Nadya Al-Wakeel-Marquard1, Franziska Seidel2, Christopher Herbst3, Jirko Kühnisch4, Titus Kuehne5, Felix Berger2, Sabine Klaassen6, Daniel R Messroghli7. 1. German Heart Center Berlin, Department of Congenital Heart Disease and Pediatric Cardiology, Augustenburger Platz 1, 13353 Berlin, Germany; Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Institute for Imaging Science and Computational Modelling in Cardiovascular Medicine, Augustenburger Platz 1, 13353 Berlin, Germany; DZHK (German Centre for Cardiovascular Research), partner site Berlin. Electronic address: alwakeel@dhzb.de. 2. German Heart Center Berlin, Department of Congenital Heart Disease and Pediatric Cardiology, Augustenburger Platz 1, 13353 Berlin, Germany; DZHK (German Centre for Cardiovascular Research), partner site Berlin; Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Department of Pediatrics, Division Cardiology, Augustenburger Platz 1, 13353 Berlin, Germany. 3. German Heart Center Berlin, Department of Congenital Heart Disease and Pediatric Cardiology, Augustenburger Platz 1, 13353 Berlin, Germany; DZHK (German Centre for Cardiovascular Research), partner site Berlin; Experimental and Clinical Research Center, a joint cooperation between the Charité Medical Faculty and the Max-Delbrück-Center for Molecular Medicine, Lindenberger Weg 80, 13125 Berlin, Germany. 4. DZHK (German Centre for Cardiovascular Research), partner site Berlin; Experimental and Clinical Research Center, a joint cooperation between the Charité Medical Faculty and the Max-Delbrück-Center for Molecular Medicine, Lindenberger Weg 80, 13125 Berlin, Germany. 5. German Heart Center Berlin, Department of Congenital Heart Disease and Pediatric Cardiology, Augustenburger Platz 1, 13353 Berlin, Germany; Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Institute for Imaging Science and Computational Modelling in Cardiovascular Medicine, Augustenburger Platz 1, 13353 Berlin, Germany; DZHK (German Centre for Cardiovascular Research), partner site Berlin. 6. DZHK (German Centre for Cardiovascular Research), partner site Berlin; Experimental and Clinical Research Center, a joint cooperation between the Charité Medical Faculty and the Max-Delbrück-Center for Molecular Medicine, Lindenberger Weg 80, 13125 Berlin, Germany; Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Department of Pediatrics, Division Cardiology, Augustenburger Platz 1, 13353 Berlin, Germany. 7. DZHK (German Centre for Cardiovascular Research), partner site Berlin; German Heart Center Berlin, Department of Internal Medicine and Cardiology, Augustenburger Platz 1, 13353 Berlin, Germany; Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Department of Cardiology, Augustenburger Platz 1, 13353 Berlin, Germany.
Abstract
BACKGROUND: In adult cardiomyopathy (CM), diffuse myocardial fibrosis is associated with adverse clinical outcome. However, its relevance in pediatric patients remains relatively unknown. The study aimed to evaluate myocardial extracellular volume (ECV) reflecting diffuse myocardial fibrosis with cardiovascular magnetic resonance (CMR) T1 mapping, and to analyze correlations with clinical and functional data in children and adolescents with different CM phenotypes. METHODS: Patients with primary dilated (DCM), hypertrophic (HCM) or left ventricular non-compaction CM (LVNC) were prospectively enrolled and compared with healthy controls. Study participants underwent standardized CMR with modified Look-Locker Inversion recovery (MOLLI) T1 mapping. RESULTS: In total, 33 patients (median age 12.0 years; DCM: n = 10, HCM: n = 13; LVNC: n = 10) and 7 controls (14.5 years) were included. DCM: ECV was higher than in controls (38.1 ± 7.5% vs. 27.2 ± 3.6%; p = 0.014). Patients with elevated ECV were younger than those with normal values (p = 0.044). ECV correlated with N-terminal pro brain natriuretic peptide (r = 0.66, p = 0.038), left ventricular ejection fraction (r = -0.63, p = 0.053), and stroke volume of left (r = -0.75, p = 0.013) and right ventricle (r = -0.67, p = 0.033). During a median follow-up of 25.3 months, 3 patients underwent heart transplantation (HTx), and 2 were listed for HTx. All 5 patients had elevated ECV. HCM/LVNC: ECV was within normal range in HCM (25.5 ± 4.5%) and LVNC (29.6 ± 4.2), and was not related with clinical and/or functional parameters. CONCLUSIONS: Our results indicate an increased burden of diffuse myocardial fibrosis in relation with younger age in pediatric DCM. ECV was associated with clinical and biventricular functional markers of heart failure in DCM.
BACKGROUND: In adult cardiomyopathy (CM), diffuse myocardial fibrosis is associated with adverse clinical outcome. However, its relevance in pediatric patients remains relatively unknown. The study aimed to evaluate myocardial extracellular volume (ECV) reflecting diffuse myocardial fibrosis with cardiovascular magnetic resonance (CMR) T1 mapping, and to analyze correlations with clinical and functional data in children and adolescents with different CM phenotypes. METHODS:Patients with primary dilated (DCM), hypertrophic (HCM) or left ventricular non-compaction CM (LVNC) were prospectively enrolled and compared with healthy controls. Study participants underwent standardized CMR with modified Look-Locker Inversion recovery (MOLLI) T1 mapping. RESULTS: In total, 33 patients (median age 12.0 years; DCM: n = 10, HCM: n = 13; LVNC: n = 10) and 7 controls (14.5 years) were included. DCM: ECV was higher than in controls (38.1 ± 7.5% vs. 27.2 ± 3.6%; p = 0.014). Patients with elevated ECV were younger than those with normal values (p = 0.044). ECV correlated with N-terminal pro brain natriuretic peptide (r = 0.66, p = 0.038), left ventricular ejection fraction (r = -0.63, p = 0.053), and stroke volume of left (r = -0.75, p = 0.013) and right ventricle (r = -0.67, p = 0.033). During a median follow-up of 25.3 months, 3 patients underwent heart transplantation (HTx), and 2 were listed for HTx. All 5 patients had elevated ECV. HCM/LVNC: ECV was within normal range in HCM (25.5 ± 4.5%) and LVNC (29.6 ± 4.2), and was not related with clinical and/or functional parameters. CONCLUSIONS: Our results indicate an increased burden of diffuse myocardial fibrosis in relation with younger age in pediatric DCM. ECV was associated with clinical and biventricular functional markers of heart failure in DCM.
Authors: Franziska Seidel; Titus Kuehne; Sebastian Kelle; Patrick Doeblin; Victoria Zieschang; Carsten Tschoepe; Nadya Al-Wakeel-Marquard; Sarah Nordmeyer Journal: ESC Heart Fail Date: 2021-10-27