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

INTERNATIONAL JOURNAL OF CARDIOLOGY

卷:271

Article

Broberg, Craig S.¹  Valente, Anne Marie²  Huang, Jennifer³  Burchill, Luke J.¹  Holt, Jonathan¹  Van Woerkom, Ryan¹  Powell, Andrew J.²  Pantely, George A.¹  Jerosch-Herold, Michael⁴ 

[1] Oregon Hlth & Sci Univ, Knight Cardiovasc Inst, Adult Congenital Heart Dis Program, Portland, OR 97201 USA

[2] Harvard Med Sch, Boston Childrens Hosp, Dept Cardiol, Boston, MA USA

[3] Oregon Hlth & Sci Univ, Div Pediat Cardiol, Portland, OR 97201 USA

[4] Harvard Med Sch, Brigham & Womens Hosp, Dept Radiol, Boston, MA USA

关键词: Congenital heart disease;    Myocardial fibrosis;    Ventricular dysfunction;    Transposition of the great arteries;    Systemic right ventricle;    Cardiac magnetic resonance;   

DOI  :  10.1016/j.ijcard.2018.04.089

来源: Elsevier

【 摘 要 】

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. (c) 2018 Elsevier B.V. All rights reserved.

【 授权许可】

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