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Myocardial fibrosis and its relation to adverse outcome in transposition of the great arteries with a systemic right ventricle.
Int J Cardiol 2018; 271:60-65IJ

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.

Authors+Show Affiliations

Adult Congenital Heart Disease Program, Knight Cardiovascular Institute, Oregon Health & Science University, Portland, OR, United States. Electronic address: brobergc@ohsu.edu.Department of Cardiology, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA. Electronic address: Anne.Valente@cardio.chboston.org.Division of Pediatric Cardiology, Oregon Health & Science University, Portland, OR, United States. Electronic address: huangje@ohsu.edu.Adult Congenital Heart Disease Program, Knight Cardiovascular Institute, Oregon Health & Science University, Portland, OR, United States.Adult Congenital Heart Disease Program, Knight Cardiovascular Institute, Oregon Health & Science University, Portland, OR, United States. Electronic address: holjo@ohsu.edu.Adult Congenital Heart Disease Program, Knight Cardiovascular Institute, Oregon Health & Science University, Portland, OR, United States. Electronic address: vanwoerr@ohsu.edu.Department of Cardiology, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA. Electronic address: andrew.powell@cardio.chboston.org.Adult Congenital Heart Disease Program, Knight Cardiovascular Institute, Oregon Health & Science University, Portland, OR, United States. Electronic address: pantelyg@ohsu.edu.Department of Radiology, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, United States.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

30223379

Citation

Broberg, Craig S., et al. "Myocardial Fibrosis and Its Relation to Adverse Outcome in Transposition of the Great Arteries With a Systemic Right Ventricle." International Journal of Cardiology, vol. 271, 2018, pp. 60-65.
Broberg CS, Valente AM, Huang J, et al. Myocardial fibrosis and its relation to adverse outcome in transposition of the great arteries with a systemic right ventricle. Int J Cardiol. 2018;271:60-65.
Broberg, C. S., Valente, A. M., Huang, J., Burchill, L. J., Holt, J., Van Woerkom, R., ... Jerosch-Herold, M. (2018). Myocardial fibrosis and its relation to adverse outcome in transposition of the great arteries with a systemic right ventricle. International Journal of Cardiology, 271, pp. 60-65. doi:10.1016/j.ijcard.2018.04.089.
Broberg CS, et al. Myocardial Fibrosis and Its Relation to Adverse Outcome in Transposition of the Great Arteries With a Systemic Right Ventricle. Int J Cardiol. 2018 Nov 15;271:60-65. PubMed PMID: 30223379.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR 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 - 2018/01/23/received PY - 2018/04/05/revised PY - 2018/04/20/accepted PY - 2019/11/15/pmc-release PY - 2018/9/19/entrez PY - 2018/9/19/pubmed PY - 2019/3/12/medline KW - Cardiac magnetic resonance KW - Congenital heart disease KW - Myocardial fibrosis KW - Systemic right ventricle KW - Transposition of the great arteries KW - Ventricular dysfunction SP - 60 EP - 65 JF - International journal of cardiology JO - Int. J. Cardiol. VL - 271 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. SN - 1874-1754 UR - https://www.unboundmedicine.com/medline/citation/30223379/Myocardial_fibrosis_and_its_relation_to_adverse_outcome_in_transposition_of_the_great_arteries_with_a_systemic_right_ventricle L2 - https://linkinghub.elsevier.com/retrieve/pii/S0167-5273(18)30535-7 DB - PRIME DP - Unbound Medicine ER -