Open Access

ARTICLE

Arrhythmia burden and related outcomes in Eisenmenger syndrome

Shankar Baskar¹, Philippa Horne², Samantha Fitzsimmons³, Philip R. Khoury¹, Joseph Vettukattill⁴, Koichiro Niwa⁵, Teiji Agaki⁶, Mark Spence⁷, Hisanori Sakazaki⁸, Gruschen Veldtman¹

1 Cincinnati Children’s Hospital Medical Center, Cincinnati, Ohio
2 The Royal London Hospital, London, United Kingdom
3 Southampton University Hospital, Southampton University School of Medicine, Southampton, United Kingdom
4 Helen DeVos Children’s Hospital, Grand Rapids, Michigan, USA
5 St Luke’s International Hospital, Tokyo, Japan
6 Okayama University Hospital, Okayama, Japan
7 Royal Victoria Hospital, Belfast, Northern Ireland
8 Hyogo Prefectural Amagasaki General Medical Center, Hyogo, Japan

* Corresponding Author: Gruschen R. Veldtman, Adolescent and Adult Congenital Program, Heart Institute, Cincinnati Children’s Hospital Medical Centre, 3333 Burnet Avenue, Cincinnati 45229, Ohio, USA. Email:

Congenital Heart Disease 2017, 12(4), 512-519. https://doi.org/10.1111/chd.12481

Abstract

Background: Patients with Eisenmenger syndrome have a shorter lifespan than the general population. A significant proportion develop arrhythmia and some, sudden death.
Objective: 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.
Methods: 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.
Results: 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].
Conclusions: 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.

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