Cardiac arrest and ventricular arrhythmia in adults with Ebstein anomaly and left ventricular non-compaction.
BACKGROUND
Ebstein anomaly is a complex, congenital heart defect that is associated with a variety of cardiac abnormalities. Studies found a similar sarcomere gene mutation in patients with Ebstein anomaly (EA) and patients with isolated left ventricular non-compaction (LVNC).


AIM
We aimed to show the prevalence of LVNC and its potential relationship with severe cardiac events (VT - ventricular tachycardia, cardiac arrest) in adult patients with EA.


METHODS
We conducted a retrospective search of our institutional database from 2010 to 2014 for patients with EA and reviewed patients' medical records (age, sex, clinical presentation, electrocardiographic, echocardiographic, and CMR - cardiac magnetic resonance features). We reviewed echocardiograms and CMR scans for concomitant morphological abnormalities (LVNC, PDA - patent ductus arteriosus, VSD - ventricular septal defect, ASD - atrial septal defect, mitral valve prolapse, BAV - bicuspid aortic valve, CoA - coarctation of aorta).


RESULTS
The studied group consisted of 84 consecutive patients (mean age 38±15 years, 50 women) with EA. We found four patients (4.8%) with LVNC, two of them had cardiac arrest, one had VT, and one was symptomless, but had QTc prolongation in Holter recordings. Concomitant abnormalities were VSD (4.8%), PDA (1.2%), CoA (1.2%), mitral valve prolapse (1.2%), and BAV (2.4%). The most common anomaly was ASD type II - 23 patients (27.3%) and WPW - Wolff-Parkinson-White's syndrome - 9 patients (10.7%).


CONCLUSIONS
Non-compaction is a notable abnormality in adult patients with EA and it may affect their prognosis. Although other concomitant lesions were more common, only patients with LVNC suffered from cardiac arrest or ventricular arrhythmia.
['Kumor|Magdalena|M|', 'Lipczyńska|Magdalena|M|', 'Biernacka|Elzbieta Katarzyna|EK|', 'Klisiewicz|Anna|A|', 'Wójcik|Anna|A|', 'Konka|Marek|M|', 'Kożuch|Katarzyna|K|', 'Szymański|Piotr|P|', 'Hoffman|Piotr|P|']
D000293:Adolescent D000328:Adult D000368:Aged D001021:Aortic Valve D001145:Arrhythmias, Cardiac D000082882:Bicuspid Aortic Valve Disease D004437:Ebstein Anomaly D004452:Echocardiography D004562:Electrocardiography D005260:Female D006323:Heart Arrest D006330:Heart Defects, Congenital D006344:Heart Septal Defects, Atrial D006345:Heart Septal Defects, Ventricular D006349:Heart Valve Diseases D006352:Heart Ventricles D006801:Humans D008297:Male D008875:Middle Aged D008945:Mitral Valve Prolapse D009154:Mutation D015995:Prevalence D011379:Prognosis D012189:Retrospective Studies D014927:Wolff-Parkinson-White Syndrome D055815:Young Adult
Arrhythmia Ebstein anomaly Non-compaction Adult congenital heart diseases