Sudden death in patients with Ebstein anomaly

Jost, Christine H. Attenhofer; Tan, Nicholas; Hassan, Abdalla; Vargas, Emily; Hodge, David O.; Dearani, Joseph A.; Connolly, Heidi M.; Asirvatham, Samuel J.; McLeod, Christopher J.

doi:10.1093/eurheartj/ehx794

Cited by 50 publications

(23 citation statements)

References 25 publications

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“…In addition, ≈30% received ICDs for sudden cardiac death prevention. This is overall consistent with prior studies highlighting the propensity for both bradyarrhythmias 3 and tachyarrhythmias 1 in patients with EA.…”

Section: Discussionsupporting

confidence: 92%

Cardiac Implantable Electronic Devices in Ebstein Anomaly: Management and Outcomes

Tan

Amin

Dearani

et al. 2022

Circ: Arrhythmia and Electrophysiology

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Background: Optimal management of cardiac implantable electronic devices (CIEDs) in patients with Ebstein anomaly during tricuspid valve (TV) surgery is unknown. Thus, we aimed to characterize CIED management/outcomes in patients with Ebstein anomaly undergoing TV surgery. Methods: Patients at the Mayo Clinic from 1987 to 2020 with Ebstein anomaly and CIED procedure were reviewed for procedural details, complications, echocardiogram, and lead parameters. Five-year cumulative incidence of CIED complications were estimated using the Kaplan-Meier method. Results: Ninety-three patients were included; 51 were female, and mean age was 40.7±17.5 years. A new CIED was implanted in 45 patients at the time of TV surgery with the majority receiving an epicardial (n=37) CIED. Among 34 patients who had preexisting CIED (11 epicardial, 23 transvenous) at time of TV surgery, 20 had a transvenous right ventricular lead managed by externalizing the lead to the TV (n=15) or extracting the transvenous lead with epicardial lead implantation (n=5). Fourteen patients underwent CIED implantation (4 epicardial, 10 transvenous) without concurrent surgery. Placement of lead across the TV was avoided in 85% of patients. The 5-year cumulative incidence of CIED complications was 24% with no significant difference between epicardial and transvenous CIEDs (26% versus 23%, P =0.96). Performance of lead parameters was similar in epicardial and transvenous leads during median (interquartile range) follow-up of 44.5 (61.1) months. Conclusions: In patients with Ebstein anomaly undergoing TV surgery, the use of epicardial leads and externalization of transvenous leads to the TV can avoid lead placement across the valve leaflets. Lead performance and CIED complications was similar between epicardial and transvenous CIEDs.

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Section: Discussionsupporting

confidence: 92%

Cardiac Implantable Electronic Devices in Ebstein Anomaly: Management and Outcomes

Tan

Amin

Dearani

et al. 2022

Circ: Arrhythmia and Electrophysiology

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“…All-cause death occurred more frequently in patients with AF, but it did not remain an independent predictor after adjusting for other covariates. Although some studies have reported an association between AF and mortality in adults with CHD,14–16 other studies focused exclusively on patients with EA did not observe this association similar to our study 7 23 28 29. This might suggest that risk of death in EA derives from a complex interaction of several factors, among which AF might play a less significant role.…”

Section: Discussioncontrasting

confidence: 51%

Prognostic implications of atrial fibrillation in adults with Ebstein anomaly

Miguel

Madhavan

Miranda

et al. 2022

Heart

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ObjectiveSupraventricular arrhythmias are common in adults with Ebstein anomaly (EA). However, there are limited data about prognostic implications of atrial fibrillation (AF) in this population. Accordingly, our aim was to assess the clinical profile and burden of AF in adults with EA, and the relationship between AF and outcomes.Methods and resultsSix hundred eighty-two consecutive adults with a median age of 36 (24–49) years from Mayo Clinic, Minnesota, USA, between 2003 and 2020 were included. Sustained episodes of AF, clinical, echocardiographic, rhythm and surgical data were collected. Prevalence of AF at baseline was 18% (126 patients); the first episode occurred at a mean age of 43±17 years. Patients with AF were older, were more likely men, and had hypertension, renal dysfunction, cardiac devices, and more advanced right-sided and left-sided remodelling. During a median follow-up of 156 (81–240) months, 62 patients (11%) developed incident AF. At the last encounter, prevalence of AF was 28% (188 patients); of those, 63 (34%) had recurrent AF. Hospitalisation for heart failure (HF) occurred in 51 patients (7%). AF (HR 2.32, 95% CI 1.18 to 4.47; p=0.01) was independently associated with hospitalisation for HF. All-cause death occurred in 53 patients (8%); it was more frequent in those with AF in the univariable analysis, although it did not remain significant in the multivariable analysis.ConclusionsAF in EA develops at relatively young ages with one-third of the cohort exhibiting a recurrent pattern. Patients with AF had a higher prevalence of comorbidities and worse right-sided and left-sided cardiac remodelling. AF was independently associated with HF hospitalisation.

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“…No significant differences were found between the different groups of CHD, but strong trends suggest a prominent risk for severe arrhythmia in patients with Ebstein’s anomaly, while the risk in patients with a common arterial trunk appeared to be negligible. Recent studies suggest that EBS patients are at significant risk for arrhythmias and sudden cardiac death [ 27 , 28 , 29 ]. It has been stated that these lethal arrhythmias in Ebstein’s anomaly are caused by the anatomic proximity of the structural abnormalities to the conduction system, namely the tricuspid annulus, the central fibrous body, the atrioventricular (AV) node, the right-sided myocardium, and the papillary muscles [ 30 ].…”

Section: Discussionmentioning

confidence: 99%

Peak Oxygen Uptake on Cardiopulmonary Exercise Test Is a Predictor for Severe Arrhythmic Events during Three-Year Follow-Up in Patients with Complex Congenital Heart Disease

Sanden

Ptushkina

Hock

et al. 2022

JCDD

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Patients with congenital heart disease (CHD) are at increased risk for severe arrhythmia and sudden cardiac death (SCD). Although implantable cardioverter defibrillators (ICD) effectively prevent SCD, risk stratification for primary prophylaxis in patients with CHD remains challenging. Patients with complex CHD undergoing CPET were included in this single-center study. Univariable and backwards stepwise multivariable logistic regression models were used to identify variables associated with the endpoint of severe arrhythmic event during three years of follow-up. Cut-off values were established with receiver operating characteristic (ROC) curve analysis. Survival analysis was conducted via Kaplan–Meier plots. Severe Arrhythmia was documented in 97 of 1194 patients (8.1%/3 years). Independent risk factors for severe arrhythmia during follow-up were old age and a low peak oxygen uptake (V.O2peak) on multivariable analysis. Patients with more advanced age and with V.O2peak values of less than 24.9 mL/min/kg were at significantly increased risk for the occurrence of severe arrhythmias during follow-up. The combined analysis of both risk factors yielded an additional benefit for risk assessment. Age at CPET and V.O2peak predict the risk for severe arrhythmic events and should be considered for risk stratification of SCD in patients with complex CHD.

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Sudden death in patients with Ebstein anomaly

Abstract: Patients with EA are at significant risk for SD. Key clinical SD predictors identified can aid in risk stratification and potentially guide primary prevention ICD implantation.

Cited by 50 publications

References 25 publications

Cardiac Implantable Electronic Devices in Ebstein Anomaly: Management and Outcomes

Cardiac Implantable Electronic Devices in Ebstein Anomaly: Management and Outcomes

Prognostic implications of atrial fibrillation in adults with Ebstein anomaly

Peak Oxygen Uptake on Cardiopulmonary Exercise Test Is a Predictor for Severe Arrhythmic Events during Three-Year Follow-Up in Patients with Complex Congenital Heart Disease

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