Prognostic Significance of Cardiac Magnetic Resonance Imaging Late Gadolinium Enhancement in Fabry Disease

Cardiac involvement is the leading cause of mortality in Fabry disease (FD).Late gadolinium enhancement (LGE) on cardiac magnetic resonance imaging predicts adverse cardiac events in other cardiomyopathies.However, it is unclear whether LGE is a significant predictor of adverse cardiac events in FD.The purpose of this study was to evaluate the prognostic significance of the presence and extent of LGE in FD.This retrospective cohort study was approved by the institutional research ethics board.The requirement for written informed consent was waived.All patients with gene-positive FD who had undergone cardiac magnetic resonance imaging with LGE between March 2008 and March 2018 and had clinical follow-up at our institution were included.Magnetic resonance imaging studies were performed with 1.5-or 3-T scanners (MAGNETOM Avanto or Skyra; Siemens Healthcare, Erlangen, Germany).Multiplane LGE images were acquired 12 to 15 minutes after intravenous contrast administration and were visually evaluated for the presence of LGE.Left ventricular endocardial and epicardial borders were contoured on short-axis LGE images to assess the extent of LGE using a signal intensity threshold of 4 SDs, expressed as a percentage of myocardial mass (Circle cmr42; Circle Cardiovascular Imaging, Calgary, Canada).The primary end point was defined as the composite of ventricular tachycardia (VT), bradycardia, heart failure, and cardiac death.Nonsustained VT and sustained VT were defined as ≥3 consecutive beats arising below the atrioventricular node with an inter-beat (RR) interval of >100 bpm lasting <30 and ≥30 seconds, respectively.Bradycardia was defined as a heart rate <60 bpm requiring device implantation for pacing.Heart failure was defined as the development of New York Heart Association functional class III/IV symptoms.Cardiac death was classified as sudden or heart failure-related death.Individual adverse cardiac events were evaluated as secondary end points.Patients without events were censored at the time of their last clinical follow-up.Statistical analysis was performed with STATA version 14.1 (StataCorp, College Station, TX).A 2-tailed value of P<0.05 was considered statistically significant.Time-to-event survival analysis using univariable Cox proportional hazard models were used to estimate the hazard ratio (HR) for the presence and extent of LGE.Eighty-two patients were included in the study with 3.8±2.8years of followup (mean age, 44.7±14.8years; 32.9% male).LGE was present in 34 patients (41.5%) with a mean LGE extent of 4.4±7.8%.Overall, 16 patients (19.5%) reached the primary end point with an incidence rate of 6.1%/y: 13 of 34 patients (38.2%) with LGE compared with 3 of 48 patients (6.3%) without LGE (HR, 7.35; 95% CI, 2.09-25.89;P=0.002; Table).The risk of the primary end point increased with the extent of LGE (HR, 1.26/5% increase in LGE; 95% CI, 1.06-1.50;P=0.008).

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