Manhal Habib1, Arnon Adler1, Kimia Fardfini1, Sara Hoss1, Kate Hanneman1, Ethan J Rowin2, Martin S Maron2, Barry J Maron2, Harry Rakowski1, Raymond H Chan3. 1. Peter Munk Cardiac Centre, Toronto General Hospital, University Health Network, Toronto, Ontario, Canada. 2. Division of Cardiology, Hypertrophic Cardiomyopathy Institute, Tufts Medical Center, Boston, Massachusetts. 3. Peter Munk Cardiac Centre, Toronto General Hospital, University Health Network, Toronto, Ontario, Canada. Electronic address: raymond.chan@utoronto.ca.
Abstract
OBJECTIVES: This study examined fibrosis progression in hypertrophic cardiomyopathy (HCM) patients, as well as its relationship to patient characteristics, clinical outcomes, and its effect on clinical decision making. BACKGROUND: Myocardial fibrosis, as quantified by late gadolinium enhancement (LGE) in cardiac magnetic resonance (CMR), provides valuable prognostic information in patients with HCM. METHODS: A total of 157 patients with HCM were enrolled in this study, with 2 sequential CMR scans separated by an interval of 4.7 ± 1.9 years. RESULTS: At the first CMR session (CMR-1), 70% of patients had LGE compared with 85% at CMR-2 (p = 0.001). The extent of LGE extent increased between the 2 CMR procedures, from 4.0 ± 5.6% to 6.3 ± 7.4% (p < 0.0001), with an average LGE progression rate of 0.5 ± 1.0%/year. LGE mass progression was correlated with higher LGE mass and extent on CMR-1 (p = 0.0017 and p = 0.007, respectively), greater indexed left ventricular (LV) mass (p < 0.0001), greater LV maximal wall thickness (p < 0.0001), apical aneurysm at CMR-1 (p < 0.0001), and lower LV ejection fraction (EF) (p = 0.029). Patients who were more likely to have a higher rate of LGE progression presented with more severe disease at baseline, characterized by LGE extent >8% of LV mass, indexed LV mass >100 g/m2, maximal wall thickness ≥20 mm, LVEF ≤60%, and apical aneurysm. There was a significant correlation between the magnitude of LGE progression and future implantation of insertable cardioverter-defibrillators (p = 0.004), EF deterioration to ≤50% (p < 0.0001), and admission for heart failure (p = 0.0006). CONCLUSIONS: Myocardial fibrosis in patients with HCM is a slowly progressive process. Progression of LGE is significantly correlated with a number of clinical outcomes such as progression to EF ≤50% and heart failure admission. Judicious use of serial CMR with LGE can provide valuable information to help patient management.
OBJECTIVES: This study examined fibrosis progression in hypertrophic cardiomyopathy (HCM) patients, as well as its relationship to patient characteristics, clinical outcomes, and its effect on clinical decision making. BACKGROUND:Myocardial fibrosis, as quantified by late gadolinium enhancement (LGE) in cardiac magnetic resonance (CMR), provides valuable prognostic information in patients with HCM. METHODS: A total of 157 patients with HCM were enrolled in this study, with 2 sequential CMR scans separated by an interval of 4.7 ± 1.9 years. RESULTS: At the first CMR session (CMR-1), 70% of patients had LGE compared with 85% at CMR-2 (p = 0.001). The extent of LGE extent increased between the 2 CMR procedures, from 4.0 ± 5.6% to 6.3 ± 7.4% (p < 0.0001), with an average LGE progression rate of 0.5 ± 1.0%/year. LGE mass progression was correlated with higher LGE mass and extent on CMR-1 (p = 0.0017 and p = 0.007, respectively), greater indexed left ventricular (LV) mass (p < 0.0001), greater LV maximal wall thickness (p < 0.0001), apical aneurysm at CMR-1 (p < 0.0001), and lower LV ejection fraction (EF) (p = 0.029). Patients who were more likely to have a higher rate of LGE progression presented with more severe disease at baseline, characterized by LGE extent >8% of LV mass, indexed LV mass >100 g/m2, maximal wall thickness ≥20 mm, LVEF ≤60%, and apical aneurysm. There was a significant correlation between the magnitude of LGE progression and future implantation of insertable cardioverter-defibrillators (p = 0.004), EF deterioration to ≤50% (p < 0.0001), and admission for heart failure (p = 0.0006). CONCLUSIONS:Myocardial fibrosis in patients with HCM is a slowly progressive process. Progression of LGE is significantly correlated with a number of clinical outcomes such as progression to EF ≤50% and heart failure admission. Judicious use of serial CMR with LGE can provide valuable information to help patient management.
Authors: Sílvia Aguiar Rosa; Boban Thomas; António Fiarresga; Ana Luísa Papoila; Marta Alves; Ricardo Pereira; Gonçalo Branco; Inês Cruz; Pedro Rio; Luis Baquero; Rui Cruz Ferreira; Miguel Mota Carmo; Luís Rocha Lopes Journal: Front Cardiovasc Med Date: 2021-12-17
Authors: Elizabeth W Thompson; Srikant Kamesh Iyer; Michael P Solomon; Zhaohuan Li; Qiang Zhang; Stefan Piechnik; Konrad Werys; Sophia Swago; Brianna F Moon; Zachary B Rodgers; Anya Hall; Rishabh Kumar; Nosheen Reza; Jessica Kim; Alisha Jamil; Benoit Desjardins; Harold Litt; Anjali Owens; Walter R T Witschey; Yuchi Han Journal: J Cardiovasc Magn Reson Date: 2021-10-25 Impact factor: 5.364