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...
| Main Authors: | , , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Elsevier
2021-11-01
|
| Series: | Journal of Cardiovascular Magnetic Resonance |
| Subjects: | |
| Online Access: | https://doi.org/10.1186/s12968-021-00782-9 |
| _version_ | 1797203210950148096 |
|---|---|
| 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 |
| collection | DOAJ |
| 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. |
| first_indexed | 2024-04-24T08:15:43Z |
| format | Article |
| id | doaj.art-16d91b3be4354ae3afbfbc9671c57739 |
| institution | Directory Open Access Journal |
| issn | 1532-429X |
| language | English |
| last_indexed | 2024-04-24T08:15:43Z |
| publishDate | 2021-11-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Journal of Cardiovascular Magnetic Resonance |
| spelling | doaj.art-16d91b3be4354ae3afbfbc9671c577392024-04-17T03:43:54ZengElsevierJournal of Cardiovascular Magnetic Resonance1532-429X2021-11-0123111110.1186/s12968-021-00782-9Adverse fibrosis remodeling and aortopulmonary collateral flow are associated with poor Fontan outcomesAndrea Pisesky0Marjolein J. E. Reichert1Charlotte de Lange2Mike Seed3Shi-Joon Yoo4Christopher Z. Lam5Lars Grosse-Wortmann6Department of Paediatrics, Division of Cardiology, The Hospital for Sick Children, University of Toronto, Labatt Family Heart CenterDepartment of Paediatrics, Division of Cardiology, The Hospital for Sick Children, University of Toronto, Labatt Family Heart CenterDivision of Radiology and Nuclear Medicine, Pediatric section, Rikshospitalet, Oslo University HospitalDepartment of Paediatrics, Division of Cardiology, The Hospital for Sick Children, University of Toronto, Labatt Family Heart CenterDepartment of Paediatrics, Division of Cardiology, The Hospital for Sick Children, University of Toronto, Labatt Family Heart CenterDepartment of Diagnostic Imaging, The Hospital for Sick Children, University of TorontoDepartment of Paediatrics, Division of Cardiology, The Hospital for Sick Children, University of Toronto, Labatt Family Heart CenterAbstract 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.https://doi.org/10.1186/s12968-021-00782-9Congenital heart diseaseFontanHeart failureFibrosisMagnetic resonance imaging |
| spellingShingle | 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 Journal of Cardiovascular Magnetic Resonance Congenital heart disease Fontan Heart failure Fibrosis Magnetic resonance imaging |
| title | 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_short | 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 |
| topic | Congenital heart disease Fontan Heart failure Fibrosis Magnetic resonance imaging |
| url | https://doi.org/10.1186/s12968-021-00782-9 |
| 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 |