Myocardial fibrosis and its relation to adverse outcome in transposition of the great arteries with a systemic right ventricle

Craig Broberg, Anne Marie Valente, Jennifer Huang, Luke Burchill, Jonathan Holt, Ryan Van Woerkom, Andrew J. Powell, George Pantely, Michael Jerosch-Herold

Research output: Contribution to journalArticle

5 Citations (Scopus)

Abstract

Background: Myocardial dysfunction has been implicated in gradual heart failure in transposition of the great arteries (TGA) with a systemic right ventricle (RV). Fibrosis can be assessed using the extracellular volume fraction (ECV). Our aim was to measure ECV and determine its associations with clinical findings and outcomes. Methods: We prospectively measured ECV in systemic RV subjects (either D-loop after atrial switch or L-loop) and healthy controls. T1 measurements for a single mid-ventricular short-axis plane before and 3, 7, and 15 min after gadolinium contrast were used to quantify systemic ventricular ECV. Individuals with elevated ECV were compared to those without. Results: In 53 TGA subjects (age 34.6 ± 10.3 years, 41% female) the mean ECV for the systemic RV (28.7 ± 4.4%) was significantly higher than the left ventricle in 22 controls (26.1 ± 2.8%, P = 0.0104). Those with an elevated ECV (n = 15, 28.3%) had a higher b-type natriuretic peptide (BNP) (P < 0.011) and a longer 6-min walk distance (P = 0.021), but did not differ by age, arrhythmia history, ventricular volume, function, or circulating collagen byproducts. At follow-up (median 4.4 years), those experiencing major cardiovascular endpoints (new arrhythmia, arrhythmia device, heart failure hospitalization, listing for transplantation, mechanical support, or cardiovascular death, n = 14) had a higher ECV. ECV, age, and BNP were independent predictors of cardiac events in Cox-proportional hazard models. Conclusions: Myocardial fibrosis is common in the systemic RV and associated with a higher BNP. Elevated CMR-derived ECV was associated with adverse clinical outcome. The findings suggest a role of diffuse myocardial fibrosis in clinical deterioration of the systemic RV.

Original languageEnglish (US)
JournalInternational Journal of Cardiology
DOIs
StateAccepted/In press - Jan 1 2018

Fingerprint

Transposition of Great Vessels
Heart Ventricles
Fibrosis
Natriuretic Peptides
Cardiac Arrhythmias
Heart Failure
Equipment Failure
Ventricular Function
Gadolinium
Proportional Hazards Models
Hospitalization
Collagen
Transplantation

Keywords

  • Cardiac magnetic resonance
  • Congenital heart disease
  • Myocardial fibrosis
  • Systemic right ventricle
  • Transposition of the great arteries
  • Ventricular dysfunction

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

Cite this

Myocardial fibrosis and its relation to adverse outcome in transposition of the great arteries with a systemic right ventricle. / Broberg, Craig; Valente, Anne Marie; Huang, Jennifer; Burchill, Luke; Holt, Jonathan; Van Woerkom, Ryan; Powell, Andrew J.; Pantely, George; Jerosch-Herold, Michael.

In: International Journal of Cardiology, 01.01.2018.

Research output: Contribution to journalArticle

Broberg, Craig ; Valente, Anne Marie ; Huang, Jennifer ; Burchill, Luke ; Holt, Jonathan ; Van Woerkom, Ryan ; Powell, Andrew J. ; Pantely, George ; Jerosch-Herold, Michael. / Myocardial fibrosis and its relation to adverse outcome in transposition of the great arteries with a systemic right ventricle. In: International Journal of Cardiology. 2018.
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abstract = "Background: Myocardial dysfunction has been implicated in gradual heart failure in transposition of the great arteries (TGA) with a systemic right ventricle (RV). Fibrosis can be assessed using the extracellular volume fraction (ECV). Our aim was to measure ECV and determine its associations with clinical findings and outcomes. Methods: We prospectively measured ECV in systemic RV subjects (either D-loop after atrial switch or L-loop) and healthy controls. T1 measurements for a single mid-ventricular short-axis plane before and 3, 7, and 15 min after gadolinium contrast were used to quantify systemic ventricular ECV. Individuals with elevated ECV were compared to those without. Results: In 53 TGA subjects (age 34.6 ± 10.3 years, 41{\%} female) the mean ECV for the systemic RV (28.7 ± 4.4{\%}) was significantly higher than the left ventricle in 22 controls (26.1 ± 2.8{\%}, P = 0.0104). Those with an elevated ECV (n = 15, 28.3{\%}) had a higher b-type natriuretic peptide (BNP) (P < 0.011) and a longer 6-min walk distance (P = 0.021), but did not differ by age, arrhythmia history, ventricular volume, function, or circulating collagen byproducts. At follow-up (median 4.4 years), those experiencing major cardiovascular endpoints (new arrhythmia, arrhythmia device, heart failure hospitalization, listing for transplantation, mechanical support, or cardiovascular death, n = 14) had a higher ECV. ECV, age, and BNP were independent predictors of cardiac events in Cox-proportional hazard models. Conclusions: Myocardial fibrosis is common in the systemic RV and associated with a higher BNP. Elevated CMR-derived ECV was associated with adverse clinical outcome. The findings suggest a role of diffuse myocardial fibrosis in clinical deterioration of the systemic RV.",
keywords = "Cardiac magnetic resonance, Congenital heart disease, Myocardial fibrosis, Systemic right ventricle, Transposition of the great arteries, Ventricular dysfunction",
author = "Craig Broberg and Valente, {Anne Marie} and Jennifer Huang and Luke Burchill and Jonathan Holt and {Van Woerkom}, Ryan and Powell, {Andrew J.} and George Pantely and Michael Jerosch-Herold",
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T1 - Myocardial fibrosis and its relation to adverse outcome in transposition of the great arteries with a systemic right ventricle

AU - Broberg, Craig

AU - Valente, Anne Marie

AU - Huang, Jennifer

AU - Burchill, Luke

AU - Holt, Jonathan

AU - Van Woerkom, Ryan

AU - Powell, Andrew J.

AU - Pantely, George

AU - Jerosch-Herold, Michael

PY - 2018/1/1

Y1 - 2018/1/1

N2 - Background: Myocardial dysfunction has been implicated in gradual heart failure in transposition of the great arteries (TGA) with a systemic right ventricle (RV). Fibrosis can be assessed using the extracellular volume fraction (ECV). Our aim was to measure ECV and determine its associations with clinical findings and outcomes. Methods: We prospectively measured ECV in systemic RV subjects (either D-loop after atrial switch or L-loop) and healthy controls. T1 measurements for a single mid-ventricular short-axis plane before and 3, 7, and 15 min after gadolinium contrast were used to quantify systemic ventricular ECV. Individuals with elevated ECV were compared to those without. Results: In 53 TGA subjects (age 34.6 ± 10.3 years, 41% female) the mean ECV for the systemic RV (28.7 ± 4.4%) was significantly higher than the left ventricle in 22 controls (26.1 ± 2.8%, P = 0.0104). Those with an elevated ECV (n = 15, 28.3%) had a higher b-type natriuretic peptide (BNP) (P < 0.011) and a longer 6-min walk distance (P = 0.021), but did not differ by age, arrhythmia history, ventricular volume, function, or circulating collagen byproducts. At follow-up (median 4.4 years), those experiencing major cardiovascular endpoints (new arrhythmia, arrhythmia device, heart failure hospitalization, listing for transplantation, mechanical support, or cardiovascular death, n = 14) had a higher ECV. ECV, age, and BNP were independent predictors of cardiac events in Cox-proportional hazard models. Conclusions: Myocardial fibrosis is common in the systemic RV and associated with a higher BNP. Elevated CMR-derived ECV was associated with adverse clinical outcome. The findings suggest a role of diffuse myocardial fibrosis in clinical deterioration of the systemic RV.

AB - Background: Myocardial dysfunction has been implicated in gradual heart failure in transposition of the great arteries (TGA) with a systemic right ventricle (RV). Fibrosis can be assessed using the extracellular volume fraction (ECV). Our aim was to measure ECV and determine its associations with clinical findings and outcomes. Methods: We prospectively measured ECV in systemic RV subjects (either D-loop after atrial switch or L-loop) and healthy controls. T1 measurements for a single mid-ventricular short-axis plane before and 3, 7, and 15 min after gadolinium contrast were used to quantify systemic ventricular ECV. Individuals with elevated ECV were compared to those without. Results: In 53 TGA subjects (age 34.6 ± 10.3 years, 41% female) the mean ECV for the systemic RV (28.7 ± 4.4%) was significantly higher than the left ventricle in 22 controls (26.1 ± 2.8%, P = 0.0104). Those with an elevated ECV (n = 15, 28.3%) had a higher b-type natriuretic peptide (BNP) (P < 0.011) and a longer 6-min walk distance (P = 0.021), but did not differ by age, arrhythmia history, ventricular volume, function, or circulating collagen byproducts. At follow-up (median 4.4 years), those experiencing major cardiovascular endpoints (new arrhythmia, arrhythmia device, heart failure hospitalization, listing for transplantation, mechanical support, or cardiovascular death, n = 14) had a higher ECV. ECV, age, and BNP were independent predictors of cardiac events in Cox-proportional hazard models. Conclusions: Myocardial fibrosis is common in the systemic RV and associated with a higher BNP. Elevated CMR-derived ECV was associated with adverse clinical outcome. The findings suggest a role of diffuse myocardial fibrosis in clinical deterioration of the systemic RV.

KW - Cardiac magnetic resonance

KW - Congenital heart disease

KW - Myocardial fibrosis

KW - Systemic right ventricle

KW - Transposition of the great arteries

KW - Ventricular dysfunction

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