Tarek Zghaib1, Anneline S J M Te Riele2, Cynthia A James1, Neda Rastegar3, Brittney Murray1, Crystal Tichnell1, Marc K Halushka4, David A Bluemke3,5, Harikrishna Tandri1, Hugh Calkins1, Ihab R Kamel3, Stefan Loy Zimmerman6. 1. Division of Cardiology, Johns Hopkins University School of Medicine, Baltimore, MD, USA. 2. Department of Cardiology, University Medical Center Utrecht, Utrecht, The Netherlands. 3. The Russell H. Morgan Department of Radiology and Radiological Sciences, Johns Hopkins University School of Medicine, 600 N. Wolfe St.; Halsted B180, Baltimore, MD, USA. 4. Department of Pathology, Johns Hopkins University School of Medicine, Baltimore, MD, USA. 5. Department of Radiology, University of Wisconsin School of Medicine and Public Health, Madison, WI, USA. 6. The Russell H. Morgan Department of Radiology and Radiological Sciences, Johns Hopkins University School of Medicine, 600 N. Wolfe St.; Halsted B180, Baltimore, MD, USA. stefan.zimmerman@jhmi.edu.
Abstract
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.
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.
Authors: Alberto Cipriani; Giulia Mattesi; Riccardo Bariani; Annagrazia Cecere; Nicolò Martini; Laura De Michieli; Stefano Da Pozzo; Simone Corradin; Giorgio De Conti; Alessandro Zorzi; Raffaella Motta; Manuel De Lazzari; Barbara Bauce; Sabino Iliceto; Cristina Basso; Domenico Corrado; Martina Perazzolo Marra Journal: Eur Radiol Date: 2022-07-05 Impact factor: 5.315