Cardiac MRI in patients with Fontan circulation: assessing risk factors for adverse outcomes

Background Cardiac magnetic resonance (CMR) imaging provides critical insight into the prognosis of Fontan patients, enhancing our understanding of their long-term outcomes. This study aimed to investigate the prognostic role of CMR in a carefully selected cohort of Fontan patients with the highest...

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Main Authors: Juha Peltonen, Riitta Paakkanen, Tiina Ojala, Sabina Ericsson, Marko Taipale, Emmi Helle, Alma Kormi, Teemu Vepsäläinen, Ilkka Mattila, Tommi Pätilä, Laura Martelius
Format: Article
Language:English
Published: BMJ Publishing Group 2025-05-01
Series:Open Heart
Online Access:https://openheart.bmj.com/content/12/1/e003306.full
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author Juha Peltonen
Riitta Paakkanen
Tiina Ojala
Sabina Ericsson
Marko Taipale
Emmi Helle
Alma Kormi
Teemu Vepsäläinen
Ilkka Mattila
Tommi Pätilä
Laura Martelius
author_facet Juha Peltonen
Riitta Paakkanen
Tiina Ojala
Sabina Ericsson
Marko Taipale
Emmi Helle
Alma Kormi
Teemu Vepsäläinen
Ilkka Mattila
Tommi Pätilä
Laura Martelius
author_sort Juha Peltonen
collection DOAJ
description Background Cardiac magnetic resonance (CMR) imaging provides critical insight into the prognosis of Fontan patients, enhancing our understanding of their long-term outcomes. This study aimed to investigate the prognostic role of CMR in a carefully selected cohort of Fontan patients with the highest initial likelihood of survival.Methods This retrospective nationwide cohort study included 148 Fontan patients who underwent post-Fontan CMR imaging in Finland between 2017 and 2023. The primary endpoint was death or listing for heart transplant. The secondary endpoint was myocardial fibrosis determined by native T1 mapping measured by CMR.Results The median time from the Fontan procedure to CMR examination was 10.8 years, with a median post-CMR follow-up of 2.55 years. Six patients (4.1%) reached the primary endpoint. Significant haemodynamic risk factors for the primary endpoint included worse global longitudinal strain (p=0.03), worse global circumferential strain (p<0.001) and reduced ejection fraction (p=0.04). Notably, patients with decreased myocardial function showed higher native T1-mapping values. Additional clinical risk factors that were associated with the primary endpoint included arrhythmias (p=0.01), protein-losing enteropathy (p=0.01), New York Heart Association functional class ≥2 (p<0.001) and liver cirrhosis (p=0.01).Conclusions CMR provides critical insights into long-term outcomes in Fontan patients. In our prioritised cohort, characterised by an initially high likelihood of survival, the observed risks of adverse outcomes corroborate findings from higher mortality cohorts. This underscores the importance of myocardial function and native myocardial T1 mapping in risk assessment, reaffirming CMR’s role in effective risk stratification for this population.
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spelling doaj-art-e7ccc64098e546ca86d8ab84058221542025-08-20T01:55:31ZengBMJ Publishing GroupOpen Heart2053-36242025-05-0112110.1136/openhrt-2025-003306Cardiac MRI in patients with Fontan circulation: assessing risk factors for adverse outcomesJuha Peltonen0Riitta Paakkanen1Tiina Ojala2Sabina Ericsson3Marko Taipale4Emmi Helle5Alma Kormi6Teemu Vepsäläinen7Ilkka Mattila8Tommi Pätilä9Laura Martelius10HUS Diagnostic Center, Radiology, Helsinki University Hospital and University of Helsinki, Helsinki, FinlandNew Children’s Hospital Pediatric Research Center, Helsinki University Hospital, Helsinki, FinlandNew Children’s Hospital Pediatric Research Center, Helsinki University Hospital, Helsinki, FinlandNew Children’s Hospital Pediatric Research Center, Helsinki University Hospital, Helsinki, FinlandNew Children’s Hospital Pediatric Research Center, Helsinki University Hospital, Helsinki, FinlandNew Children’s Hospital Pediatric Research Center, Helsinki University Hospital, Helsinki, FinlandNew Children’s Hospital Pediatric Research Center, Helsinki University Hospital, Helsinki, FinlandNew Children’s Hospital Pediatric Research Center, Helsinki University Hospital, Helsinki, FinlandDepartment of Cardiac and Transplantation Surgery, New Children’s Hospital, Helsinki University Hospital and University of Helsinki, Helsinki, FinlandDepartment of Cardiac and Transplantation Surgery, New Children’s Hospital, Helsinki University Hospital and University of Helsinki, Helsinki, FinlandHUS Diagnostic Center, Radiology, Helsinki University Hospital and University of Helsinki, Helsinki, FinlandBackground Cardiac magnetic resonance (CMR) imaging provides critical insight into the prognosis of Fontan patients, enhancing our understanding of their long-term outcomes. This study aimed to investigate the prognostic role of CMR in a carefully selected cohort of Fontan patients with the highest initial likelihood of survival.Methods This retrospective nationwide cohort study included 148 Fontan patients who underwent post-Fontan CMR imaging in Finland between 2017 and 2023. The primary endpoint was death or listing for heart transplant. The secondary endpoint was myocardial fibrosis determined by native T1 mapping measured by CMR.Results The median time from the Fontan procedure to CMR examination was 10.8 years, with a median post-CMR follow-up of 2.55 years. Six patients (4.1%) reached the primary endpoint. Significant haemodynamic risk factors for the primary endpoint included worse global longitudinal strain (p=0.03), worse global circumferential strain (p<0.001) and reduced ejection fraction (p=0.04). Notably, patients with decreased myocardial function showed higher native T1-mapping values. Additional clinical risk factors that were associated with the primary endpoint included arrhythmias (p=0.01), protein-losing enteropathy (p=0.01), New York Heart Association functional class ≥2 (p<0.001) and liver cirrhosis (p=0.01).Conclusions CMR provides critical insights into long-term outcomes in Fontan patients. In our prioritised cohort, characterised by an initially high likelihood of survival, the observed risks of adverse outcomes corroborate findings from higher mortality cohorts. This underscores the importance of myocardial function and native myocardial T1 mapping in risk assessment, reaffirming CMR’s role in effective risk stratification for this population.https://openheart.bmj.com/content/12/1/e003306.full
spellingShingle Juha Peltonen
Riitta Paakkanen
Tiina Ojala
Sabina Ericsson
Marko Taipale
Emmi Helle
Alma Kormi
Teemu Vepsäläinen
Ilkka Mattila
Tommi Pätilä
Laura Martelius
Cardiac MRI in patients with Fontan circulation: assessing risk factors for adverse outcomes
Open Heart
title Cardiac MRI in patients with Fontan circulation: assessing risk factors for adverse outcomes
title_full Cardiac MRI in patients with Fontan circulation: assessing risk factors for adverse outcomes
title_fullStr Cardiac MRI in patients with Fontan circulation: assessing risk factors for adverse outcomes
title_full_unstemmed Cardiac MRI in patients with Fontan circulation: assessing risk factors for adverse outcomes
title_short Cardiac MRI in patients with Fontan circulation: assessing risk factors for adverse outcomes
title_sort cardiac mri in patients with fontan circulation assessing risk factors for adverse outcomes
url https://openheart.bmj.com/content/12/1/e003306.full
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