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Published on: December 11, 2017
Left Ventricular Pacing-Associated Cardiomyopathy Revealing Myocardial Vulnerability in Congenital Complete Heart
Sachie Kaneko1,2, Aya Miyazaki2,3, Nao Inoue2,3
1Department of Pediatrics, Seirei Hamamatsu General Hospital, Hamamatsu, Japan.
Background:
Mechanisms underlying cardiac manifestations of immune-mediated congenital complete heart block (CCHB) remain unclear. Dilated cardiomyopathy (DCM) in immune-mediated CCHB has been associated with RV pacing, whereas reports of LV pacing-associated DCM are lacking.
Case Summary:
A girl with immune-mediated CCHB showed endocardial fibroelastosis (EFE) at 20 weeks of gestation before heart block developed at 22 weeks. After LV basal-lateral pacing, she developed DCM with LV dyssynchrony. Cardiac resynchronization therapy (CRT) improved LV function and induced reverse remodeling.
Conclusions:
Myocardial injury may precede conduction system involvement in immune-mediated CCHB. Subclinical immune-mediated myocardial vulnerability and pacing-related dyssynchrony may contribute to DCM progression.
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