Pre-clinical left ventricular myocardial remodeling in patients with Friedreich's ataxia: A cardiac MRI study

Autor(es)

Takazaki, Karen A. G. ^[1] ; Quinaglia, Thiago ^[2] ; Venancio, Thiago D. ^[1] ; Martinez, Alberto R. M. ^[1] ; Shah, Ravi V. ^{[3, 4]} ; Neilan, Tomas G. ^{[3, 4]} ; Jerosch-Herold, Michael ^{[5, 6]} ; Coelho-Filho, Otavio R. ^[2] ; Franca, Jr., Marcondes C. ^[1]

Afiliação do(s) autor(es)

^[1] Univ Campinas UNICAMP, Sch Med Sci, Dept Neurol, Div Med, Sect Neurol, Campinas, SP - Brazil

^[2] Univ Campinas UNICAMP, Sch Med Sci, Dept Internal Med, Div Med, Sect Cardiol, Div Med, Campinas, SP - Brazil

^[3] Harvard Med Sch, Massachusetts Gen Hosp, Sect Cardiovasc Imaging Res Program, Div Med, Boston, MA 02115 - USA

^[4] Harvard Med Sch, Massachusetts Gen Hosp, Dept Cardiol, Cardiac MR PET CT Program, Boston, MA 02115 - USA

^[5] Harvard Med Sch, Boston, MA 02115 - USA

^[6] Brigham & Womens Hosp, Dept Radiol, Div Med, Sect MRI Phys, 75 Francis St, Boston, MA 02115 - USA

• Tipo de documento: Artigo Científico
• Fonte: PLoS One; v. 16, n. 3 MAR 26 2021.
• Citações Web of Science: 0

Resumo

Background Heart Failure (HF) is the most common cause of death in Friedreich's ataxia (FRDA), an inherited mitochondrial disease. Myocardial fibrosis and myocardial hypertrophy are well-documented autopsy features among FRDA patients with HF. Objectives To leverage the unique tissue characterization features of cardiac magnetic resonance (CMR) for characterizing myocardial remodeling in patients with genetically confirmed FRDA without HF and preserved left ventricular ejection fraction (LVEF > 55%). Methods Twenty-seven FRDA's patients (age 27.6 9.7 years, 15 women) and 10 healthy controls (32.67.3 years, 5 women) underwent a CMR for assessment of LV function, myocardial T1, late gadolinium enhancement (LGE), extracellular volume fraction (ECV), and intracellular water-lifetime (tau(ic)), a marker of cardiomyocyte size. Results As compared to controls, FRDA patients had a preserved LVEF (LVEF: 70.5 +/- 7.4% vs. 63.9 +/- 9.0%, P<0.058), larger LV mass index (LVMASSi: 61 +/- 21.7 vs. 45 +/- 4.2g/m(2), P<0.02), and decreased LV end-diastolic volume index (LVEDVi 53.1 +/- 12.0 vs. 75.7 +/- 16.1ml/m(2), P<0.001), compared with controls. Additionally, ECV and cardiomyocyte size (tau(ic),) were larger in FRDA patients (ECV: 0.36 +/- 0.05 vs. 0.25 +/- 0.02, P<0.001; tau(ic): 0.15 +/- 0.08 vs. 0.06 +/- 0.03 s, P = 0.02). ECV and tau(ic) were positively associated with LV mass-to-volume ratio (ECV: r = 0.57, P = 0.003; tau(ic): r = 0.39; P = 0.05). LVMASSi and cardiomyocyte mass-index {[}(1-ECV).LVMASSi] declined with age at the CMR exam, independent of the age at initial diagnosis. Conclusions LV hypertrophy and concentric LV remodeling in FRDA are associated at the tissue level with an expansion of the ECV and an increase in cardiomyocyte size. The adverse tissue remodeling assessed by ECV and tau(ic) is associated with more severe cardiomyopathy classification, suggesting a role for these markers in tracking disease progression. (AU)

• Processo FAPESP: 15/15402-2 - Caracterização da fibrose intersticial e da hipertrofia dos cardiomiócitos pela ressonância magnética cardíaca: implicações no remodelamento precoce e na transição para insuficiência cardíaca
• Beneficiário: Otávio Rizzi Coelho-Filho
• Modalidade de apoio: Auxílio à Pesquisa - Jovens Pesquisadores
• Processo FAPESP: 13/01766-7 - Contribuição ao diagnóstico, à fisiopatologia e à terapêutica das neuronopatias sensitivas
• Beneficiário: Marcondes Cavalcante Franca Junior
• Modalidade de apoio: Auxílio à Pesquisa - Jovens Pesquisadores
• Processo FAPESP: 16/26209-1 - EMU concedido no processo 2015/15402-2: sistema de ergoespirometria
• Beneficiário: Otávio Rizzi Coelho-Filho
• Modalidade de apoio: Auxílio à Pesquisa - Programa Equipamentos Multiusuários
• Processo FAPESP: 17/03708-5 - EMU concedido no processo 2015/15402-2: equipamento de ecocardiograma
• Beneficiário: Otávio Rizzi Coelho-Filho
• Modalidade de apoio: Auxílio à Pesquisa - Programa Equipamentos Multiusuários