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

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

Research output: Contribution to journalArticle

3 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. T 1 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)
Pages (from-to)60-65
Number of pages6
JournalInternational Journal of Cardiology
Volume271
DOIs
StatePublished - Nov 15 2018
Externally publishedYes

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

In: International Journal of Cardiology, Vol. 271, 15.11.2018, p. 60-65.

Research output: Contribution to journalArticle

Broberg, Craig S. ; Valente, Anne Marie ; Huang, Jennifer ; Burchill, Luke J. ; Holt, Jonathan ; Van Woerkom, Ryan ; Powell, Andrew J. ; Pantely, George A. ; 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 ; Vol. 271. pp. 60-65.
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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. T 1 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 = "Broberg, {Craig S.} and Valente, {Anne Marie} and Jennifer Huang and Burchill, {Luke J.} and Jonathan Holt and {Van Woerkom}, Ryan and Powell, {Andrew J.} and Pantely, {George A.} 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 S.

AU - Valente, Anne Marie

AU - Huang, Jennifer

AU - Burchill, Luke J.

AU - Holt, Jonathan

AU - Van Woerkom, Ryan

AU - Powell, Andrew J.

AU - Pantely, George A.

AU - Jerosch-Herold, Michael

PY - 2018/11/15

Y1 - 2018/11/15

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. T 1 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. T 1 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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