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Background Patients with Eisenmenger syndrome are known to have a high incidence of sudden cardiac death (SCD), yet the underlying causes are not well understood. We sought to define the predictors of SCD in this population. Methods and Results A retrospective analysis of all patients with Eisenmenger syndrome from 2 large tertiary referral centers was performed. ECGs, prolonged ambulatory recordings, echocardiograms, and clinical histories were reviewed; and the cause of death was identified. A total of 246 patients (85 [34.6%] men) with a mean age of 37.3 (±14.2) years were followed up for a median of 7 years. Over the study period, 136 patients died, with 40 experiencing SCD and 74 experiencing cardiac death (sudden and nonsudden). Age, atrial fibrillation, prolonged QRS duration, complete heart block, right atrial enlargement, right bundle branch block, increased right atrial pressure, impaired biventricular function, and the presence of a pacemaker were associated with increased risk of SCD, whereas advanced pulmonary hypertension therapies were protective. Atrial fibrillation (11.45-fold increased risk; P <0.001) and QRS duration ≥120 ms (2.06-fold increased risk; P =0.034) remained significant predictors of SCD in the multivariate analysis, whereas advanced pulmonary hypertension therapies were strongly protective against SCD ( P <0.001). Conclusions Atrial arrhythmias, impaired ventricular function, and conduction system disease were associated with increased risk of SCD in this cohort of patients with Eisenmenger syndrome, providing an opportunity for early risk stratification and potential intervention. Clinical heart failure symptoms (New York Heart Association class ≥II) were predictive of increased mortality but not of SCD, suggesting a potential arrhythmic cause behind SCD.

Background: Tachyarrhythmias and bradyarrhythmias affect 20%-50% of adult patients with tetralogy of Fallot (TOF) and some of these patients will require cardiac implantable electronic devices (CIED) such as pacemaker and/or internal cardioverter defibrillator., Methods: The Mayo Adult Congenital Heart Disease database was queried for patients with repaired TOF and history of CIED implantation, 1990-2017. The study objectives were: (1) determine the occurrence of device-related complications defined as lead failure, lead recall, device infection and lead thrombus; and (2) determine the occurrence and risk factors for defibrillator shock., Results: There were 99 patients (age 46±14 years and 66 (66%) men) with CIED, and the CIEDs were 41 (41%) pacemakers and 73 (73%) defibrillators. Indication for defibrillator implantation was for primary prevention in 28 (38%) and secondary prevention in 45 (62%). Device-related complications occurred in 20 (20%) patients (lead failure 17, lead recall 4, device infection 12 and thrombus 3). Twenty-five per cent of all device infections occurred within 30 days from the time of device generator change. Annualised rates of appropriate and inappropriate shocks were 5.7% and 6.2%, respectively. The use of class III antiarrhythmic drug was protective against defibrillator shock., Conclusion: The current study provides useful outcome data to aid patient counselling and clinical decision-making. Further studies are required to explore ways to decrease the risk of postprocedural infection after device generator change, and to determine which patients will benefit from empirical antiarrhythmic therapy as a strategy to decrease incidence of inappropriate defibrillator shock., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Background Patients with tetralogy of Fallot ( TOF ) remain at risk for cardiovascular events despite successful repair. Some of the current risk stratification tools require advanced imaging and invasive studies, and hence are difficult to apply to routine patient care. A recent study showed that QRS fragmentation ( QRS -f) is predictive of mortality in patients with TOF. The current study aims to validate this result by assessing whether severity of QRS -f could predict all-cause mortality in a different TOF population. Methods and Results The authors reviewed the Mayo Adult Congenital Heart Disease database for patients with TOF who had ECG from 1990-2017. QRS -f was defined as notches in QRS complex in ≥2 contiguous leads on ECG , not related to bundle branch block, and classified as none, mild (≤3 leads), moderate (4 leads), or severe (≥5 leads). Of 465 patients (age 37±14 years) in the study, QRS -f was present in 161 (35%): mild (n=43, 9%), moderate (n=77, 17%), and severe (n=41, 9%). There were 55 deaths (12%) during 13.6±8.2 years of follow-up. Severity of QRS -f remained an independent predictor of all-cause mortality after adjustment for other ECG parameters, patient demographics, and atrial and ventricular arrhythmia (hazard ratio, 1.74 per class; 95% confidence interval, 1.08-2.93 [ P=0.041]). Conclusions The presence of severe QRS -f may be used as complementary data to the usual clinical indices to determine whether interventions such as invasive electrophysiology study should be performed in patients with nonsustained ventricular tachycardia or to proceed with pulmonary valve replacement in patients with severe pulmonary regurgitation with ventricular volumes below the guideline-directed threshold for intervention.

Background: There are limited data about the risk of thrombotic and embolic complication (TEC) in adults with atrial arrhythmia after Fontan operation., Objectives: This study sought to determine the risk of TEC in this population and the role of anticoagulation therapy in TEC prevention., Methods: This was a retrospective review of adults with atrial arrhythmia after Fontan operation who were evaluated at the Mayo Clinic between 1994 to 2014. TEC was classified into 2 groups: systemic TEC, defined as intracardiac thrombus, ischemic stroke, or systemic arterial embolus; and nonsystemic TEC, defined as Fontan conduit/right atrial thrombus or pulmonary embolus. Patients were divided into 3 groups: anticoagulation, antiplatelet, and no therapy cohorts., Results: We followed 278 patients, mean age 31 ± 9 years, for 88 ± 14 months (1,464 patient-years). Patient groups included antiplatelet (n = 181), anticoagulation (n = 91), and no therapy (n = 6). There were 97 TEC in 81 patients (29%); 32 were systemic, yielding an event rate of 2.1 systemic TEC per 100 patient-years, and 65 were nonsystemic TEC, yielding an event rate of 4.4 nonsystemic TEC per 100 patient-years. Prevalence of TEC was 18% and 55% at 5 and 10 years, respectively. Atriopulmonary connection was a risk factor for TEC (hazard ratio: 2.31; 95% confidence interval: 1.61 to 4.64), and TEC were associated with higher risk of death and hospitalization (p < 0.0001). Anticoagulation was protective against TEC and resulted in a reduction of TEC risk by 2.5 TEC per 100 patient-years. Anticoagulation was also associated with lower risk of death and hospitalization (p = 0.02). Bleeding complications occurred in 21 (7%) patients and were similar in all groups., Conclusions: Anticoagulation was associated with lower TEC rate and lower risk of death and hospitalization, without a significant increase in bleeding risk. Perhaps anticoagulation should be the preferred preventive strategy., (Copyright © 2016 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Background: Cardiac implantable electronic devices (CIEDs) are commonly associated with transvenous lead-related thrombi that can cause pulmonary embolism (PE)., Methods and Results: We retrospectively evaluated all patients with transvenous CIED leads implanted at Mayo Clinic Rochester between 1 January 2000, and 25 October 2010. Pulmonary embolism outcomes during follow-up were screened using diagnosis codes and confirmed with imaging study reports. Of 5646 CIED patients (age 67.3 ± 16.3 years, 64% men, mean follow-up 4.69 years) 88 developed PE (1.6%), incidence 3.32 [95% confidence interval (CI) 2.68-4.07] per 1000 person-years [men: 3.04 (95% CI 2.29-3.96) per 1000 person-years; women: 3.81 (95% CI 2.72-5.20) per 1000 person-years]. Other than transvenous CIED lead(s), 84% had another established risk factor for PE such as deep vein thrombosis (28%), recent surgery (27%), malignancy (25%), or prior history of venous thromboembolism (15%). At the time of PE, 22% had been hospitalized for ≥ 48 h, and 59% had been hospitalized in the preceding 30 days. Pulmonary embolism occurred in 22% despite being on systemic anticoagulation therapy. Out of 88 patients with PE, 45 subsequently died, mortality rate 93 (95% CI 67-123) per 1000 person-years (hazard ratio 2.0, 95% CI 1.5-2.7, P < 0.0001)., Conclusions: Though lead-related thrombus is commonly seen in patients with transvenous CIED leads, clinical PE occurs with a low incidence. It is possible that embolism of lead thrombus is uncommon or emboli are too small to cause consequential pulmonary infarction., (Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2015. For permissions please email: journals.permissions@oup.com.)

Objectives: We sought to evaluate safety, efficacy, and outcome of direct current cardioversion (DCCV) for atrial arrhythmias in adults with congenital heart disease (CHD)., Background: Atrial arrhythmias are increasingly noted in adults with CHD. The outcome of DCCV for atrial arrhythmias in this population is unknown., Methods: Our study was a retrospective review of patients 18 years or older with CHD who underwent DCCV between June 2000 and July 2003. This constituted the CHD group. Patient characteristics reviewed included the specific cardiac diagnosis and arrhythmia history. A subset of patients had transesophageal echocardiography (TEE) before DCCV; this subset was reviewed to evaluate spontaneous echocardiographic contrast. The outcome data evaluated included success of DCCV, complications, recurrence of arrhythmia, antiarrhythmic medication use, electrophysiology or pacemaker procedure in follow-up, and all-cause mortality. The recurrence rate of the arrhythmia was compared to a control group consisting of an age, gender, and rhythm matched group of patients who have no CHD and who underwent DCCV for atrial arrhythmias., Results: Sixty-three patients in the CHD group underwent 80 DCCVs, 59 of which were TEE-guided. Atrial flutter was more common in the CHD group (37 of 80 DCCV, 46%) than in the control group (13 of 56, 23%) (p<0.001). DCCV was successful in 75 (94%). Mean follow-up was 387 days. No thromboembolic events were noted. All-cause mortality on follow-up was 11%. There was no death related to DCCV. Twenty-five patients in the CHD group (40%) remained in sinus rhythm throughout follow-up. This was similar to that observed in the control group (30/56, 54%, p=0.13). Recurrent arrhythmia in the CHD group was predicted by the presence of atrial fibrillation (p=0.009) and less so spontaneous echo contrast in the left atrium (p=0.05)., Conclusions: DCCV with appropriate anticoagulation is safe and effective for patients with CHD, even in the presence of an intracardiac shunt and spontaneous contrast on TEE. However, the recurrence rate is substantial. Spontaneous echo contrast in the left atrium along with atrial fibrillation predicts arrhythmia recurrence following DCCV in patients with CHD., (Copyright © 2010 Elsevier Ireland Ltd. All rights reserved.)

Background: Permanent pacing (PM) in patients with congenital heart disease (CHD) presents unique challenges-with little known about the long-term outcomes., Methods: Pacemaker complications and reinterventions were reviewed over a 38-year period and were grouped by epicardial or endocardial approaches., Results: The average age at intervention was 37 ± 19 years for 106 patients and 259 PM procedures were performed (2.4 ± 2 per patient). From the first PM procedure, patients were followed for 11.6 ± 14 years. The most common indications for initial PM intervention were heart block (25%) and sinus node dysfunction (20%), yet reintervention was driven primarily by lead failure (49%). Endocardial systems were initially implanted in 73 patients (67%). Epicardial pacing was more common in patients with complex CHD (p = 0.006), cyanosis (p < 0.001), residual shunts (0.01), or Ebstein's anomaly (p = 0.01). Fifty-one devices (28%) developed lead or generator complications. Epicardial systems were most likely to develop lead failure (p < 0.0001), predominantly in the ventricular lead (p < 0.0001). Endocardial systems were found to be more durable than the epicardial systems (p = 0.023), and Ebstein's anomaly or an epicardial system was an independent predictor of lead failure., Conclusions: Permanent pacing in CHD is associated with considerable morbidity and the need for repeat intervention, especially in those with Ebstein's anomaly. Epicardial pacing systems appear to have a higher incidence of lead failure and are significantly less durable in this group.

The number of children with congenital heart disease surviving into adulthood has increased dramatically. Refinement in surgical techniques and effective subsequent care has mainly been responsible for this change. However, many of these survivors have significant lifestyle altering symptoms associated with rhythm disorders. These may result from the primary congenital anomaly or from surgical correction. Tachyarrhythmias often result from re-entrant circuits around surgical scars in the setting of a markedly enlarged cardiac chamber. Symptomatic bradyarrhythmias may result from surgical interruption of the conduction system or from the use of antiarrhythmic medication. The management of these lifestyle altering rhythm disorders frequently involves cardiac devices, including pacemakers, antitachycardia devices, defibrillators and resynchronization devices. We review the underlying causes for common rhythm disorders and discuss the specific challenges with cardiac device use in the adult congenital heart disease population. In addition, we address pacing for improvement of ventricular function and innovations likely to impact the field.