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The spectrum of depolarization abnormalities in the natural history of arrhythmogenic cardiomyopathy

Session Improving diagnosis in cardiomyopathies

Speaker Doctor Alexandros Protonotarios

Event : ESC Congress 2016

  • Topic : valvular, myocardial, pericardial, pulmonary, congenital heart disease
  • Sub-topic : Clinical
  • Session type : Moderated Posters

Authors : A Protonotarios (Naxos,GR), A Anastasakis (Athens,GR), L Antoniades (Nicosia,CY), P Sotiropoulou (Athens,GR), D Tousoulis (Athens,GR), A Tsastsopoulou (Naxos,GR)

Authors:
A. Protonotarios1 , A. Anastasakis2 , L. Antoniades3 , P. Sotiropoulou2 , D. Tousoulis2 , A. Tsastsopoulou1 , 1Nikos Protonotarios Medical Centre, Department of Cardiology - Naxos - Greece , 2University of Athens Medical School, 1st Department of Cardiology - Athens - Greece , 3Nicosia General Hospital, Department of Cardiology - Nicosia - Cyprus ,

Topic(s):
Cardiomyopathies

Citation:
European Heart Journal ( 2016 ) 37 ( Abstract Supplement ), 225

Background: Arrhythmogenic cardiomyopathy (ACM) is characterized by specific depolarization abnormalities, consisting mainly of abnormal terminal activation duration (TAD), epsilon waves and right bundle branch block (RBBB).

Purpose: This study aimed to classify depolarization abnormalities into stages that exhibit a successive association in time and investigate their correlations with structural as well as arrhythmic disease profile.

Methods: Eighty-six unselected patients fulfilling the 2010 Task Force diagnostic criteria were enrolled from a multicentre ACM cohort. All subjects were serially evaluated with standard 12-lead ECG and two-dimensional echocardiography. Patients were grouped according to depolarization abnormalities into those with (I) none, (II) abnormal TAD solely, (III) epsilon waves and (IV) RBBB. Comparisons were made with categorical variables using both the chi-square for association and for trend test and continuous variables using both the Kruskal-Wallis test and the Spearman's correlation coefficient in order to identify overall differences as well as correlations between variables. All statistical tests were performed at the 5% level of significance.

Results: Comparisons of clinical characteristics between groups are reported in Table. In patients with long follow-up, evolution of depolarization abnormalities was noted (I->II, n=2; II->III, n=6; II->IV, n=1; III->IV, n=2) but no retrograde transitions.

Conclusions: Depolarization abnormalities appear to constitute manifestations secondary to structural alterations of the right ventricle. A classification of depolarization abnormalities has been proposed that corresponds to disease evolution and has a close relation with right ventricular structural abnormalities and risk for sustained ventricular arrhythmias.

Table 1. Clinical features according to stage
Parameters(I) None (n=31)(II) TAD (n=23)(III) Epsilon wave (n=18)(IV) RBBB (n=14)Overall p-valueCorrelation p-value
Right ventricular outflow tract diameter (PLAX) (mm)30±532±641±1343±7<0.0001<0.0001
Right ventricular inflow tract diameter (mm)35±540±746±1052±12<0.0001<0.0001
Right ventricular wall motion abnormalities20 (65)18 (78)17 (94)14 (100)`0.0140.001
Right ventricular aneurysm13 (42)11 (48)9 (50)11 (79)0.150.042
Left ventricular wall motion abnormalities11 (35)7 (30)4 (22)5 (36)0.780.72
Sustained ventricular tachycardia10 (32)4 (17)11 (61)9 (62)0.0060.009
Sudden cardiac death2 (6)7 (30)4 (22)2 (14)0.130.38
Heart failure5 (163 (13)3 (17)5 (36)0.340.19
Values for categorical variables reported as n (%) and for continuous variables mean ± SD. PLAX = Parasternal long axis.

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