Atsuko Kato1, Eugénie Riesenkampff2, Deane Yim3, Shi-Joon Yoo4, Mike Seed5, Lars Grosse-Wortmann6. 1. Division of Cardiology, The Labatt Family Heart Centre, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada. Electronic address: atsuko.kato@sickkids.ca. 2. Division of Cardiology, The Labatt Family Heart Centre, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada. Electronic address: jenriesenkampff@gmx.net. 3. Division of Cardiology, The Labatt Family Heart Centre, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada. Electronic address: deane.yim@gmail.com. 4. Division of Cardiology, The Labatt Family Heart Centre, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada; Department of Diagnostic Imaging, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada. Electronic address: shi-joon.yoo@sickkids.ca. 5. Division of Cardiology, The Labatt Family Heart Centre, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada; Department of Diagnostic Imaging, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada. Electronic address: mike.seed@sickkids.ca. 6. Division of Cardiology, The Labatt Family Heart Centre, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada; Department of Diagnostic Imaging, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada. Electronic address: lars.grosse-wortmann@sickkids.ca.
Abstract
BACKGROUND: Patients with single ventricle (SV) circulations are at risk for ventricular dysfunction. This study investigates whether there is evidence of increased myocardial fibrosis and myocardial dysfunction in children after the Fontan operation. METHODS: Consecutive children after the Fontan operation who underwent cardiac magnetic resonance (CMR) T1 relaxometry with a modified look-locker inversion recovery approach were included in this retrospective study. Native T1 times (T1) and extracellular volume fractions (ECV) in the free wall of the dominant ventricle (left, SLV; right, SRV) were compared with controls and correlated with hemodynamic and clinical parameters. RESULTS: Twenty-one SV patients (9.7±4.6years; 13 SLV; 8 SRV) and 24 healthy control children (13.9±2.6years, p=0.002) were included. T1 and ECV were higher in SRV patients than in controls (1036±46ms vs 974±27ms, p<0.001; 28±4% vs 22±3%, p=0.002) and SLV patients (978±39ms, p=0.002; 23±5%, p=0.012) while there was no difference between SLV patients and controls. Age at bidirectional cavopulmonary connection was correlated with T1 (R=0.55, p=0.015), while systolic blood pressure (R=-0.68, p<0.001) and body weight (R=-0.54, p=0.012) inversely correlated with ECV. T1 negatively correlated with radial and circumferential strain by CMR feature tracking. CONCLUSIONS: Fontan patients with a SRV show increased CMR markers of diffuse myocardial fibrosis, which are associated with decreased myocardial contractility. Whether their increased fibrosis burden conveys a greater risk for long-term complications in this population remains to be investigated.
BACKGROUND:Patients with single ventricle (SV) circulations are at risk for ventricular dysfunction. This study investigates whether there is evidence of increased myocardial fibrosis and myocardial dysfunction in children after the Fontan operation. METHODS: Consecutive children after the Fontan operation who underwent cardiac magnetic resonance (CMR) T1 relaxometry with a modified look-locker inversion recovery approach were included in this retrospective study. Native T1 times (T1) and extracellular volume fractions (ECV) in the free wall of the dominant ventricle (left, SLV; right, SRV) were compared with controls and correlated with hemodynamic and clinical parameters. RESULTS: Twenty-one SV patients (9.7±4.6years; 13 SLV; 8 SRV) and 24 healthy control children (13.9±2.6years, p=0.002) were included. T1 and ECV were higher in SRV patients than in controls (1036±46ms vs 974±27ms, p<0.001; 28±4% vs 22±3%, p=0.002) and SLV patients (978±39ms, p=0.002; 23±5%, p=0.012) while there was no difference between SLV patients and controls. Age at bidirectional cavopulmonary connection was correlated with T1 (R=0.55, p=0.015), while systolic blood pressure (R=-0.68, p<0.001) and body weight (R=-0.54, p=0.012) inversely correlated with ECV. T1 negatively correlated with radial and circumferential strain by CMR feature tracking. CONCLUSIONS: Fontan patients with a SRV show increased CMR markers of diffuse myocardial fibrosis, which are associated with decreased myocardial contractility. Whether their increased fibrosis burden conveys a greater risk for long-term complications in this population remains to be investigated.
Authors: Michael Huntgeburth; Ingo Germund; Lianne M Geerdink; Narayanswami Sreeram; Floris E A Udink Ten Cate Journal: Cardiovasc Diagn Ther Date: 2019-10
Authors: Joseph J Pagano; Deane Yim; Christopher Z Lam; Shi-Joon Yoo; Mike Seed; Lars Grosse-Wortmann Journal: Radiol Cardiothorac Imaging Date: 2020-08-13
Authors: Matthew J Campbell; Michael D Quartermain; Meryl S Cohen; Jennifer Faerber; Oluwatimelehin Okunowo; Yan Wang; Valerie Capone; Jenna DiFrancesco; Laura Mercer-Rosa; David J Goldberg Journal: Echocardiography Date: 2020-08-13 Impact factor: 1.724
Authors: Alessia Callegari; Simona Marcora; Barbara Burkhardt; Michael Voutat; Christian Johannes Kellenberger; Julia Geiger; Emanuela Regina Valsangiacomo Buechel Journal: Pediatr Cardiol Date: 2021-07-27 Impact factor: 1.655