDS. Conclusion-Arterial tortuosity measured by magnetic resonance angiography is a reproducible marker of adverse cardiovascular outcomes in connective tissue disorders. (Circulation. 2011;124:388-396.)

Basilar Artery Angulation in Association with Aging and Pontine Lacunar Infarction: A Multicenter Observational Study

“…The patients with DPLI in the present study also showed a significantly higher percentage of unilateral dominance. The vertebral artery is reported to be significantly associated with the size of the transverse foramina 12) and its tortuosity is associated with adverse outcomes in young patients with connective tissue disorders 13) . However, there have been no previous studies regarding the differences in the confluent angles between the terminal portion of the vertebral artery and the proximal portion of the BA.…”

Segmental Aortic Stiffness in Children and Young Adults With Connective Tissue Disorders

“…5 To refine medical and surgical guidelines for managing patients with CTDs, novel indicators of vascular phenotype severity such as aortic stiffness and vertebral tortuosity index have been proposed. 6,7 CTD patients are known to have structural changes within the aortic wall including degeneration of elastic fibers, increased collagen content, and smooth muscle cell loss. These changes result in increased aortic wall stiffness, which has been demonstrated by using cardiac MRI (CMR) and other techniques.…”

A case of a single intracranial vertebral artery and cerebral infarct

“…Such a vascular anomaly might be at risk of iatrogenic injury during manual therapy, trauma, positioning for surgery and removal of tumours at the base of the skull. Indeed, a previous study showed that tortuosity of the VA is associated with increased risk of arterial dissection and iatrogenic injury [9].…”