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Mohamad S Alabdaljabar,1,2 Babar Hasan,3 Peter A Noseworthy,4 Joseph F Maalouf,4,5 Naser M Ammash,4,5 Shahrukh K Hashmi5,6 1Department of Internal Medicine, Mayo Clinic, Rochester, MN, USA; 2College of Medicine, Alfaisal University, Riyadh, Saudi Arabia; 3Sindh Institute of Urology and Transplantation (SIUT), Karachi, Pakistan; 4Department of Cardiovascular Medicine, Mayo Clinic, Rochester, MN, USA; 5Department of Medicine, Sheikh Shakhbout Medical City, Abu Dhabi, United Arab Emirates; 6Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, MN, USACorrespondence: Shahrukh K Hashmi, Department of Medicine, SSMC, Abu Dhabi, United Arab Emirates, Email shhashmi@ssmc.aeAbstract: Artificial intelligence (AI) and machine learning (ML) is a promising field of cardiovascular medicine. Many AI tools have been shown to be efficacious with a high level of accuracy. Yet, their use in real life is not well established. In the era of health technology and data science, it is crucial to consider how these tools could improve healthcare delivery. This is particularly important in countries with limited resources, such as low- and middle-income countries (LMICs). LMICs have many barriers in the care continuum of cardiovascular diseases (CVD), and big portion of these barriers come from scarcity of resources, mainly financial and human power constraints. AI/ML could potentially improve healthcare delivery if appropriately applied in these countries. Expectedly, the current literature lacks original articles about AI/ML originating from these countries. It is important to start early with a stepwise approach to understand the obstacles these countries face in order to develop AI/ML-based solutions. This could be detrimental to many patients’ lives, in addition to other expected advantages in other sectors, including the economy sector. In this report, we aim to review what is known about AI/ML in cardiovascular medicine, and to discuss how it could benefit LMICs.Keywords: artificial intelligence, machine learning, cardiology, low, middle, income, countries

Objective: To determine the long-term outcome of patients after surgical repair for pulmonary valve stenosis (PVS). Patients and Methods: Fifty-three patients (30 males; mean - SD age, 10+/-13 years; range, 5 days to 50 years) were identified who had surgical treatment for PVS between 1951 and 1982. The status of each patient was determined by medical record review. Results: The mean +/- SD age at follow-up was 43+/-15 years (age range, 19-77 years). Mean follow-up was 33 years (range, 18-51 years). At a median follow-up of 34 years, 35 reinterventions had been performed in 28 patients (53%), Including pulmonary valve replacement for free pulmonary regurgitation in 21 patients (mean interval after initial surgery, 33 years; range, 14-45 years), open valvotomy in 5 and pulmonary balloon valvuloplasty in 3 for residual PVS, closure of atrial septal defect in 2, right ventricular outflow tract reconstruction in 1, closure of iatrogenic ventricular septal defect in 1, ligation of aortopulmonary fistula in 1, and tricuspid valve annuloplasty with simultaneous coronary artery bypass grafting in 1. In addition, atrial and ventricular arrhythmias were common, occurring in 20 patients (38%). Patients who underwent reintervention were more likely to have undergone closed pulmonary valvotomy as the initial repair (P=.008). Conclusion: Although overall survival after surgical treatment of isolated PVS remains excellent, many patients undergo late reintervention after 30 years of follow-up, emphasizing the need for lifelong cardiac follow-up. [ABSTRACT FROM AUTHOR]

Infections due to Coxiella burnetii, the causative organism of Q fever, are extremely rare in North America. Endocarditis due to the organism has an unusual presentation and poses echocardiographic and laboratory challenges in establishing a diagnosis. We describe the presentation and clinical course of a 40-year-old American man with Q fever endocarditis and briefly discuss the salient issues regarding this entity. [ABSTRACT FROM AUTHOR]

OBJECTIVE: To determine the frequency of intracranial aneurysms (IAs) detected in patients with coarctation of the aorta (CoA) with use of magnetic resonance angiography. PATIENTS AND METHODS: From January 1, 1980, to September 30, 2002, 277 adult patients with CoA were seen at the Mayo Clinic in Rochester, Minn, and were invited to participate in a study to detect IAs. Of these 277 patients (mean +/- SD age, 41.6 +/- 16.5; 70 men), 100 underwent cranial magnetic resonance angiography. RESULTS: Ten patients had an IA (95% confidence interval, 5%-18%), with a mean diameter of 3.5 mm (range, 2.0-8.0 mm). The frequency of IA was significantly higher than that predicted in the general population (10% vs 2%; P < .001). One patient had surgical intervention. No clinical or geographical differences were found between patients with CoA and IA and patients with CoA and no IA. CONCLUSIONS: The frequency of IA among patients with CoA is approximately 5-fold that of the general population. Although no risk factors were identified in this cohort, additional prospective evaluation is warranted. These data suggest that noninvasive cerebral imaging to screen for IA should be considered in patients with CoA. [ABSTRACT FROM AUTHOR]

Background: This retrospective review focuses on peripartum anesthetic management and outcome of a series of five pregnant women with left ventricular noncompaction (LVNC)., Methods: The Mayo Clinic Advanced Cohort Explorer medical database was utilized to identify women diagnosed with LVNC who had been admitted for delivery at the Mayo Clinic in Rochester, Minnesota, between January 2001 and September 2021. Echocardiograms were independently reviewed by two board-certified echocardiographers, and those determined by both to meet the Jenni criteria and/or having compatible findings on magnetic resonance imaging (MRI) were included. Electronic medical records were reviewed for information pertaining to cardiac function, labor, delivery, and postpartum management., Results: We identified 44 patients whose medical record included the term "noncompaction" or "hypertrabeculation" and who had delivered at our institution during the study period. Upon detailed review of the medical records, 36 did not meet criteria for LVNC, and three additional patients did not receive the diagnosis until after delivery, leaving five patients with confirmed LVNC who had undergone six deliveries during the study interval. All five patients had a history of arrhythmias or had developed arrhythmias during pregnancy. One patient underwent emergency cesarean delivery due to sustained ventricular tachycardia requiring three intra-operative cardioversions., Conclusions: This case series adds new evidence to that already available about pregnancies among women with LVNC. Favorable obstetrical outcomes were achievable when multidisciplinary teams were prepared to manage the maternal and fetal consequences of intrapartum cardiac arrhythmias and hemodynamic instability., Competing Interests: Declaration of interests None., (Copyright © 2022 Elsevier Ltd. All rights reserved.)

The multifaceted connections between the heart and the brain have been extensively studied at the anatomy, pathophysiology, and clinical levels. Studies have suggested a vital role for both cardiologists and neurologists in the management of various cardiovascular and neurological disorders. However, a true heart-brain team-based approach remained confined to large, specialized centers. In this paper, we review the various intersection areas of cardiology and neurology with regard to ischemic stroke. We focus our discussion on the challenges and opportunity for a heart-team approach to stroke in the context of atrial fibrillation, carotid disease, and patent foramen ovale, and in the setting of strokes complicating transcatheter endovascular interventions., (© 2022 The Authors.)

Background: Left atrial (LA) function can be impaired by the atrial fibrillation (AF) ablation and might be associated with the risk of recurrence. We sought to determine whether the post-procedural changes in LA function impact the risk of recurrence following AF ablation., Methods: We retrospectively reviewed patients who underwent AF ablation between 2009 and 2011 and underwent transthoracic echocardiography before ablation, 1-day and 3-month after ablation. Peak left atrial contraction strain (PACS) and left atrial emptying fraction (LAEF) were evaluated during sinus rhythm and compared across the three time points. The primary endpoint was atrial tachyarrhythmia recurrence after ablation., Results: A total of 144 patients were enrolled (mean age 61 ± 11 years, 77% male, 46% persistent AF). PACS and LAEF initially decreased 1-day following ablation but partially recovered within 3 months in PAF patients, with a similar trend in the PerAF patients. After median 24 months follow-up, 68 (47%) patients had recurrence. Patients with recurrence had higher PACS 1-day than that in non-recurrence subjects (-10.9 ± 5.0% vs. -13.4 ± 4.7%, p = 0.003). PACS 1-day -12% distinguished recurrence cases with a sensitivity of 67.7% and specificity of 60.5%. The Kaplan-Meier curves showed significant difference in 5-year cumulative probability of recurrence between those with PACS ≥ -12% and PACS < -12% (log rank p < 0.0001). Multivariate regression showed that PACS 1-day was an independent risk factor of arrhythmia recurrence., Conclusions: Left atrial function deteriorates immediately following AF ablation and partially recovers in 3 months but remains abnormal in the majority of patients. PACS 1-day post procedure predicts arrhythmia recurrence at long-term follow-up.

Background: Von Willebrand factor (VWF) elevation correlates with the left atrial blood stasis in nonvalvular atrial fibrillation (NVAF). However, the long-term impact of elevated VWF in patients with NVAF is not well established., Methods: To assess the impact of VWF and a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13) in conjunction with echocardiographic measures of left atrium blood stasis on clinical outcomes, 414 NVAF prospectively recruited (October 4, 2007, to April 27, 2009) patients were followed for 3 years. VWF antigen, VWF activity, ADAMTS13 activity, and echocardiographic findings were assessed at baseline. Thromboembolism (TE) (stroke/transient ischemic attack (TIA)), myocardial infarction, or TE of other locations), major bleeding, clinically relevant nonmajor bleeding, and all-cause mortality were assessed by clinical follow-up, questionnaire, or telephone communication., Results: Among 374 patients (mean age, 63.4 ± 12.7 years; 25% females) who had complete follow-up data, there were 33 TE in 32 patients (8.6%), 18 deaths (5.1%), and 33 bleeding events (21 major bleeding and 12 clinically relevant nonmajor bleeding) in 25 patients (6.7%). VWF antigen was predictive of TE in the univariate examination (hazard ratio [HR]: 1.007, 95% confidence interval [CI]: 1.002, 1.013, P  = 0.011) but not in multivariate analysis. VWF was an independent predictor of all-cause mortality (HR: 1.011, 95% CI: 1.003, 1.020, P  = 0.011) and a composite of TE and all-cause mortality (HR: 1.006, 95% CI: 1.001, 1.012, P  = 0.039) in multivariate analysis. ADAMTS13 was not predictive of clinical outcomes in multivariate analysis., Conclusions: Among patients with NVAF, VWF is an independent predictor of poor outcomes including death and a composite of death and TE. As such, VWF measure may help identify high-risk patients and provide further stratification beyond CHA 2 DS 2 -VASc assessment., (© 2020 Canadian Cardiovascular Society. Published by Elsevier Inc.)

Objectives: The purpose of this retrospective cohort study was to compare remodeling of left ventricular (LV) structure and function after transcatheter stent therapy with remodeling of LV structure and function after surgical therapy for COA., Background: Transcatheter stent therapy is as effective as surgery in producing acute hemodynamic improvement in patients with coarctation of aorta (COA). However, LV remodeling after transcatheter COA intervention has not been systematically investigated., Methods: LV remodeling was assessed at 1, 3, and 5 years post-intervention by using LV mass index (LVMI), LV end-diastolic dimension, LV ejection fraction, LV global longitudinal strain (LVGLS), LV mitral annular tissue Doppler early velocity (LVe'), and ratio of mitral inflow pulsed wave Doppler early velocity and e' (E/e') ratio., Results: There were 44 patients in the transcatheter group and 128 patients in the surgical group. Compared to the surgical group, the transcatheter group had less regression of LVMI (-4.6; 95% confidence interval [CI]: -5.5 to -3.7 vs. -7.3; 95% CI: -8.4 to -6.6 g/m 2 ; p < 0.001), less improvement in LVGLS (2.1; 95% CI: 1.8 to 2.4 vs. 2.9; 95% CI: 2.6 to 3.2%; p = 0.024), and in e' (1.0 ; 95% CI: 0.7 to 1.2 vs. 1.5 ; 95% CI: 1.3 to 1.7 cm/s; p = 0.009) at 5 years post-intervention. Exploratory analysis showed a correlation between change in LVMI and LVGLS, and between change in LVMI and mitral annular tissue Doppler early velocity (e'), and this correlations were independent of the type of intervention received., Conclusions: Transcatheter stent therapy was associated with less remodeling of LV structure and function during mid-term follow-up. As transcatheter stent therapy becomes more widely used in the adult COA population, there is a need for ongoing clinical monitoring to determine if these observed differences in LV remodeling translate to differences in clinical outcomes., (Copyright © 2020 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Objectives: To evaluate the outcomes of transcatheter coronary artery fistula (CAF) closure and to identify anatomic/procedural factors that may impact outcomes., Background: Due to the rarity of CAF, reported experience with transcatheter closure remains limited and anatomic and procedural factors that may lead to unsuccessful closure, complications, or recanalization of CAF are unclear., Methods: All patients who underwent transcatheter CAF closure at Mayo Clinic from 1997 to 2018 were retrospectively reviewed. CAF anatomic characteristics, procedural techniques, and clinical/angiographic outcomes were assessed., Results: A total of 45 patients underwent transcatheter closure of 56 CAFs. The most commonly used devices were embolization coils in 40 (71.4%) CAFs, vascular occluders in 10 (17.8%), or covered stent in 2 (3.6%). Acute procedural success with no or trivial residual flow occurred in 50 (89.3%) CAFs. Residual flow was small in three (5.4%) and large in three (5.4%). Eight (17.8%) patients had complications, including device migration in three, intracranial hemorrhage from anticoagulation in one, and myocardial infarction (MI) in four. MI was a result of covered stent thrombosis or stagnation of flow after closure of large distal CAF. Twenty-two patients with 27 CAFs had follow-up angiography after successful index procedure at median time of 423 (IQ 97-1348) days. Of these, 23 (85.2%) had no/trace flow and 4 had large flow from recanalization., Conclusions: Transcatheter CAF closure is associated with a favorable acute procedural success and complication rate in selected patients. Procedural success and risk for complication are highly dependent on CAF anatomy and closure technique., (© 2020 Wiley Periodicals, Inc.)

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.

A 65-year-old woman presented with an acute inferior ST-segment elevation myocardial infarction and was found to have a rare single coronary artery anomaly on coronary angiography. A complete occlusion at the mid right coronary artery was identified as the culprit lesion and underwent successful percutaneous revascularization with stenting. ( Level of Difficulty: Beginner. )., (© 2020 The Authors.)

Background: Left heart filling pressures, as measured by pulmonary artery wedge pressure (PAWP), is associated with heart failure related mortality. Because of the prognostic importance of PAWP, several echocardiographic indices have been proposed for noninvasive assessment of PAWP. However, these indices have not been validated in the congenital heart disease population. The purpose of this study was to determine the correlation between echocardiographic indices of PAWP, and the effect of high PAWP on transplant-free survival in adults with tetralogy of Fallot (TOF)., Methods: Retrospective study of adult TOF patients that underwent cardiac catheterization at Mayo Clinic, 1990-2017. We selected these pre-defined set of echocardiographic indices of LV diastolic function: mitral valve early velocity (E), mitral valve early and late velocity ratio (E/A), mitral valve deceleration time (DT), mitral annular tissue Doppler early velocity (e'), and left atrial volume index (LAVI)., Results: Of the echocardiographic indices analyzed among 213 patients (age 37 ± 14 years), only E velocity (β = 5.83, standard error = 1.52, p < 0.001) and LAVI (β = 0.14, standard error = 0.05, p = 0.007) correlated with PAWP. LAVI > 28 ml/m 2 had sensitivity of 79% and specificity of 63% (AUC 0.712), and E velocity > 1.0 m/s had sensitivity of 66% and specificity of 89% (AUC 0.692), for detecting PAWP > 15 mmHg. LAVI > 28 ml/m 2 was associated with reduced 10-year transplant-free survival (49% vs 90%, p < 0.001)., Conclusion: This study supports the use of LAVI for noninvasive assessment of PAWP and for prognostication. Further studies are required to validate these results in a different population., Competing Interests: The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (© 2019 Published by Elsevier B.V.)

Exercise-induced hypertension is a predictor of cardiovascular events in patients with coarctation of aorta (COA). However, it is unclear whether mild COA diagnosis is an independent risk factor of exercise-induced hypertension. We hypothesized that for every unit increase in exercise, patients with COA (without hemodynamically significant coarctation) will have a higher rise in systolic blood pressure (SBP) compared with matched controls. One hundred forty-nine patients with COA (aortic coarctation peak velocity <2 m/s) who underwent exercise testing were matched 1:1 to controls using propensity score method based on age, sex, body mass index, hypertension diagnosis, and SBP at rest. We compared exercise-induced change in SBP between patients with COA and controls and also assessed the correlation between Doppler-derived aortic vascular function indices (effective arterial elastance index and total arterial compliance index) and exercise-induced changes in SBP. Compared with controls, patients with COA had a greater change in SBP per unit metabolic equivalent (β=2.86; 95% CI, 1.96-4.77 versus 1.07, 95% CI, -0.15 to 1.75; P =0.018) and per unit oxygen pulse (β=4.57; 95% CI, 2.97-7.12 versus 1.45, 95% CI, -0.79 to 2.09, P <0.001). There was a correlation between SBP peak -SBP rest and elastance index ( r =0.38, P =0.032) and between SBP peak -SBP rest and total arterial compliance index ( r =-0.51, P =0.001), suggesting an association between vascular dysfunction and exercise-induced BP changes. Patients with COA, without significant obstruction, had higher exercise-induced changes in SBP after adjustment for other risk factors for hypertension. Considering the already known prognostic importance of exercise-induced hypertension, the current study highlights the potential role of exercise testing for risk stratification of patients with mild COA.

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.)

Cardiac magnetic resonance imaging derived right ventricular (RV) volumes are often necessary for optimal timing of pulmonary valve replacement in patients with tetralogy of Fallot (TOF). This practice is based on previous studies that reported preoperative RV volumetric thresholds that predicted postoperative RV remodeling. As a result, pulmonary valve replacements are being performed even in asymptomatic patients based on RV volumetric thresholds that predict complete postoperative RVOT remodeling. Hence, RV volumes are now being used as surrogate markers/endpoints for future cardiovascular outcomes. Unfortunately, there are no studies showing survival benefit for performing pulmonary valve replacement at smaller RV volumes. This review underscores some of the limitations of using RV volumes as surrogate markers for clinical outcomes, and also highlights knowledge gaps about the pathophysiologic mechanism of cardiovascular death in the TOF population., Competing Interests: The authors declare that they have no competing financial interests for this paper., (© 2019 The Authors.)

Right ventricular (RV) volume overload due to chronic pulmonary regurgitation is the common mechanism for hemodynamic deterioration after tetralogy of Fallot (TOF) repair. As a result, RV volumetric indices are used for clinical risk stratification in this population. Since RV afterload is a determinant of RV hemodynamic performance, we hypothesized that afterload-adjusted RV volumetric indices will have a better correlation with disease severity compared with RV volumetric indices alone in patients with TOF. Cross-sectional study of adults with previous TOF repair that received care at Mayo Clinic, 2002-2015. We defined disease severity as atrial arrhythmia and/or impaired exercise capacity. We created afterload-adjusted RV volumetric indices by indexing these indices to RV systolic pressure (RVSP) as follows: RV end-diastolic volume (RVEDVi)/RVSP, RV end-systolic volume (RVESVi)/RVSP, and RV ejection fraction (RVEF)/RVSP. The RV volumetric indices were: RVEDVi 141 ± 43 ml/m 2 , RVESVi 79 ± 38 ml/m 2 , and RVEF 44 ± 10%, and RVSP was 48 ± 9 mm Hg. RVESVi was the only RV volumetric parameter that was associated with disease severity (odds ratio [OR] 1.13, 95% confidence interval [CI] 1.01 to 1.32, p = 0.041) with area under the curve (AUC) of 0.612. In contrast RVEF/RVSP (OR 0.73, 95% CI 0.38 to 0.92, p = 0.037, AUC 0.649), and RVESVi/RVSP (OR 1.28, 95% CI 1.14-1.55, p = 0.008, AUC 0.798) were associated with disease severity. Compared with RV volumetric indices alone, the combined RV volumetric and afterload indices had better correlation with disease severity as measured by AUC. Afterload-adjusted RV volumetric indices had better correlation with disease severity, and may potentially improve risk stratification in this population., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: There are limited data about the outcomes mechanical prostheses in adults with tetralogy of Fallot (TOF). The purpose of the study was to describe the incidence of mechanical valve related adverse events (MVRAE), reoperation and all-cause mortality in TOF patients with mechanical valve prostheses., Methods: We reviewed the MACHD (Mayo Adult Congenital Heart Disease) database and identified all adult TOF patients with mechanical valve prostheses, 1990-2017. MVRAE was defined as valve thrombosis, endocarditis, embolic stroke or major bleeding complications., Results: A total of 44 prostheses were implanted in 29 patients (age 44 ± 13 years; men 18 [62%]), and 10 (36%) patients received multiple mechanical prostheses. The median number of prior sternotomies was 3 (range 2-7). Target intentional normalized ratio (INR) was 2.0-3.0 for patients with isolated mechanical aortic prostheses (n = 12, 41%), 2.5-4.0 for mechanical prostheses in non-aortic positions. There were no surgical deaths, and 10 MVRAE (endocarditis [n = 4], major bleeding complications [n = 5] and valve thrombosis [n = 1]) occurred in 7 (24%) patients during a median follow-up of 11 (5 18) years. The 10-year survival and freedom from reoperation were 87% and 95% respectively., Conclusions: Surgical mortality and valve thrombosis for mechanical valve prostheses are low presumably due to appropriate patient selection and meticulous anticoagulation. Endocarditis and bleeding complications are major concerns. Further studies are required to determine the appropriate target INR that provides the optimal balance between preventing valve thrombosis and avoiding major bleeding complications mechanical valve prostheses in different positions., (Copyright © 2019. Published by Elsevier B.V.)

There is a paucity of cardiac surgery outcomes data for patients with Noonan syndrome (NS). Our objective was to evaluate early results in these patients. Between January 1999 and December 2015, 29 patients (18 males, 62%) with NS underwent cardiac surgery at our institution. Mean age was 23 ± 17.9 years; 12 (41%) were under 18 years of age. Fourteen patients (48%) had prior sternotomies. The primary operations for the main diagnosis were pulmonary valve/conduit replacement/repair (n = 14, 48%), septal myectomy for obstructive hypertrophic cardiomyopathy (n = 7, 24%), aortic valve replacement/repair (n = 4, 14%), atrial septal defect (ASD) repair (n = 2, 7%), and cardiac transplantation (n = 2, 7%). Concomitant procedures were performed in 24 patients (83%), most commonly right ventricular outflow tract reconstruction (n = 13, 45%), mitral valve repair/replacement (n = 7, 24%), and ASD repair (n = 6, 21%). Mean bypass and cross-clamp times were 88.8 ± 51 minutes and 54.7 ± 67 minutes, respectively. There was 1 early death (3%). Postoperative morbidity occurred in 18 patients (62%), most commonly arrhythmias (n = 14, 48%) or respiratory insufficiency/pneumonia (n = 6, 21%). There were 2 early reoperations and 4 early readmissions. Univariate factors associated with morbidity included male gender (P = 0.03) and longer cross-clamp time (P = 0.02). Median length of hospital stay was 6 days (interquartile range 5-10.5 days). Patients with NS frequently have multiple cardiac lesions requiring a broad spectrum of operations. Early mortality is low despite procedure complexity. Although early postoperative morbidity is common, patients overall do well with reasonable hospital lengths of stay. Additional studies are needed to evaluate long-term outcomes and quality of life., (Copyright © 2018. Published by Elsevier Inc.)

One of the goals of lifelong care in adults with tetralogy of Fallot (TOF) is early identification and treatment of patients at high risk for adverse events. Clinical risk stratification tools are critical for achieving this goal. We reviewed the Mayo Adult Congenital Heart Disease database and identified 465 TOF patients (age 37 ± 14 years, men 223 [48%]) seen at Mayo Clinic Rochester between 1990 and 2017. The aim was to determine the risk factors for death and/or heart transplant through a comprehensive analysis of 8 groups of variables (demographics, co-morbidities, medications, heart rhythm, echocardiography, cardiac magnetic resonance imaging, cardiac catheterization, and cardiopulmonary exercise test data) using univariable and multivariable Cox proportional hazard models. The end point of death and/or transplant occurred in 57 (12%) patients during a follow-up of 13.6 ± 8.2 years, yielding an event rate of 0.9% per year. Independent risk factors were age >42 years, atrial fibrillation, ≥moderate QRS fragmentation, left ventricular ejection fraction <50%, right ventricular end-diastolic pressure >16 mm Hg, and left ventricle end-diastolic pressure >16 mm Hg. There is nearly a twofold increase in the risk of death and/or transplant per unit increase in number of risk factors (hazard ratio 1.92, 95% confidence interval 1.62 to 2.27, p <0.001). In conclusion, the current study provides risk stratification indices based on a comprehensive risk model of all clinical variables in an unselected TOF population. Further studies are required to determine whether interventions targeted at modifying these risk factors will alter the annual event rate., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: There are limited outcome data in adults with tetralogy of Fallot and pulmonary atresia. The purpose of this study was to describe re-operations and all-cause mortality in adults with tetralogy of Fallot and pulmonary atresia., Methods: Retrospective review of adults with repaired tetralogy of Fallot and pulmonary atresia who received care at the Mayo Adult Congenital Heart Disease Clinic, 1990-2016. All-cause mortality was calculated as events per 100 patient-years from the time of first presentation to the Adult Congenital Heart Disease Clinic., Results: Of the 221 patients, the age at initial tetralogy of Fallot repair was 6 (5-13) years, and the age at first presentation to the clinic was 27 - 8 years. All patients had at least one right ventricular to pulmonary artery conduit re-operation. There were 31 deaths (14%) at mean age of 41 - 14 years. The causes of death were end-stage heart failure (n = 17), sudden cardiac death (n=9), post-operative death after cardiac surgery (n = 2), sepsis with multi-system organ failure (n = 2), and unknown (n = 1). All-cause mortality rate was 1.7 per 100 patient-years. The risk factors for all-cause mortality were older age (>12 years) at the time of repair (hazard ratio 1.41, 95 confidence interval 1.06-2.02, p = 0.033), non-sustained ventricular tachycardia (hazard ratio 1.36, 95 confidence interval 1.17-2.47, p = 0.015), and left ventricular ejection fraction <50% (hazard ratio 1.39, 95 confidence interval 1.08-2.31, p = 0.031)., Conclusion: Based on a review of 221 adults with repaired tetralogy of Fallot and pulmonary atresia, all patients had re-operations and all-cause mortality rate was 1.7 events per 100 patient-years. The current study provides important outcomes data for risk stratification in adults with tetralogy of Fallot and pulmonary atresia.

Background: We hypothesized that noninvasively measured right ventricular (RV) to pulmonary arterial (RV-PA) coupling would be abnormal in chronic pulmonary regurgitation (PR) even in the setting of normal RV ejection fraction, and that RV-PA coupling indices would have a better correlation with peak oxygen consumption (VO 2 ) compared with RV systolic indices alone., Methods: This was a retrospective study of 129 adults (repaired tetralogy of Fallot [TOF] n = 84 and valvular pulmonic stenosis [VPS] with previous intervention n = 45) with ≥ moderate native PR and RV ejection fraction > 50%. The 84 TOF patients were propensity matched with 84 patients with normal echocardiogram (control); age 28 ± 7 years and male sex n = 39 (46%). RV-PA coupling was measured according to fractional area change (FAC)/RV systolic pressure (RVSP) and tricuspid annular plane systolic excursion (TAPSE)/RVSP., Results: RV systolic function indices were similar between TOF and control groups (FAC 43 ± 6% vs 41 ± 5% [P = 0.164] and TAPSE 22 ± 5 mm vs 24 ± 6 mm [P = 0.263]). However, RV-PA coupling was lower in the TOF group (FAC/RVSP 1.10 ± 0.29 vs 1.48 ± 0.22 [P < 0.001]; TAPSE/RVSP 0.51 ± 0.15 vs 0.78 ± 0.11 [P < 0.001]) because of higher RV afterload (RVSP 42 ± 3 mm Hg vs 31 ± 3 mm Hg [P = 0.012]). FAC/RVSP (r = 0.61; P < 0.001) and TAPSE/RVSP (r = 0.69; P < 0.001) correlated with peak VO 2 especially in the patients with impaired exercise capacity whereas FAC and TAPSE were independent of peak VO 2 . Similar comparisons between VPS and control groups showed no difference in TAPSE and FAC between groups, but lower FAC/RVSP and TAPSE/RVSP in the VPS group., Conclusions: There is abnormal RV-PA coupling in chronic PR, and noninvasively measured RV-PA coupling might potentially be prognostic because of its correlation with exercise capacity., (Copyright © 2019 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

Background The purpose of this study was to compare the incidence of pregnancy-related adverse outcomes ( PRAO ) between patients with versus without hemodynamically significant right ventricle outflow tract ( RVOT) . Methods and Results This was a retrospective cohort study of all pregnant patients with isolated RVOT lesions undergoing evaluation at the Mayo Clinic, 1990 to 2017. Hemodynamic significance was defined as ≥moderate pulmonary/conduit stenosis (≥3 m/s) and/or ≥moderate regurgitation. Patients with concomitant significant left heart disease were excluded. PRAO was defined as cardiovascular, obstetric, and/or neonatal complications occurring during the pregnancy through 6 weeks postpartum. A total of 224 pregnancies in 114 patients with RVOT lesions were identified; 38 pregnancies occurred in 24 patients with hemodynamically significant RVOT . Forty-eight (21%) pregnancies ended in spontaneous abortion. Of the 173 completed pregnancies, median gestational age at delivery was 38 (35-40) weeks and median birth weight 2965 (2065-4122) g. Seven pregnancies (4%) were complicated by cardiovascular events, 14 (8%) by obstetric complications, with adverse neonatal outcomes occurring in 38 (22%). There were no maternal deaths. The incidence of spontaneous abortion and PRAO were similar in both the RVOT and hemodynamically significant RVOT groups. As an isolated condition, Tetralogy of Fallot-pulmonary atresia was associated with spontaneous abortion and neonatal complications. Conclusions The risk of cardiovascular complications was low in patients with isolated RVOT lesions, and hemodynamically significant RVOT lesions were not associated with either cardiovascular complications or PRAO . Further studies are required to explore the factors responsible for PRAO in patients with Tetralogy of Fallot-pulmonary atresia.

Background: Aortic dilation and aortic valve disease are known long-term complication of tetralogy of Fallot (TOF), but the risk of aortic dissection and the indications for prophylactic aortic surgery are unknown in this population. The purpose of this study was to: (1) determine the prevalence of significant aortic valve disease and/or significant aortic aneurysm (AVD-AA); (2) determine the incidence of progressive aortic dilation and aortic dissection in patients with TOF., Methods: Retrospective review of adults with repaired TOF, and no prior aortic valve/aorta surgery, who had ≥2 measurements of the thoracic aorta >12 months apart, 1990-2017. The aortic root and mid-ascending aorta were measured at the onset of QRS complex from leading edge to leading edge. Significant aortic valve disease was defined as the presence of ≥moderate aortic stenosis and/or ≥moderate aortic regurgitation. Significant aortic aneurysm was defined as aortic root or mid-ascending aorta dimension ≥50 mm. Progressive aortic dilation was defined as increase in aortic dimension ≥2 mm., Results: Of the 453 consecutive patients (37±13 years, men 216 (49%)) in the study, aortic aneurysm was present in 312 (69%) based on normative data; progressive aortic dilation occurred in 40 (9%), and there was no case of aortic dissection. Significant AVD-AA occurred in 52 (12%) patients; and 15 of them (29%) underwent aortic surgery without any surgical mortality., Conclusions: Although aortic aneurysm was common, progressive aortic dilation was uncommon and aortic dissection did not occur in our patients with TOF with significant aortic aneurysms who did not undergo aortic surgery. This has important clinical implication in deciding the frequency of imaging follow-up and timing of surgical intervention in this population., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Objectives: This study hypothesized that atrial fibrillation was associated with heart failure (HF) hospitalization, and that patients who received rhythm control therapy had a lower incidence of HF hospitalization and mortality., Background: Atrial fibrillation is a known risk factor for HF hospitalization and mortality in patients with acquired heart disease. Although atrial arrhythmias are common in adults with tetralogy of Fallot (TOF), data about prevalence and outcomes of therapy for atrial fibrillation are very limited., Methods: The MACHD (Mayo Adult Congenital Heart Disease) database was queried for adults with repaired TOF and documented atrial fibrillation from 1990 to 2017. Primary endpoint was HF hospitalization defined as admission for volume overload (pulmonary congestion and/or peripheral edema) requiring intravenous diuretics. Secondary endpoint was the effect of rhythm control therapy on HF hospitalization and all-cause mortality. Patients were divided into rhythm control and rate control groups based on the therapy initiated at the time of arrhythmia diagnosis., Results: Of 415 patients, 27 (7%) had 42 HF hospitalizations. Of these 415 patients, 88 (21%) had atrial fibrillation at age 49 ± 13 years. Atrial fibrillation was an independent risk factor for HF hospitalization (adjusted hazard ratio: 2.67; 95% confidence interval: 1.04 to 7.34; p = 0.045). The 88 patients were divided into the rhythm control group (n = 61, 69%) and the rate control group (n = 27, 31%). The rate control group had higher unadjusted annual incidence of HF hospitalization (13% vs. 3%; p = 0.001) and all-cause mortality (11% vs. 4%; p = 0.002)., Conclusions: Atrial fibrillation was a risk factor for HF hospitalization and mortality in TOF patients, and rhythm control therapy was protective against these adverse events., (Copyright © 2019. Published by Elsevier Inc.)

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 Available outcomes data for anticoagulation therapy in adults with congenital heart disease ( CHD ) provide assessment of global risk of this therapy for CHD patients (a heterogeneous population), but the risk of complications for the different CHD diagnoses is unknown. The purpose of the study was to describe the indications for anticoagulation, and the incidence and risk factors for major bleeding complication in adults with tetralogy of Fallot. Methods and Results We queried Mayo Adult Congenital Heart Disease (MACHD) database for tetralogy of Fallot patients (aged ≥18 years) that received anticoagulation, 1990-2017. Of 130 patients (42±14 years, 75 men [58%]), warfarin and direct oral anticoagulants were used in 125 (96%) and 5 (4%), respectively because atrial arrhythmias (n=109), mechanical prosthetic valve (n=29), intracardiac thrombus (n=4), pulmonary embolism (n=6), stroke (n=3), and perioperative anticoagulation (n=44). The median hypertension, abnormal renal or liver function; stroke; bleeding history or predisposition; labile international normalized ratio; elderly (>65 years); drug or alcohol use score for the entire cohort was 1 (0-2) and 27 (21%) had hypertension, abnormal renal or liver function; stroke; bleeding history or predisposition; labile international normalized ratio; elderly (>65 years); drug or alcohol use score ≥2. There were 14 minor bleeding events (1.6% per year) and 11 major bleeding events (1.3% per year) in 8 patients during median follow-up of 74 months (856 patient-years). Mechanical prosthesis (hazard ratio 1.78, CI 1.29-3.77, P=0.021) and hypertension, abnormal renal or liver function; stroke; bleeding history or predisposition; labile international normalized ratio; elderly (>65 years); drug or alcohol use score ≥2 (hazard ratio 1.41, CI 1.03-3.88, P=0.046) were risk factors for major bleeding events. All-cause mortality was higher in patients with major bleeding events (n=6, 75%) compared with patients without major bleeding events (n=25, 21%), P=0.001. Conclusions Considering the heterogeneity of the CHD population, data from the current study may be better suited for clinical decision-making in tetralogy of Fallot patients.

Background: Arrhythmias, conduction abnormalities, and intracardiac thrombus are common in patients with cardiac amyloidosis (CA). Outcomes of direct-current cardioversion (DCCV) for atrial arrhythmias in patients with CA are unknown., Objectives: This study sought to examine DCCV procedural outcomes in patients with CA., Methods: Patients with CA scheduled for DCCV for atrial arrhythmias from January 2000 through December 2012 were identified and matched 2:1 with control patients by age, sex, type of atrial arrhythmia, and date of DCCV., Results: CA patients (n = 58, mean age 69 ± 9 years, 81% male) were included. CA patients had a significantly higher cardioversion cancellation rate (28% vs. 7%; p < 0.001) compared with control patients, mainly due to intracardiac thrombus identified on transesophageal echocardiogram (13 of 16 [81%] vs. 2 of 8 [25%]; p = 0.02); 4 of 13 of the CA patients (31%) with intracardiac thrombus on transesophageal echocardiogram received adequate anticoagulation ≥3 weeks and another 2 of 13 (15%) had arrhythmia duration <48 h. DCCV success rate (90% vs. 94%; p = 0.4) was not different. Procedural complications were more frequent in CA versus control patients (6 of 42 [14%] vs. 2 of 106 [2%]; p = 0.007); complications in CA included ventricular arrhythmias in 2 and severe bradyarrhythmias requiring pacemaker implantation in 2. The only complication in the control group was self-limited bradyarrhythmias., Conclusions: Patients with CA undergoing DCCV had a significantly high cancellation rate mainly due to a high incidence of intracardiac thrombus even among patients who received adequate anticoagulation. Although the success rate of restoring sinus rhythm was high, tachyarrhythmias and bradyarrhythmias complicating DCCV were significantly more frequent in CA patients compared with control patients., (Copyright © 2019 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

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.

The population of adults with congenital heart disease (ACHD) is increasing constantly due to medical, surgical and interventional successes and the input from advanced cardiovascular imaging. ACHD patients are at continuing risk of residua and sequelae related to their CHD contributing to significant morbidity and mortality. Consequently, lifelong expert surveillance is recommended for most patients. Healthcare providers are still working out how best to achieve this objective, how to train enough experts to provide high quality care, and how to organize the delivery of care. Echocardiography is crucial to clinical surveillance providing a comprehensive assessment of cardiac morphology, physiology, pathophysiology, and function. Thus it contributes significantly to the overall clinical management of ACHD patients. The International Society for Adult Congenital Heart Disease (ISACHD; www.isachd.org) is the leading organization of professionals worldwide dedicated to the pursuit of excellence in the care of ACHD patients. Recognizing the critical role of imaging in the diagnosis and management of ACHD, ISACHD established a task force to provide guidance on echocardiographic studies and reporting. The rationale is that standardization of echocardiographic imaging and reporting carries the potential to improve the overall quality of these exams around the world and facilitate collaborative multicenter research. The standardized ACHD protocols provided by the ISACHD task force (found in the appendices) include specific recommendations for data acquisition and reporting for each of the major adult congenital heart lesions. These protocols give a comprehensive and structured approach in the evaluation of ACHD patients and help to ensure excellent patient care., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Although current guidelines advocate using the CHA 2 DS 2 -VASc score to assess the risk of stroke in patients with atrial fibrillation (AF), compared with transesophageal echocardiography (TEE), its ability to predict left atrial appendage thrombus (LAAT) is limited. We studied 3,324 consecutive patients with sustained AF from our prospective registry of patients who underwent first-time TEE-guided electrical cardioversion (ECV) from May 2000 through March 2012. The association of CHA 2 DS 2 -VASc score or TEE risk factors with the occurrence of LAAT was analyzed. The mean (SD) age was 69 (12.5) years and 67% were men. LAAT was identified in 49 (1.5%) during pre-ECV TEE. Compared with patients without LAAT, those with LAAT had lower peak left atrial appendage emptying velocity (LAAEV) (17.2 ± 8.5 vs 36.6 ± 20.8; p <0.001) and left ventricular ejection fraction (LVEF) (39.9 ± 17.6 vs 51.4 ± 13.7; p <0.001); their CHA 2 DS 2 -VASc score also was higher, but the difference was not statistically significant (3.6 ± 1.4 vs 3.2 ± 1.6; p = 0.06). Multivariate logistic regression analysis identified an LVEF ≤40% (adjusted odds ratio 2.48, 95% confidence interval 1.38 to 4.46), LAAEV 20.3 to 33.9 cm/s (odds ratio 12.19, 95% confidence interval 1.53 to 96.86), and LAAEV ≤20.2 cm/s as independent predictors of LAAT. An LAAEV cut-point of 20 cm/s and an LVEF ≤40% were optimal for detecting LAAT (sensitivity 75% and 62%; specificity 77% and 75%; area under the curve 0.822 and 0.776, respectively). On follow-up, LAAT was an independent risk factor of subsequent ischemic stroke but did not influence survival. In conclusion, reduced LVEF and reduced LAAEV are important pathophysiologic correlates of left atrial appendage thrombogenesis and subsequent ischemic stroke in patients who underwent TEE-guided ECV for AF., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background: This study aimed to determine the prevalence of hypertensive response to exercise (HRE) and its association with cardiovascular adverse events (CAEs) in patients with repaired coarctation of aorta (rCOA)., Methods: We retrospectively reviewed records of adult patients with rCOA who had cardiopulmonary exercise tests (CPETs) and follow-up from 1994 to 2014 at Mayo Clinic. Patients with residual COA, defined as aortic isthmus peak velocity >2.5 m/s, were excluded. HRE was defined as peak systolic blood pressure >200 mm Hg; CAEs were defined as cardiovascular death, stroke, acute coronary syndrome, heart failure hospitalization, and left ventricular ejection fraction (LVEF) < 35%., Results: One hundred thirty-eight patients (82 men [59%]) underwent 213 CPETs, with follow-up of 85 ± 13 months. Age at initial COA repair was 9 ± 3 years; age at initial CPET was 40 ± 13 years. HRE occurred in 26 (19%) patients, and 24 (92%) of the patients with HRE had normal resting blood pressure. There were no differences in age, blood pressure at rest, and CPET findings between patients with HRE and those with normotensive response to exercise. There were 28 CAEs in 24 patients (17%), and HRE was an independent risk factor for CAE (hazard ratio [HR], 1.46 [1.13-2.52]; P = 0.04)., Conclusions: HRE can occur even in the setting of normal blood pressure at rest, and it is a risk factor for CAE. We speculate that patients with HRE represent a high-risk group of patients who, presumably, have occult, advanced vascular dysfunction. CPET can identify these patients. The benefit of intensive antihypertension therapy needs to be confirmed., (Copyright © 2018 Canadian Cardiovascular Society. Published by 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.)

Aims: To examine differences and similarities in bicuspid aortic valve (BAV) and tricuspid aortic valve (TAV) stenosis occurring during the same age and whether any differences impact outcomes following aortic valve replacement (AVR)., Methods and Results: An age-matched cohort of 198 BAV stenosis and 198 TAV stenosis patients was identified from 888 consecutive patients undergoing AVR for severe AS. Mean age 68 ± 6 years; 68% male. Patients with BAV were less likely to have multiple comorbidities, as measured by the Charlson Comorbidity Index (CCI) >2 (3 vs. 10%, P = 0.007). Indexed aortic valve area (0.44 ± 0.09 vs. 0.42 ± 0.08 cm2/m2, P = 0.17) and left ventricular ejection fraction (LVEF) were similar (62 ± 11 vs. 61 ± 12%, P = 0.12), but E/e' ≥15 (46 vs. 66%, P = 0.0002) was less common in BAV stenosis. A tissue aortic valve prosthesis was more commonly utilized in both groups (81 vs. 78%, P = 0.54). Overall indexed effective orifice area was larger in BAV compared with TAV (1.08 ± 0.33 vs. 0.96 ± 0.25 cm2/m2, P = 0.0008). Five-year survival following AVR was lower in TAV compared with BAV stenosis (61 vs. 79%, P = 0.02). Independent predictors of survival following AVR were LVEF < 50% [hazard ratio (HR): 4.8, P = 0.0005], CCI > 2 (HR: 3.1, P = 0.015), effective orifice area index ≤0.85 cm2/m2 (HR: 2.5, P = 0.004), and bioprosthesis (HR: 3.7, P = 0.02)., Conclusion: In an age-matched cohort, TAV compared with BAV stenosis is associated with greater prevalence of cardiovascular risk factors and cardiac impairment and worse survival after AVR., (Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2017. For permissions, please email: journals.permissions@oup.com.)

Anomalous origin of the left coronary artery from the pulmonary artery is rare and typically results in mitral regurgitation, ventricular arrhythmias, heart failure, and sudden death. The condition most often manifests itself in early childhood, but some individuals are diagnosed much later. We describe the case of a 75-year-old woman with heart failure in whom stepwise multimodal imaging revealed anomalous origin of the left coronary artery from the pulmonary artery.

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.)

Aims: von Willebrand factor (VWF) is an independent risk factor for adverse events in patients with non-valvular atrial fibrillation (NVAF). However, it is unclear if VWF level remains elevated and predictive of stroke during entire course of NVAF., Methods and Results: In order to determine if VWF is a time-dependent blood variable, VWF antigen measured by latex immunoassay in 425 NVAF patients and 100 controls with normal sinus rhythm (NSR) was analyzed according to NVAF duration (<1 month: n = 76, 1-12 months: n = 98, and >12 months: n = 251). The mean VWF antigen level in NVAF patients with <1-month duration (167 ± 59%) was not different compared to those with 1-12 months (157 ± 50%, P = .24) and >12 months duration (156 ± 54%, P = .11) but higher compared to NSR controls (143 ± 48%, P = .003). Higher VWF level correlated with higher CHADS 2 scores and with progressing intensity of blood stasis in the left atrium and thrombus formation in all three time periods of atrial fibrillation duration. Patients not treated with warfarin had VWF 30% higher in the first month compared to following months., Conclusions: von Willebrand Factor is steadily elevated throughout the course of dysrhythmia in NVAF patients treated with warfarin and in those with higher intensity of left atrium blood stasis., (© 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Background: Nonpulsatile pulmonary blood flow in Fontan circulation results in pulmonary vascular disease, but the potential relationships between pulmonary vascular resistance index (PVRI) and Fontan failure have not been studied. The objective was to determine whether the absence of subpulmonary ventricle in the Fontan circulation would make patients more vulnerable to even low-level elevations in PVRI, and when coupled with low cardiac index, this would identify patients at increased risk of Fontan failure., Methods and Results: Two hundred sixty-one adult Fontan patients underwent cardiac catheterization; age 26±3 years, men 146 (56%), atriopulmonary Fontan 144 (55%). Patients were divided into 2 groups: those with high PVRI (>2 WU·m 2 ) and low cardiac index <2.5 L min -1 m -2 (group 1, n=70, 30%), and those with normal PVRI and normal cardiac index (group 2, n=182, 70%). Fontan failure was defined by the composite of all-cause mortality, listing for heart transplantation, or initiation of palliative care. There were 68 (26%) cases of Fontan failure during a mean follow-up of 8.6±2.4 years. When compared with group 2, freedom from Fontan failure was significantly lower in group 1: 66% versus 89% at 5 years. The combination of high PVRI and low cardiac index was an independent risk factor for Fontan failure (hazard ratio, 1.84; 95% confidence interval, 1.09-2.85)., Conclusions: When coupled with low cardiac index, even mild elevations in PVRI identify patients at high risk of Fontan failure. This suggests that pulmonary vascular disease is a key mechanism underlying Fontan failure and supports further studies to understand the pathophysiology and target treatments to pulmonary vascular tone in this population., (© 2017 American Heart Association, Inc.)