Integrating Cardiac MRI Imaging and Multidisciplinary Clinical Care is Associated With Improved Outcomes in Patients With Fabry Disease

• Published in: Current problems in cardiology 2023-02, Vol.48 (2), p.101476-101476, Article 101476
• Main Authors: Perera, Kevin, Kashyap, Niharika, Wang, Kaiming, Omar, Fadya, Prosia, Easter, Thompson, Richard B., Paterson, D. Ian, Fine, Nowell M., White, James A., Khan, Aneal, Oudit, Gavin Y.
• Format: Article
• Language: English
• Subjects: Fabry Disease - complications; Fabry Disease - epidemiology; Fabry Disease - therapy; Heart Diseases; Heart Failure - complications; Humans; Magnetic Resonance Imaging; Quality of Life
• ISSN: 0146-2806; 1535-6280
• DOI: 10.1016/j.cpcardiol.2022.101476

Given the inherent complexities of Fabry disease (FD) and evolving landscape of cardiovascular clinical management, there is no established ideal clinical care model for these patients. We identified clinical factors predictive of increased risk of major adverse cardiac events (MACE) in patients with FD targeted to improve clinical outcomes. Ninety-five patients studied over a median follow-up time of 6.3 years, and 26 patients reached the composite endpoint with a high prevalence of heart failure and cerebrovascular events and no cardiac-related mortality. Patients with MACE had worse health-related quality of life scores. Hypertrophy and presence of myocardial fibrosis increase risk of MACE by 4-5 times, and dyslipidemia increases risk of MACE by 3 times. Early Fabry-specific treatment and close monitoring of comorbidities reduce cardiac complications and mortality. These findings highlight the importance of comprehensive multidisciplinary management to help improve outcomes in FD patients. The combined pathological effects of the hallmarks of Fabry disease pathology leads to changes in heart morphology and function that can be detected by cardiac magnetic resonance imaging (CMR). Presence of left ventricular hypertrophy and late gadolinium enhancement in CMR studies and presence of comorbidities including hypertension and dyslipidemia have prognostic value for MACE. Cerebrovascular events, sever heart failure, and atrial fibrillation were the most frequent major adverse cardiac events. Frequent monitoring and management of risk factors and early intervention with Fabry-specific therapies attenuate disease progression and prevent poor outcomes. [Display omitted]