Late gadolinium enhancement (HR 7.2; 95% CI 1.5-34) and left ventricular hypertrophy (HR 3.0; 95% CI 1.1-8.1) at cardiac MRI independently predicted adverse cardiac events.
Cohort (n=90)
Blinded evaluators
No
Are left ventricular hypertrophy and late gadolinium enhancement on cardiac MRI associated with adverse cardiac events in patients with Fabry disease?
Cardiac MRI findings of left ventricular hypertrophy and late gadolinium enhancement independently predict adverse cardiac events in patients with Fabry disease, with extensive LGE conferring the highest risk.
Effect estimate: HR 7.2 (95% CI 1.5-34)
p-value: p=0.01
Background Cardiac involvement is the leading cause of mortality in patients with Fabry disease. Identification of imaging findings that predict adverse cardiac events is needed to enable identification of high-risk patients. Purpose To establish the prognostic value of cardiac MRI findings in men and women with Fabry disease. Materials and Methods Consecutive women and men with gene-positive Fabry disease who had undergone cardiac MRI at a single large tertiary referral hospital between March 2008 and January 2019 were included in this retrospective cohort study. Evaluators of cardiac MRI studies were blinded to all clinical information. Adverse cardiac events were assessed as a composite end point, defined as ventricular tachycardia, bradycardia requiring device implantation, severe heart failure, and cardiac death. Statistical analysis included Cox proportional hazard models adjusted for age and Mainz Severity Score Index (a measure of the severity of Fabry disease). Results Ninety patients (mean age, 44 years ± 15 standard deviation; 59 women) were evaluated. After a median follow-up period of 3.6 years, the composite end point was reached in 21 patients (incidence rate, 7.6% per year). Left ventricular hypertrophy (LVH) and late gadolinium enhancement (LGE) were independent predictors of the composite end point in adjusted analysis (LVH hazard ratio HR, 3.0; 95% confidence interval CI: 1.1, 8.1; P = .03; and LGE HR, 7.2; 95% CI: 1.5, 34; P = .01). Patients with extensive LGE (≥15% of left ventricular mass) were at highest risk (HR, 12; 95% CI: 2.0, 67; P = .006). Sex did not modify the relationship between the composite end point and any of the cardiac MRI parameters, including LVH (P = .15 for interaction term) and LGE (P = .38 for interaction term). Conclusion Cardiac MRI findings of left ventricular hypertrophy and late gadolinium enhancement can be used to identify patients with Fabry disease who are at high risk of adverse cardiac events. © RSNA, 2019 See also the editorial by Zimmerman in this issue.
Hanneman et al. (Tue,) conducted a cohort in Fabry disease (n=90). Cardiac MRI (Left ventricular hypertrophy and late gadolinium enhancement) was evaluated on Composite of ventricular tachycardia, bradycardia requiring device implantation, severe heart failure, and cardiac death (HR 7.2, 95% CI 1.5-34, p=0.01). Late gadolinium enhancement (HR 7.2; 95% CI 1.5-34) and left ventricular hypertrophy (HR 3.0; 95% CI 1.1-8.1) at cardiac MRI independently predicted adverse cardiac events.