Adverse fibrosis remodeling and aortopulmonary collateral flow are associated with poor Fontan outcomes

Abstract Background The extent and significance in of cardiac remodeling in Fontan patients are unclear and were the subject of this study. Methods This retrospective cohort study compared cardiovascular magnetic resonance (CMR) imaging markers of cardiac function, myocardial fibrosis, and hemodynam...

Descripción completa

Guardado en:

Detalles Bibliográficos
Autores principales:	Andrea Pisesky, Marjolein J. E. Reichert, Charlotte de Lange, Mike Seed, Shi-Joon Yoo, Christopher Z. Lam, Lars Grosse-Wortmann
Formato:	article
Lenguaje:	EN
Publicado:	BMC 2021
Materias:	Congenital heart disease Fontan Heart failure Fibrosis Magnetic resonance imaging Diseases of the circulatory (Cardiovascular) system RC666-701
Acceso en línea:	https://doaj.org/article/16d91b3be4354ae3afbfbc9671c57739
Etiquetas:	Agregar Etiqueta Sin Etiquetas, Sea el primero en etiquetar este registro!

id	oai:doaj.org-article:16d91b3be4354ae3afbfbc9671c57739
record_format	dspace
spelling	oai:doaj.org-article:16d91b3be4354ae3afbfbc9671c577392021-11-21T12:09:00ZAdverse fibrosis remodeling and aortopulmonary collateral flow are associated with poor Fontan outcomes10.1186/s12968-021-00782-91532-429Xhttps://doaj.org/article/16d91b3be4354ae3afbfbc9671c577392021-11-01T00:00:00Zhttps://doi.org/10.1186/s12968-021-00782-9https://doaj.org/toc/1532-429XAbstract Background The extent and significance in of cardiac remodeling in Fontan patients are unclear and were the subject of this study. Methods This retrospective cohort study compared cardiovascular magnetic resonance (CMR) imaging markers of cardiac function, myocardial fibrosis, and hemodynamics in young Fontan patients to controls. Results Fifty-five Fontan patients and 44 healthy controls were included (median age 14 years (range 7–17 years) vs 13 years (range 4–14 years), p = 0.057). Fontan patients had a higher indexed end-diastolic ventricular volume (EDVI 129 ml/m2 vs 93 ml/m2, p < 0.001), and lower ejection fraction (EF 45% vs 58%, p < 0.001), circumferential (CS − 23.5% vs − 30.8%, p < 0.001), radial (6.4% vs 8.2%, p < 0.001), and longitudinal strain (− 13.3% vs − 24.8%, p < 0.001). Compared to healthy controls, Fontan patients had higher extracellular volume fraction (ECV) (26.3% vs 20.6%, p < 0.001) and native T1 (1041 ms vs 986 ms, p < 0.001). Patients with a dominant right ventricle demonstrated larger ventricles (EDVI 146 ml/m2 vs 120 ml/m2, p = 0.03), lower EF (41% vs 47%, p = 0.008), worse CS (− 20.1% vs − 25.6%, p = 0.003), and a trend towards higher ECV (28.3% versus 24.1%, p = 0.09). Worse EF and CS correlated with longer cumulative bypass (R = − 0.36, p = 0.003 and R = 0.46, p < 0.001), cross-clamp (R = − 0.41, p = 0.001 and R = 0.40, p = 0.003) and circulatory arrest times (R = − 0.42, p < 0.001 and R = 0.27, p = 0.03). T1 correlated with aortopulmonary collateral (APC) flow (R = 0.36, p = 0.009) which, in the linear regression model, was independent of ventricular morphology (p = 0.9) and EDVI (p = 0.2). The composite outcome (cardiac readmission, cardiac reintervention, Fontan failure or any clinically significant arrhythmia) was associated with increased native T1 (1063 ms vs 1026 ms, p = 0.029) and EDVI (146 ml/m2 vs 118 ml/m2, p = 0.013), as well as decreased EF (42% vs 46%, p = 0.045) and worse CS (− 22% vs − 25%, p = 0.029). APC flow (HR 5.5 CI 1.9–16.2, p = 0.002) was independently associated with the composite outcome, independent of ventricular morphology (HR 0.71 CI 0.30–1.69 p = 0.44) and T1 (HR1.006 CI 1.0–1.13, p = 0.07). Conclusions Pediatric Fontan patients have ventricular dysfunction, altered myocardial mechanics and increased fibrotic remodeling. Cumulative exposure to cardiopulmonary bypass and increased aortopulmonary collateral flow are associated with myocardial dysfunction and fibrosis. Cardiac dysfunction, fibrosis, and collateral flow are associated with adverse outcomes.Andrea PiseskyMarjolein J. E. ReichertCharlotte de LangeMike SeedShi-Joon YooChristopher Z. LamLars Grosse-WortmannBMCarticleCongenital heart diseaseFontanHeart failureFibrosisMagnetic resonance imagingDiseases of the circulatory (Cardiovascular) systemRC666-701ENJournal of Cardiovascular Magnetic Resonance, Vol 23, Iss 1, Pp 1-11 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Congenital heart disease Fontan Heart failure Fibrosis Magnetic resonance imaging Diseases of the circulatory (Cardiovascular) system RC666-701
spellingShingle	Congenital heart disease Fontan Heart failure Fibrosis Magnetic resonance imaging Diseases of the circulatory (Cardiovascular) system RC666-701 Andrea Pisesky Marjolein J. E. Reichert Charlotte de Lange Mike Seed Shi-Joon Yoo Christopher Z. Lam Lars Grosse-Wortmann Adverse fibrosis remodeling and aortopulmonary collateral flow are associated with poor Fontan outcomes
description	Abstract Background The extent and significance in of cardiac remodeling in Fontan patients are unclear and were the subject of this study. Methods This retrospective cohort study compared cardiovascular magnetic resonance (CMR) imaging markers of cardiac function, myocardial fibrosis, and hemodynamics in young Fontan patients to controls. Results Fifty-five Fontan patients and 44 healthy controls were included (median age 14 years (range 7–17 years) vs 13 years (range 4–14 years), p = 0.057). Fontan patients had a higher indexed end-diastolic ventricular volume (EDVI 129 ml/m2 vs 93 ml/m2, p < 0.001), and lower ejection fraction (EF 45% vs 58%, p < 0.001), circumferential (CS − 23.5% vs − 30.8%, p < 0.001), radial (6.4% vs 8.2%, p < 0.001), and longitudinal strain (− 13.3% vs − 24.8%, p < 0.001). Compared to healthy controls, Fontan patients had higher extracellular volume fraction (ECV) (26.3% vs 20.6%, p < 0.001) and native T1 (1041 ms vs 986 ms, p < 0.001). Patients with a dominant right ventricle demonstrated larger ventricles (EDVI 146 ml/m2 vs 120 ml/m2, p = 0.03), lower EF (41% vs 47%, p = 0.008), worse CS (− 20.1% vs − 25.6%, p = 0.003), and a trend towards higher ECV (28.3% versus 24.1%, p = 0.09). Worse EF and CS correlated with longer cumulative bypass (R = − 0.36, p = 0.003 and R = 0.46, p < 0.001), cross-clamp (R = − 0.41, p = 0.001 and R = 0.40, p = 0.003) and circulatory arrest times (R = − 0.42, p < 0.001 and R = 0.27, p = 0.03). T1 correlated with aortopulmonary collateral (APC) flow (R = 0.36, p = 0.009) which, in the linear regression model, was independent of ventricular morphology (p = 0.9) and EDVI (p = 0.2). The composite outcome (cardiac readmission, cardiac reintervention, Fontan failure or any clinically significant arrhythmia) was associated with increased native T1 (1063 ms vs 1026 ms, p = 0.029) and EDVI (146 ml/m2 vs 118 ml/m2, p = 0.013), as well as decreased EF (42% vs 46%, p = 0.045) and worse CS (− 22% vs − 25%, p = 0.029). APC flow (HR 5.5 CI 1.9–16.2, p = 0.002) was independently associated with the composite outcome, independent of ventricular morphology (HR 0.71 CI 0.30–1.69 p = 0.44) and T1 (HR1.006 CI 1.0–1.13, p = 0.07). Conclusions Pediatric Fontan patients have ventricular dysfunction, altered myocardial mechanics and increased fibrotic remodeling. Cumulative exposure to cardiopulmonary bypass and increased aortopulmonary collateral flow are associated with myocardial dysfunction and fibrosis. Cardiac dysfunction, fibrosis, and collateral flow are associated with adverse outcomes.
format	article
author	Andrea Pisesky Marjolein J. E. Reichert Charlotte de Lange Mike Seed Shi-Joon Yoo Christopher Z. Lam Lars Grosse-Wortmann
author_facet	Andrea Pisesky Marjolein J. E. Reichert Charlotte de Lange Mike Seed Shi-Joon Yoo Christopher Z. Lam Lars Grosse-Wortmann
author_sort	Andrea Pisesky
title	Adverse fibrosis remodeling and aortopulmonary collateral flow are associated with poor Fontan outcomes
title_short	Adverse fibrosis remodeling and aortopulmonary collateral flow are associated with poor Fontan outcomes
title_full	Adverse fibrosis remodeling and aortopulmonary collateral flow are associated with poor Fontan outcomes
title_fullStr	Adverse fibrosis remodeling and aortopulmonary collateral flow are associated with poor Fontan outcomes
title_full_unstemmed	Adverse fibrosis remodeling and aortopulmonary collateral flow are associated with poor Fontan outcomes
title_sort	adverse fibrosis remodeling and aortopulmonary collateral flow are associated with poor fontan outcomes
publisher	BMC
publishDate	2021
url	https://doaj.org/article/16d91b3be4354ae3afbfbc9671c57739
work_keys_str_mv	AT andreapisesky adversefibrosisremodelingandaortopulmonarycollateralflowareassociatedwithpoorfontanoutcomes AT marjoleinjereichert adversefibrosisremodelingandaortopulmonarycollateralflowareassociatedwithpoorfontanoutcomes AT charlottedelange adversefibrosisremodelingandaortopulmonarycollateralflowareassociatedwithpoorfontanoutcomes AT mikeseed adversefibrosisremodelingandaortopulmonarycollateralflowareassociatedwithpoorfontanoutcomes AT shijoonyoo adversefibrosisremodelingandaortopulmonarycollateralflowareassociatedwithpoorfontanoutcomes AT christopherzlam adversefibrosisremodelingandaortopulmonarycollateralflowareassociatedwithpoorfontanoutcomes AT larsgrossewortmann adversefibrosisremodelingandaortopulmonarycollateralflowareassociatedwithpoorfontanoutcomes
_version_	1718419170807250944

Adverse fibrosis remodeling and aortopulmonary collateral flow are associated with poor Fontan outcomes

Ejemplares similares