TY - JOUR
T1 - Left ventricular fibro-fatty replacement in arrhythmogenic right ventricular dysplasia/cardiomyopathy
T2 - prevalence, patterns, and association with arrhythmias
AU - Zghaib, Tarek
AU - Te Riele, Anneline S.J.M.
AU - James, Cynthia A.
AU - Rastegar, Neda
AU - Murray, Brittney
AU - Tichnell, Crystal
AU - Halushka, Marc K.
AU - Bluemke, David Alan
AU - Tandri, Harikrishna
AU - Calkins, Hugh
AU - Kamel, Ihab R.
AU - Zimmerman, Stefan Loy
N1 - Funding Information:
The Johns Hopkins ARVD/C Program is supported the Dr. Francis P. Chiaramonte Private Foundation and Foundation Leducq (16 CVD 02) (all to HC). The Johns Hopkins ARVD/C Program is supported by the Leyla Erkan Family Fund for ARVD Research, the Dr. Francis P. Chiramonte Private Foundation, the Dr. Satish, Rupal, and Robin Shah ARVD Fund at Johns Hopkins, the Bogle Foundation, the Healing Hearts Foundation, the Campanella family, the Patrick J. Harrison Family, the Peter French Memorial Foundation, and the Wilmerding Endowments. The authors also wish to acknowledge funding from Foundation Leducq (16 CVD 02 to HC), the Netherlands Heart Foundation (2015T058 to AtR) and the Cardiovascular Research Netherlands CVON-PREDICT (Young Talent Program to AtR). Stefan L Zimmerman receives salary support as an advisor to Siemens Healthcare. The Johns Hopkins arrhythmogenic right ventricular cardiomyopathy/dysplasia program receives research support from Boston Scientific. The rest of the authors have no financial disclosures related to this present work. The sponsors or funders had no role in the design and conduct of the study, in the collection, analysis, and interpretation of the data, and in the preparation, review, or approval of the manuscript.
Publisher Copyright:
© 2021, The Author(s).
PY - 2021/12
Y1 - 2021/12
N2 - Background: Left ventricular (LV) fibrofatty infiltration in arrhythmogenic right ventricular (RV) dysplasia/cardiomyopathy (ARVD/C) has been reported, however, detailed cardiovascular magnetic resonance (CMR) characteristics and association with outcomes are uncertain. We aim to describe LV findings on CMR in ARVD/C patients and their relationship with arrhythmic outcomes. Methods: CMR of 73 subjects with ARVD/C according to the 2010 Task Force Criteria (TFC) were analyzed for LV involvement, defined as ≥ 1 of the following features: LV wall motion abnormality, LV late gadolinium enhancement (LGE), LV fat infiltration, or LV ejection fraction (LVEF) < 50%. Ventricular volumes and function, regional wall motion abnormalities, and the presence of ventricular fat or fibrosis were recorded. Findings on CMR were correlated with arrhythmic outcomes. Results: Of the 73 subjects, 50.7% had CMR evidence for LV involvement. Proband status and advanced RV dysfunction were independently associated with LV abnormalities. The most common pattern of LV involvement was focal fatty infiltration in the sub-epicardium of the apicolateral LV with a “bite-like” pattern. LGE in the LV was found in the same distribution and most often had a linear appearance. LV involvement was more common with non-PKP2 genetic mutation variants, regardless of proband status. Only RV structural disease on CMR (HR 3.47, 95% CI 1.13–10.70) and prior arrhythmia (HR 2.85, 95% CI 1.33–6.10) were independently associated with arrhythmic events. Conclusion: Among patients with 2010 TFC for ARVD/C, CMR evidence for LV abnormalities are seen in half of patients and typically manifest as fibrofatty infiltration in the subepicardium of the apicolateral wall and are not associated with arrhythmic outcomes.
AB - Background: Left ventricular (LV) fibrofatty infiltration in arrhythmogenic right ventricular (RV) dysplasia/cardiomyopathy (ARVD/C) has been reported, however, detailed cardiovascular magnetic resonance (CMR) characteristics and association with outcomes are uncertain. We aim to describe LV findings on CMR in ARVD/C patients and their relationship with arrhythmic outcomes. Methods: CMR of 73 subjects with ARVD/C according to the 2010 Task Force Criteria (TFC) were analyzed for LV involvement, defined as ≥ 1 of the following features: LV wall motion abnormality, LV late gadolinium enhancement (LGE), LV fat infiltration, or LV ejection fraction (LVEF) < 50%. Ventricular volumes and function, regional wall motion abnormalities, and the presence of ventricular fat or fibrosis were recorded. Findings on CMR were correlated with arrhythmic outcomes. Results: Of the 73 subjects, 50.7% had CMR evidence for LV involvement. Proband status and advanced RV dysfunction were independently associated with LV abnormalities. The most common pattern of LV involvement was focal fatty infiltration in the sub-epicardium of the apicolateral LV with a “bite-like” pattern. LGE in the LV was found in the same distribution and most often had a linear appearance. LV involvement was more common with non-PKP2 genetic mutation variants, regardless of proband status. Only RV structural disease on CMR (HR 3.47, 95% CI 1.13–10.70) and prior arrhythmia (HR 2.85, 95% CI 1.33–6.10) were independently associated with arrhythmic events. Conclusion: Among patients with 2010 TFC for ARVD/C, CMR evidence for LV abnormalities are seen in half of patients and typically manifest as fibrofatty infiltration in the subepicardium of the apicolateral wall and are not associated with arrhythmic outcomes.
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U2 - 10.1186/s12968-020-00702-3
DO - 10.1186/s12968-020-00702-3
M3 - Article
C2 - 34011348
AN - SCOPUS:85106569808
SN - 1097-6647
VL - 23
JO - Journal of Cardiovascular Magnetic Resonance
JF - Journal of Cardiovascular Magnetic Resonance
IS - 1
M1 - 58
ER -