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Late onset arrhythmogenic cardiomyopathy: clinical features and prognosis

Session Poster session 3

Speaker Doctor Alexandros Protonotarios

Event : ESC Congress 2016

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

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

A. Protonotarios1 , A. Anastasakis2 , L. Antoniades3 , P. Sotiropoulou2 , D. Tousoulis2 , A. Tsatsopoulou1 , 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 ,


European Heart Journal ( 2016 ) 37 ( Abstract Supplement ), 572

Background: Arrhythmogenic cardiomyopathy (ACM) as a major cause of sudden cardiac death in the young and athletes has been repeatedly investigated. Little is known about the patient sub-population, which presents with ACM at a later age.

Purpose: We aimed to investigate the clinical features and characteristics of ACM in patients who presented at 50 years of age or older and compare them with younger ones.

Methods: Eighty-six unselected patients fulfilling the 2010 Task Force diagnostic criteria were consecutively enrolled from a multicentre ACM cohort. All subjects were serially evaluated with standard 12-lead ECG, two-dimensional echocardiography and 24-hour Holter monitoring. Patients were grouped according to their age into those of less than 50 years and those at 50 years of age or more. Categorical variables were compared using the chi-square or Fischer's exact test as appropriate and continuous variables were compared using the Mann-Whitney U-test. All statistical tests were performed at the 5% level of significance.

Results: Eighteen patients (21%) presented at the age of 50 years or more. Ten of them were probands. Clinical comparisons with the rest of study population are presented in Table.

Conclusions: Right ventricular wall motion abnormalities, particularly aneurysms, are more frequent in late onset ACM; although these patients have similar risk for sustained ventricular tachycardia episodes with the younger group, sudden cardiac death is unlikely.

Clinical features in late onset disease
VariablesAge <50 yearsAge ≥50 yearsp-value
Male gender43 (63)8 (44)0.15
Terminal activation duration ≥55 ms32/58 (55)7/14 (50)0.73
Epsilon waves19 (28)5 (28)0.99
Right bundle branch block10 (15)4 (22)0.44
T-wave inversions ≥V435 (51)4 (22)0.045
Right ventricular outflow tract diameter (PLAX) (mm)35±1037±70.12
Right ventricular inflow tract diameter (mm)41±1044±100.35
Right ventricular wall motion abnormalities52 (76)17 (94)0.09
Right ventricular aneurysms30 (44)14 (78)0.01
Sustained ventricular tachycardia27 (40)7 (39)0.95
Sudden cardiac death15 (22)0 (0)0.03
Heart failure13 (19)3 (17)0.81
Values for categorical variables reported as n (%) and for continuous variables mean ± SD. PLAX = Parasternal long axis.

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