Vertebral Tortuosity Is Associated With Increased Rate of Cardiovascular Events in Vascular Ehlers-Danlos Syndrome.

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.

Scar quantification by cardiovascular magnetic resonance as an independent predictor of long-term survival in patients with ischemic heart failure treated by coronary artery bypass graft surgery.

BACKGROUND: Scar burden by late gadolinium enhancement (LGE) cardiovascular magnetic resonance (CMR) is associated with functional recovery after coronary artery bypass surgery (CABG). There is limited data on long-term mortality after CABG based on left ventricular (LV) scar burden. METHODS: Patients who underwent LGE CMR between January 2003 and February 2010 within 1 month prior to CABG were included. A standard 16 segment model was used for scar quantification. A score of 1 for no scar, 2 for ≤ 50 % and 3 for > 50 % transmurality was assigned for each segment. LV scar score (LVSS) defined as the sum of segment scores divided by 16. All-cause mortality was ascertained by social security death index. RESULTS: One hundred ninety-six patients met the inclusion criteria. 185 CMR studies were available. History of prior MI was present in 64 % and prior CABG in 5.4 % of patients. Scar was present in 72 % of patients and median LVEF was 38 %. Over a median follow up of 8.3 years, there were 64 deaths (34.6 %). There was no statistically significant difference in mortality between Scar and No-scar groups (37 % versus 29 %). In the group with scar, a lower scar burden (defined either < 4 segments with scar or based on LVSS) was independently associated with increased survival. CONCLUSION: In patients undergoing surgical revascularization, scar burden is negatively associated with survival in patients with scar. However, there is no difference in survival based on presence or absence of scar alone. CMR prior to CABG adds additional prognostic information.