PMID- 32114891
OWN - NLM
STAT- MEDLINE
DCOM- 20210317
LR  - 20210317
IS  - 2047-9980 (Electronic)
IS  - 2047-9980 (Linking)
VI  - 9
IP  - 5
DP  - 2020 Mar 3
TI  - Arrhythmogenic Right Ventricular Cardiomyopathy: Characterization of Left 
      Ventricular Phenotype and Differential Diagnosis With Dilated Cardiomyopathy.
PG  - e014628
LID - 10.1161/JAHA.119.014628 [doi]
LID - e014628
AB  - Background This study assessed the prevalence of left ventricular (LV) 
      involvement and characterized the clinical, electrocardiographic, and imaging 
      features of LV phenotype in patients with arrhythmogenic right ventricular 
      cardiomyopathy (ARVC). Differential diagnosis between ARVC-LV phenotype and 
      dilated cardiomyopathy (DCM) was evaluated. Methods and Results The study 
      population included 87 ARVC patients (median age 34 years) and 153 DCM patients 
      (median age 51 years). All underwent cardiac magnetic resonance with quantitative 
      tissue characterization. Fifty-eight ARVC patients (67%) had LV involvement, with 
      both LV systolic dysfunction and LV late gadolinium enhancement (LGE) in 41/58 
      (71%) and LV-LGE in isolation in 17 (29%). Compared with DCM, the ARVC-LV 
      phenotype was statistically significantly more often characterized by low QRS 
      voltages in limb leads, T-wave inversion in the inferolateral leads and major 
      ventricular arrhythmias. LV-LGE was found in all ARVC patients with LV systolic 
      dysfunction and in 69/153 (45%) of DCM patients. Patients with ARVC and LV 
      systolic dysfunction had a greater amount of LV-LGE (25% versus 13% of LV mass; 
      P<0.01), mostly localized in the subepicardial LV wall layers. An LV-LGE >/=20% had 
      a 100% specificity for diagnosis of ARVC-LV phenotype. An inverse correlation 
      between LV ejection fraction and LV-LGE extent was found in the ARVC-LV phenotype 
      (r=-0.63; P<0.01), but not in DCM (r=-0.01; P=0.94). Conclusions LV involvement 
      in ARVC is common and characterized by clinical and cardiac magnetic resonance 
      features which differ from those seen in DCM. The most distinctive feature of 
      ARVC-LV phenotype is the large amount of LV-LGE/fibrosis, which impacts directly 
      and negatively on the LV systolic function.
FAU - Cipriani, Alberto
AU  - Cipriani A
AD  - Department of Cardio-Thoraco-Vascular Sciences and Public Health University of 
      Padua Italy.
FAU - Bauce, Barbara
AU  - Bauce B
AD  - Department of Cardio-Thoraco-Vascular Sciences and Public Health University of 
      Padua Italy.
FAU - De Lazzari, Manuel
AU  - De Lazzari M
AD  - Department of Cardio-Thoraco-Vascular Sciences and Public Health University of 
      Padua Italy.
FAU - Rigato, Ilaria
AU  - Rigato I
AD  - Department of Cardio-Thoraco-Vascular Sciences and Public Health University of 
      Padua Italy.
FAU - Bariani, Riccardo
AU  - Bariani R
AD  - Department of Cardio-Thoraco-Vascular Sciences and Public Health University of 
      Padua Italy.
FAU - Meneghin, Samuele
AU  - Meneghin S
AD  - Department of Cardio-Thoraco-Vascular Sciences and Public Health University of 
      Padua Italy.
FAU - Pilichou, Kalliopi
AU  - Pilichou K
AD  - Department of Cardio-Thoraco-Vascular Sciences and Public Health University of 
      Padua Italy.
FAU - Motta, Raffaella
AU  - Motta R
AD  - Department of Medicine Institute of Radiology University of Padua Italy.
FAU - Aliberti, Camillo
AU  - Aliberti C
AD  - Department of Medicine Institute of Radiology University of Padua Italy.
FAU - Thiene, Gaetano
AU  - Thiene G
AD  - Department of Cardio-Thoraco-Vascular Sciences and Public Health University of 
      Padua Italy.
FAU - McKenna, William J
AU  - McKenna WJ
AD  - Institute of Cardiovascular Science University College London London United 
      Kingdom.
FAU - Zorzi, Alessandro
AU  - Zorzi A
AD  - Department of Cardio-Thoraco-Vascular Sciences and Public Health University of 
      Padua Italy.
FAU - Iliceto, Sabino
AU  - Iliceto S
AD  - Department of Cardio-Thoraco-Vascular Sciences and Public Health University of 
      Padua Italy.
FAU - Basso, Cristina
AU  - Basso C
AD  - Department of Cardio-Thoraco-Vascular Sciences and Public Health University of 
      Padua Italy.
FAU - Perazzolo Marra, Martina
AU  - Perazzolo Marra M
AD  - Department of Cardio-Thoraco-Vascular Sciences and Public Health University of 
      Padua Italy.
FAU - Corrado, Domenico
AU  - Corrado D
AD  - Department of Cardio-Thoraco-Vascular Sciences and Public Health University of 
      Padua Italy.
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
DEP - 20200302
PL  - England
TA  - J Am Heart Assoc
JT  - Journal of the American Heart Association
JID - 101580524
RN  - 0 (Contrast Media)
RN  - K2I13DR72L (Gadolinium DTPA)
SB  - IM
MH  - Adult
MH  - Arrhythmogenic Right Ventricular Dysplasia/*diagnosis
MH  - Cardiomyopathy, Dilated/*diagnosis
MH  - Cohort Studies
MH  - Contrast Media
MH  - Diagnosis, Differential
MH  - Electrocardiography
MH  - Female
MH  - Gadolinium DTPA
MH  - Humans
MH  - Magnetic Resonance Imaging
MH  - Male
MH  - Middle Aged
MH  - Sensitivity and Specificity
MH  - Ventricular Dysfunction, Left/*diagnosis
MH  - Young Adult
PMC - PMC7335583
OTO - NOTNLM
OT  - arrhythmogenic right ventricular cardiomyopathy
OT  - cardiac magnetic resonance
OT  - dilated cardiomyopathy
OT  - late gadolinium enhancement
OT  - left ventricular involvement
OT  - left ventricular phenotype
EDAT- 2020/03/03 06:00
MHDA- 2021/03/18 06:00
PMCR- 2020/03/03
CRDT- 2020/03/03 06:00
PHST- 2020/03/03 06:00 [entrez]
PHST- 2020/03/03 06:00 [pubmed]
PHST- 2021/03/18 06:00 [medline]
PHST- 2020/03/03 00:00 [pmc-release]
AID - JAH34883 [pii]
AID - 10.1161/JAHA.119.014628 [doi]
PST - ppublish
SO  - J Am Heart Assoc. 2020 Mar 3;9(5):e014628. doi: 10.1161/JAHA.119.014628. Epub 
      2020 Mar 2.