@Article{chd.12481,
AUTHOR = {Shankar Baskar, Philippa Horne, Samantha Fitzsimmons, Philip R. Khoury, Joseph Vettukattill, Koichiro Niwa, Teiji Agaki, Mark Spence, Hisanori Sakazaki, Gruschen Veldtman},
TITLE = {Arrhythmia burden and related outcomes in Eisenmenger syndrome},
JOURNAL = {Structural and Congenital Heart Disease},
VOLUME = {12},
YEAR = {2017},
NUMBER = {4},
PAGES = {512--519},
URL = {http://www.techscience.com/schd/v12n4/39186},
ISSN = {3071-1738},
ABSTRACT = {<b>Background:</b> Patients with Eisenmenger syndrome have a shorter lifespan than the general population. A significant proportion develop arrhythmia and some, sudden death.<br/>
<b>Objective:</b> The aims of this study were to characterize the frequency, type and effects of arrhythmias in adult patients with Eisenmenger’s syndrome and to identify risk factors for arrhythmias.<br/>
<b>Methods:</b> This retrospective study included patients aged ≥18 years of age with Eisenmenger’s
syndrome from three institutions. Arrhythmias were noted from electrocardiograms and Holter
study reviews.<br/>
<b>Results:</b> A total of 167 patients, 96 females, 63 males (gender not available in 9 patients) were
included in this study. The mean age was 38 ± 9 years (range: 18–63 years) with a majority in
NYHA functional class II or III (57% and 32% respectively). Twenty-eight patients (17%) had significant tachyarrhythmia: paroxysmal supraventricular tachycardia (8 patients, 29%), atrial fibrillation
(6 patients, 21%), atrial fibrillation and flutter (2 patients, 7%), nonsustained ventricular tachycardia
(6 patients, 21%) and sustained ventricular tachycardia (6 patients, 21%). Among the entire study
group, 26 patients (16%) were currently on antiarrhythmic therapy and 77 patients (49%) were on
advanced therapies for pulmonary hypertension. Down syndrome was present in 78 patients
(46%). There were 21 (13%) documented deaths, of which 8 (5%) were sudden death. Patients
with arrhythmia were older [P = .01] and were more likely to have atrioventricular valvar regurgitation [Odds ratio: 4.33]. Advanced pulmonary hypertension therapy was associated with decreased
all-cause mortality in logistic regression analysis [odds ratio: 0.31], while antiarrhythmic therapy
was associated with sudden death [odds ratio: 6.24].<br/>
<b>Conclusions:</b> Arrhythmias are common among patients with Eisenmenger syndrome occurring in
around 1 in 5 individuals and are associated with all-cause mortality and sudden death.},
DOI = {10.1111/chd.12481}
}