Right ventricular insertion site fibrosis in a dilated cardiomyopathy referral population: phenotypic associations and value for the prediction of heart failure admission or death

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dc.contributor.authorMikami, Yoko
dc.contributor.authorCornhill, Aidan
dc.contributor.authorDykstra, Steven
dc.contributor.authorSatriano, Alessandro
dc.contributor.authorHansen, Reis
dc.contributor.authorFlewitt, Jacqueline
dc.contributor.authorSeib, Michelle
dc.contributor.authorRivest, Sandra
dc.contributor.authorSandonato, Rosa
dc.contributor.authorLydell, Carmen P.
dc.contributor.authorHowarth, Andrew G.
dc.contributor.authorHeydari, Bobak
dc.contributor.authorMerchant, Naeem
dc.contributor.authorFine, Nowell
dc.contributor.authorWhite, James A.
dc.date.accessioned2021-06-20T00:03:23Z
dc.date.available2021-06-20T00:03:23Z
dc.date.issued2021-06-17
dc.date.updated2021-06-20T00:03:22Z
dc.description.abstractAbstract Background Dilated cardiomyopathy (DCM) is increasingly recognized as a heterogenous disease with distinct phenotypes on late gadolinium enhancement (LGE) cardiovascular magnetic resonance (CMR) imaging. While mid-wall striae (MWS) fibrosis is a widely recognized phenotypic risk marker, other fibrosis patterns are prevalent but poorly defined. Right ventricular (RV) insertion (RVI) site fibrosis is commonly seen, but without objective criteria has been considered a non-specific finding. In this study we developed objective criteria for RVI fibrosis and studied its clinical relevance in a large cohort of patients with DCM. Methods We prospectively enrolled 645 DCM patients referred for LGE-CMR. All underwent standardized imaging protocols and baseline health evaluations. LGE images were blindly scored using objective criteria, inclusive of RVI site and MWS fibrosis. Associations between LGE patterns and CMR-based markers of adverse chamber remodeling were evaluated. Independent associations of LGE fibrosis patterns with the primary composite clinical outcome of heart failure admission or death were determined by multivariable analysis. Results The mean age was 56 ± 14 (28% female) with a mean left ventricular (LV) ejection fraction (LVEF) of 37%. At a median of 1061 days, 129 patients (20%) experienced the primary outcome. Any abnormal LGE was present in 306 patients (47%), inclusive of 274 (42%) meeting criteria for RVI site fibrosis and 167 (26%) for MWS fibrosis. All with MWS fibrosis showed RVI site fibrosis. Solitary RVI site fibrosis was associated with higher bi-ventricular volumes [LV end-systolic volume index (78 ± 39 vs. 66 ± 33 ml/m2, p = 0.01), RV end-diastolic volume index (94 ± 28 vs. 84 ± 22 ml/m2 (p < 0.01), RV end-systolic volume index (56 ± 26 vs. 45 ± 17 ml/m2, p < 0.01)], lower bi-ventricular function [LVEF 35 ± 12 vs. 39 ± 10% (p < 0.01), RV ejection fraction (RVEF) 43 ± 12 vs. 48 ± 10% (p < 0.01)], and higher extracellular volume (ECV). Patient with solitary RVI site fibrosis experienced a non-significant 1.4-fold risk of the primary outcome, increasing to a significant 2.6-fold risk when accompanied by MWS fibrosis. Conclusions RVI site fibrosis in the absence of MWS fibrosis is associated with bi-ventricular remodelling and intermediate risk of heart failure admission or death. Our study findings suggest RVI site fibrosis to be pre-requisite for the incremental development of MWS fibrosis, a more advanced phenotype associated with greater LV remodeling and risk of clinical events.
dc.identifier.citationJournal of Cardiovascular Magnetic Resonance. 2021 Jun 17;23(1):79
dc.identifier.doihttps://doi.org/10.1186/s12968-021-00761-0
dc.identifier.urihttp://hdl.handle.net/1880/113510
dc.language.rfc3066en
dc.rights.holderThe Author(s)
dc.titleRight ventricular insertion site fibrosis in a dilated cardiomyopathy referral population: phenotypic associations and value for the prediction of heart failure admission or death
dc.typeJournal Article
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