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Background: Atrial arrhythmia is a late complication after tetralogy of Fallot (TOF) repair, but arrhythmia outcomes data are limited., Objectives: The purpose of the study was to describe atrial arrhythmia presentations, outcomes of antiarrhythmic therapy, and impact of arrhythmia on transplant-free survival., Methods: We reviewed the MACHD (Mayo Adult Congenital Heart Disease) Registry and identified 113 patients (age 49 ± 13 years) with documented arrhythmia, and 302 patients without history of arrhythmia, 1990-2017. We classified arrhythmias into atrial fibrillation and atrial flutter/tachycardia based on the rhythm on the first abnormal electrocardiogram., Results: At the time of first documented arrhythmia, 58(51%) had atrial fibrillation while 55(49%) had atrial flutter/tachycardia. Of the 113 patients, 14(12%) received rhythm control with class I/III antiarrhythmic drugs (AAD), 79(70%) had direct current cardioversion, 9(8%) received rate control with class II/IV AAD, and 11(10%) received only anticoagulation. Successful cardioversion occurred in 100(89%) patients, and arrhythmia recurrence rate was 16 per 100 patient-years. The multivariate risk factors for death and/or heart transplant were atrial fibrillation (HR 1.94, CI 1.10-3.15, P = .031) and older age (HR 1.63, CI 1.12-2.43, P = .019) per 5 year increment., Conclusions: Atrial fibrillation, but not atrial flutter, was associated with reduced survival in our repaired TOF cohort. Further studies are required to determine if more aggressive antiarrhythmic therapy will improve survival in patients with atrial fibrillation., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: The objective was to better understand Doppler hemodynamics and exercise capacity in patients with Fontan palliation by delineating the hemodynamic mechanism for temporal changes in their peak oxygen consumption (V̇o 2 )., Methods: We performed a retrospective review of adult Fontan patients with systemic left ventricle (LV) who underwent serial transthoracic echocardiograms (TTE) and cardiopulmonary exercise tests (CPET) at Mayo Clinic in 2000-2015. TTE and CPET data were used (1) to determine agreement between V̇o 2 and Doppler-derived LV function indices (eg, stroke volume index [SVI] and cardiac index [CI]) and (2) to determine agreement between temporal changes in peak V̇o 2 and LV function indices., Results: Seventy-five patients (44 men; 59%) underwent 191 pairs of TTE and CPET. At baseline, mean age was 24±3 years, peak V̇o 2 was 22.9±4.1 mL/kg/min (63±11 percent predicted), SVI was 43±15 mL/m 2 , and CI was 2.9±0.9 L/min/m 2 . Peak V̇o 2 correlated with SVI (r=0.30, P<.001) and with CI (r=0.45, P<.001) in the 153 pairs of TTE and CPET in patients without cirrhosis. Temporal changes in percent predicted peak V̇o 2 correlated with changes in SVI (r=0.48, P=.005) and CI (r=0.49, P=.004) among the 33 patients without interventions during the study. In the 19 patients with Fontan conversion, percent predicted peak V̇o 2 and chronotropic index improved., Conclusions: Overall, there was a temporal decline in peak V̇o 2 that correlated with decline in Doppler SVI. In the patients who had Fontan conversion operation, there was a temporal improvement in peak V̇o 2 that correlated with improvement in chronotropic index., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Background: Cardiac pacing can be challenging after a Fontan operation, and limited data exist regarding pacing in adult Fontan patients. The objectives of our study were to determine risk factors for pacing and occurrence of device-related complications (DRCs) and pacemaker reinterventions., Methods: We performed a retrospective review of Fontan patients from 1994 through 2014. We defined DRCs as lead failure, lead recall, cardiac perforation, lead thrombus/vegetation, or device-related infection, and cardiovascular adverse events (CAEs) as venous thrombosis, stroke, death, or heart transplant. Pacemaker reintervention was defined as lead failure or recall., Results: Of 439 patients, 166 (38%) had pacemakers implanted (79 during childhood; 87, adulthood); 114 patients (69%) received epicardial leads initially, and 52 (31%), endocardial leads. Pacing was initially atrial in 52 patients (31%); ventricular, 30 (18%); or dual chamber, 84 (51%). There were 37 reinterventions (1.9% per year) and 48 DRCs (2.4% per year). Pacemaker implantation during childhood was a risk factor for DRCs (hazard ratio, 2.01 [CI, 1.22-5.63]; P = .03). There were 70 CAEs (venous thrombosis, 5; stroke, 11; transplant, 8; and death, 46), yielding a rate of 3.5% per year. DRCs, CAEs, and reintervention rates were comparable for patients with epicardial or endocardial leads., Conclusions: More than one-third of adult Fontan patients referred to Mayo Clinic had pacemaker implantation. Epicardial leads were associated with high rate of pacemaker reinterventions but similar DRC rates in comparison to endocardial leads., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Background: Left atrial appendage emptying flow velocity (LAAEV) depends largely on left atrioventricular compliance and may play a role in mediating the perpetuation of atrial fibrillation (AF) and AF-related outcomes., Methods: We identified 3,251 consecutive patients with sustained AF undergoing first-time successful transesophageal echocardiography (TEE)-guided electrical cardioversion who were enrolled in a prospective registry between May 2000 and March 2012. Left atrial appendage emptying flow velocity was stratified into quartiles: ≤20.2, 20.3-33.9, 34-49.9, and ≥50 cm/s. Multivariate Cox regression models were used to identify independent predictors of AF recurrence, ischemic stroke, and all-cause mortality., Results: The mean (SD) age was 69 (12.6) years and 67% were men. Compared with the fourth quartile, patients in the first-third quartiles were significantly older, had higher CHA2DS2-VASc (congestive heart failure, hypertension, age ≥75 years, diabetes mellitus, stroke/transient ischemic attack [TIA], vascular disease, age 65-74 years, sex category) scores, greater frequency of atrial spontaneous echo contrast, and AF of longer duration. Kaplan-Meier analysis showed a decreased probability of event-free survival with decreasing quartiles of LAAEV. Five-year cumulative event rates across first-fourth quartiles were 83%, 80%, 73%, and 73% (P < .001) for first AF recurrence; 7.5%, 7.0%, 4.1%, and 4.0%, for stroke (P = .01); and 31.3%, 26.1%, 24.1%, and 19.4%, for mortality (P < .001), respectively. Multivariate Cox regression analysis revealed an independent association of the first and second quartiles with AF recurrence (P < .001 and P < .001, respectively) and stroke (P = .03, and P = .04, respectively), and of the first quartile with mortality (P = .003)., Conclusions: Patients with decreased LAAEV have an increased risk of AF recurrence, stroke, and mortality after successful electrical cardioversion. Real-time measurement of LAAEV by TEE may be a useful physiologic biomarker for individualizing treatment decisions in patients with AF., (Copyright © 2015 The Authors. Published by Elsevier Inc. All rights reserved.)

Background: Postoperative atrial fibrillation (POAF) is a common complication after cardiac surgery. Data are lacking on the long-term prognostic implications of POAF. We hypothesized that POAF, which reflects underlying cardiovascular pathophysiologic substrate, is a predictive marker of late AF and long-term mortality., Methods: We identified 603 Olmsted County, Minnesota, residents without prior documented history of AF who underwent coronary artery bypass graft and/or valve surgery from 2000 to 2005. Patients were monitored for first documentation of late AF or death at >30 days postoperatively. Multivariate Cox regression models were used to assess the independent association of POAF with late AF and long-term mortality., Results: After a mean follow-up of 8.3 ± 4.2 years, freedom from late AF was less with POAF than no POAF (57.4% vs 88.9%, P < .001). The risk of late AF was highest within the first year at 18%. Univariate analysis demonstrated that POAF was associated with significantly increased risk of late AF [hazard ratio (HR), 5.09; 95% CI, 3.65-7.22] and long-term mortality (HR, 1.79; 95% CI, 1.38-2.22). After adjustment for age, sex, and clinical and surgical risk factors, POAF remained independently associated with development of late AF (HR, 3.52; 95% CI, 2.42-5.13) but not long-term mortality (HR, 1.16; 95% CI, 0.87-1.55). Conversely, late AF was independently predictive of long-term mortality (HR, 3.25; 95% CI, 2.42-4.35). Diastolic dysfunction independently influenced the risk of late AF and long-term mortality., Conclusions: Postoperative atrial fibrillation was an independent predictive marker of late AF, whereas late AF, but not POAF, was independently associated with long-term mortality. Patients who develop new-onset POAF should be considered for continuous anticoagulation at least during the first year following cardiac surgery., (Copyright © 2015 The Authors. Published by Elsevier Inc. All rights reserved.)

Background: The effect of patient sex on recurrence of atrial fibrillation after a successful direct current cardioversion is unknown., Methods: This prospective study included 773 patients (486 [63%] men and 287 [37%] women) undergoing successful direct current cardioversion of atrial fibrillation between May 2000 and July 2003. Patient characteristics at presentation were recorded. The primary end point was the time between cardioversion and the first documented recurrence of arrhythmia., Results: At presentation, women were older and had a higher prevalence of hypertension and valvular disease compared with men. In addition, women had worse mechanical left atrial appendage function. Arrhythmia recurrence was more prevalent in women (50.0% at 1 year compared with 43.4% in men, and 75.8% at 2 years compared with 67.0% in men; P = .03). On the basis of multivariate analysis, patient sex was a significant predictor of arrhythmia recurrence. There was no significant difference in overall mortality between men and women., Conclusions: Women were more likely than men to have recurrence of atrial fibrillation after successful direct current cardioversion. Patient sex should be taken into account with other clinical factors when making the decision about cardioversion for atrial fibrillation.

Background: Transthoracic cardioversion fails to restore sinus rhythm in 6% to 33% of patients with atrial fibrillation. This study sought to determine the relative efficacy of biphasic waveforms compared with monophasic waveforms in the treatment of atrial arrhythmias., Methods: A total of 912 patients underwent 1022 transthoracic cardioversions between May 2000 and December 2001. A monophasic damped sine waveform was used in the first 304 cases, and a rectilinear biphasic defibrillator was used in the next 718 cases., Results: Use of a biphasic waveform was associated with 94% success in conversion to sinus rhythm compared with 84% with a monophasic waveform (P < .001). The cumulative energy required to restore sinus rhythm was lower with biphasic shocks in both atrial fibrillation and atrial flutter groups (554 +/- 413 J for monophasic vs 199 +/- 216 J for biphasic shocks in the atrial fibrillation group, P < .001; 251 +/- 302 J vs 108 +/- 184 J, respectively, in the atrial flutter group, P < .001). In a multivariate analysis, use of a biphasic shock was associated with a 3.9-fold increase in success of cardioversion., Conclusion: When used to cardiovert atrial arrhythmias, the rectilinear biphasic waveform was associated with higher success rates and lower cumulative energies than the monophasic damped sine waveform.

The purpose of this study was to determine the most discriminating clinical and echocardiographic features that are most helpful in correctly identifying Ebstein's anomaly of the tricuspid valve from other causes of tricuspid regurgitation. Ebstein's anomaly is an uncommon malformation of the tricuspid valve with diagnostic echocardiographic features. Other cardiac disorders associated with tricuspid valve regurgitation and predominate right-sided heart chamber enlargement can be misdiagnosed as Ebstein's anomaly. All patients who were referred to our institution between 1982 and 1995 with the diagnosis of Ebstein's anomaly but were found to have other abnormalities of the tricuspid value or right ventricle were identified. Their clinical, echocardiographic, and surgical records were reviewed retrospectively. Twenty-two patients (12 males and 10 females), aged 7 to 68 years (mean 33 years), were referred to our institution with the diagnosis of Ebstein's anomaly but were found to have another abnormality that mimicked clinical and diagnostic features of Ebstein's anomaly. The most common initial symptom was exercise intolerance (13 [59%] patients) followed by atrial arrhythmia (seven [32%] patients). Two patients had cyanosis. Three patients had paroxysmal and six had chronic atrial fibrillation/flutter. Cardiomegaly on chest x-ray film was noted in 18 (82%) patients. Referral diagnosis of Ebstein's anomaly had been made by echocardiography (12 patients), cardiac catheterization (four patients), both techniques (five patients), and echocardiography and magnetic resonance imaging (one patient). All 22 patients had predominate right atrial and right ventricular enlargement, and 18 (82%) of 22 patients also had right ventricular dysfunction. However, Ebstein's anomaly was confidently ruled out with repeat comprehensive echocardiography at our institution by establishing (1) absence of significant apical displacement of the septal tricuspid valve leaflet (> or = 8 mm/m2) and (2) lack of a redundant, elongated, anterior tricuspid valve leaflet in all 22 patients (100%). All had significant tricuspid regurgitation caused by tricuspid valve dysplasia (nine patients), tricuspid valve prolapse (four patients), trauma (four patients), right ventricular dysplasia (three patients), endocarditis (one patient), and annular dilation caused by free pulmonary regurgitation (one patient). In all 15 patients who subsequently underwent surgery (tricuspid valve repair [seven patients] or replacement [eight patients]), the absence of Ebstein's anomaly was confirmed. Echocardiographic absence of the characteristic degree of displacement of the septal leaflet of the tricuspid valve (> or = 8 mm/m2) and the presence of a nonelongated, nonredundant anterior tricuspid valve leaflet consistently excluded the diagnosis of Ebstein's anomaly. Under such circumstances, other anomalies of the tricuspid valve or right ventricle were consistently identified. Recognition of the mimics of Ebstein's anomaly had important surgical implications.

Atrial pacing and dipyridamole transesophageal echocardiography have been shown to be sensitive and specific tests for the detection of coronary artery disease. However, the sensitivity and specificity of dobutamine transesophageal echocardiography have not been reported. The purpose of this study was to determine the feasibility, sensitivity, and specificity of dobutamine transesophageal echocardiography for the detection of coronary artery disease. Transesophageal echocardiographic assessment of left ventricular function was performed in 81 adult patients aged 62 +/- 12 years during stepwise infusion of dobutamine from 5.0 to 40 micrograms/kg/min. Ischemia was diagnosed by the development of severe hypokinesis, akinesis, or dyskinesis of a previously contractile left ventricular segment. Coronary artery disease was defined by angiography as a reduction in luminal diameter of > or = 70% of an epicardial or > or = 50% of the left main coronary artery. In patients who had undergone coronary artery bypass graft surgery, a stenotic bypass graft was defined as a reduction in luminal diameter of > or = 70%. In patients without previous CABG, significant coronary artery disease was present in 21 patients: 5 with single-vessel disease, 7 double-vessel disease, 8 triple-vessel disease, and 1 left main coronary disease. Dobutamine transesophageal echocardiography had a sensitivity of 90% (19 of 21) and specificity of 94% (49 of 52) for the detection of coronary artery disease. In patients with previous CABG (n = 8), the sensitivity and specificity for the detection of bypass graft stenosis were 100% (4 of 4) and 75% (3 of 4), respectively.(ABSTRACT TRUNCATED AT 250 WORDS)

Two-dimensional transthoracic echocardiography has been shown to be a reliable and accurate method of measuring stenotic mitral valve orifice area. Little data exist on the role of two-dimensional transesophageal echocardiography for this purpose. Thus in 45 adult patients with mitral stenosis mitral valve area was determined by direct planimetry with the use of two-dimensional transesophageal and transthoracic echocardiography. Transesophageal was less feasible than transthoracic echocardiography in the 45 patients (69% vs. 89%, p < 0.025). In 14 patients, two-dimensional transesophageal echocardiography was not feasible, primarily because of leaflet dropout. In 30 patients, transesophageal and transthoracic echocardiography were feasible, and measurements of mitral valve area by the two techniques correlated well (r = 0.91, SEE = 0.33 cm2, p < 0.0001). Mean mitral valve orifice area determined by transesophageal echocardiography (1.54 +/- 0.75 cm2; range 0.56 to 3.49 cm2) and by transthoracic echocardiography (1.55 +/- 0.78 cm2; range 0.62 to 3.68 cm2) did not differ (p = NS). The absolute (0.24 +/- 0.22 cm2) and percent (19% +/- 21%) differences between mitral valve area determined by transesophageal versus transthoracic echocardiography were small. These data show that mitral valve area in patients with mitral stenosis can be accurately measured by direct planimetry with two-dimensional transesophageal echocardiography. Technical refinements such as lateral-gain-compensation features may improve the feasibility of two-dimensional transesophageal echocardiography for measurements of mitral stenosis area, and this technique may become an adjunct to transthoracic echocardiography in the assessment of severity of mitral stenosis.