Value of the Echocardiographic Findings of Arrhythmogenic Right Ventricular Dysplasia with Left Ventricular Involvement in a Child

1996 ◽  
Vol 17 (1) ◽  
pp. 40-42
Author(s):  
S. Atalay ◽  
A. Imamoglu ◽  
H. Gümüs ◽  
M. Gürdal ◽  
M. Ozenci
Keyword(s):  
Right Ventricular ◽  
Arrhythmogenic Right Ventricular Dysplasia ◽  
Left Ventricular ◽  
Right Ventricular Dysplasia ◽  
Echocardiographic Findings

Left ventricular abnormalities in arrhythmogenic right ventricular dysplasia

1986 ◽  
Vol 58 (6) ◽  
pp. 568-570 ◽  
Author(s):  
John G. Webb ◽  
Charles R. Kerr ◽  
Victor F. Huckell ◽  
Henry F. Mizgala ◽  
Donald R. Ricci
Keyword(s):  
Right Ventricular ◽  
Arrhythmogenic Right Ventricular Dysplasia ◽  
Left Ventricular ◽  
Right Ventricular Dysplasia

scholarly journals Histological evidence of left ventricular involvement in arrhythmogenic right ventricular dysplasia.

1989 ◽  
Vol 53 (12) ◽  
pp. 1530-1534 ◽  
Author(s):  
MASASHI HORIMOTO ◽  
NAOKI FUNAYAMA ◽  
MOTOHIKO SATOH ◽  
TAKEKI IGARASHI ◽  
MORIE SEKIGUCHI
Keyword(s):  
Right Ventricular ◽  
Arrhythmogenic Right Ventricular Dysplasia ◽  
Left Ventricular ◽  
Histological Evidence ◽  
Right Ventricular Dysplasia

scholarly journals Prevalence of Left Ventricular Regional Dysfunction in Arrhythmogenic Right Ventricular Dysplasia

2010 ◽  
Vol 3 (3) ◽  
pp. 290-297 ◽  
Author(s):  
Aditya Jain ◽  
Monda L. Shehata ◽  
Matthias Stuber ◽  
Seth J. Berkowitz ◽  
Hugh Calkins ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Arrhythmogenic Right Ventricular Dysplasia ◽  
Left Ventricular ◽  
Right Ventricular Dysplasia ◽  
Regional Dysfunction

scholarly journals Combination of Arrhythmogenic Right Ventricular Dysplasia with Left Ventricular Non-Compaction as a Special Form of Cardiomyopathy: Clinic, Diagnostics, Genetic, Natural Course

2020 ◽  
Vol 75 (6) ◽  
pp. 594-604
Author(s):  
Yu. A. Lutokhina ◽  
O. V. Blagova ◽  
A. G. Shestak ◽  
M. Е. Polyak ◽  
A. A. Bukaeva ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Arrhythmogenic Right Ventricular Dysplasia ◽  
Left Ventricular ◽  
Pathogenic Variants ◽  
Right Ventricular Dysplasia ◽  
Therapy Effect ◽  
Appropriate Shocks ◽  
Gene Panels ◽  
Electrocardiogram Ecg

Background. A few cases of combination of arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVC) with left ventricular noncompaction (LVNC) have been described. Aims to study the genetics, diagnostical features and clinical course of the combination of ARVC with LVNC. Methods. 58 patients with ARVC diagnosis (26 men; mean age 39.1 14.2 years; mean follow-up period 21.5 [6; 60] months) and 125 patients with LVNC (74 men; mean age 46.4 15.1 years; mean follow-up period 14 [3; 40] months). All patients underwent electrocardiogram (ECG), echocardiography, 24-h ECG monitoring. Heart MRI was performed in 53 (91.4%) patients with ARVC and 60 (48%) with LVNC, heart CT in 18 (31%) patients with ARVC and 89 (71.2%) with LVNC. For all patients with combination of ARVC and LVNC DNA-diagnostic was performed using both ARVC (PKP2, DSG2, DSP, DSC2, JUP, TMEM43, TGFB3, PLN, LMNA, DES, CTTNA3, EMD, SCN5A, LDB3, CRYAB, FLNC) and LVNC (MYH7, MYBPC3, TAZ, TPM1, LDB3, MYL2, MYL3, ACTC1, TNNT2, TNI3) gene panels. Results. Combination of ARVC and LVNC was found in 9 patients (15.5% of patients form ARVC cohort and 7.2% from LVNC cohort). These patients were distinguished from patients with isolated ARVC or LVNC by aggressive ventricular arrhythmias (frequent premature ventricular beats, sustained ventricular tachycardia, significantly worse antiarrhythmic therapy effect, appropriate shocks of implanted cardioverter-defibrillators (ICD) in all patients with ICD). Patients with combination of ARVC + LVNC were also distinguished from patients with isolated LVNC by the dilatation of RV, low QRS voltage on ECG, presence of AV block, absence of signs of LV hypertrophy on ECG. LV dilatation with reduction of its ejection fraction distinguished patients with mixed cardiomyopathy from patients with isolated ARVC. Potentially pathogenic variants (IVV classes of pathogenicity) and variants of unclear clinical significance (III class of pathogenicity) were found in both desmosomal and non-desmosomal genes in 78% of patients, including 3 (33%) in DSP gene. Conclusions. The combination of ARVC and LVNC can be caused by mutations in both desmosomal and non-desmosomal genes and has typical features: aggressive, resistant ventricular rhythm abnormalities leading to appropriate ICD shocks and a high risk of sudden cardiac death.

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