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Background: There are limited data about the stage D heart failure (advanced HF) in adults with congenital heart disease. Our study objectives were (1) to determine the incidence of new-onset advanced HF in patients and the relationship between advanced HF and all-cause mortality and (2) to determine the relationship between therapies for advanced HF and all-cause mortality. Methods: Retrospective cohort study of adults with congenital heart disease at Mayo Clinic (2003–2019). We defined advanced HF using the European Society of Cardiology diagnostic criteria for advanced HF. Therapies received by the patients with advanced HF were classified into 3 mutually exclusive groups (treatment pathways): (1) conventional cardiac intervention, (2) transplant listing, and (3) palliative care. Results: Of 5309 patients without advanced HF at baseline assessment, 432 (8%) developed advanced HF during follow-up (1.1%/y), and the incidence of advanced HF was higher in patients with severe or complex congenital heart disease. Onset of advanced HF was associated with 6-fold increase in the risk of mortality. Conventional cardiac intervention was associated with significantly higher risk of mortality as compared to transplant listing. The longer the interval from the initial onset of advanced HF to transplant evaluation, the lower the odds of being listed for transplant. Conclusions: Based on these data, we postulate that early identification of patients with advanced HF, and a timely referral for transplant evaluation (instead of conventional cardiac intervention) may offer the best chance of survival for these critically ill patients. Further studies are required to validate this postulation.

Interventions in adults with congenital heart disease (ACHD) focus on surgical and percutaneous interventions in light of rapidly evolving ACHD clinical practice. To bring rigour to our process and amplify the cumulative nature of evidence ACHD care we used the ADAPTE process; we systematically adjudicated, updated, and adapted existing guidelines by Canadian, American, and European cardiac societies from 2010 to 2020. We applied this to interventions related to right and left ventricular outflow obstruction, tetralogy of Fallot, coarctation, aortopathy associated with bicuspid aortic valve, atrioventricular canal defects, Ebstein anomaly, complete and congenitally corrected transposition, and patients with the Fontan operation. In addition to tables indexed to evidence, clinical flow diagrams are included for each lesion to facilitate a practical approach to clinical decision-making. Excluded are recommendations for pacemakers, defibrillators, and arrhythmia-directed interventions covered in separate designated documents. Similarly, where overlap occurs with other guidelines for valvular interventions, reference is made to parallel publications. There is a paucity of high-level quality of evidence in the form of randomized clinical trials to support guidelines in ACHD. We accounted for this in the wording of the strength of recommendations put forth by our national and international experts. As data grow on long-term follow-up, we expect that the evidence driving clinical practice will become increasingly granular. These recommendations are meant to be used to guide dialogue between clinicians, interventional cardiologists, surgeons, and patients making complex decisions relative to ACHD interventions.

Patients with Shone complex (SC) have multiple left-sided obstructive lesions and thus are at risk for left ventricular (LV) remodeling, LV diastolic dysfunction and pulmonary hypertension. Yet, to date, there has been no description of hemodynamics in adults with SC. Retrospective chart review of 25 patients with SC who underwent cardiac catheterization at Mayo Clinic, MN between 2002 and 2019 was performed. SC was defined as multiple left-sided obstructive lesions in the presence of an anatomically abnormal mitral valve. Median age was 32 years (22.5, 42) and 15 patients (60%) were female. The majority of patients (84%) had history of coarctation of the aorta, 10 (40%) had subaortic stenosis, 11 (44%) had prior aortic valve replacement, and 10 (40%) had prior mitral valve replacement. Structural disease at the time of catheterization which warranted intervention within the next year was present in 13 patients (52%). The mean LV end-diastolic pressure was 21.3 ± 9.0 mm Hg (>15 mm Hg in 71%), pulmonary artery peak systolic pressure was 55.4 ± 13.4 mm Hg, and the pulmonary artery mean pressure was 37.0 ± 9.4 mm Hg (>20 mm Hg in 96%). During a mean follow-up of 8.3 ± 4.4 years, there were 7 deaths (28%) and 3 additional patients (12%) underwent cardiac transplantation. In conclusion, adults with SC who underwent catheterization showed significant left-sided heart and pulmonary vascular remodeling. Elevated LV end-diastolic pressure and pulmonary artery pressures were highly prevalent. There were high mortality and cardiac transplant rates in our cohort.

The purpose of this study was to describe procedural outcomes, hemodynamic improvement, regression of left ventricular (LV) mass hypertrophy, and cardiovascular. The primary outcomes were procedural complications, reinterventions, and hemodynamic improvement after coarctation of aorta (COA) repair. The secondary outcomes were improvement in the severity of hypertension, regression of LV mass index, and incidence of cardiovascular events (atrial fibrillation, ventricular tachycardia, heart failure hospitalization, and cardiovascular death) after COA repair. Secondary outcomes were assessed only in patients with isolated COA who had clinical and imaging follow-up at 1 year and 3 years postintervention. Of 172 patients that underwent COA repair (surgical 161; transcatheter 11), there were no procedural deaths, and all patients had residual COA gradient P =0.4 at baseline, 1 year and 3 years). Postintervention hypertension (both stage 1 and 2) were independent risk factors for suboptimal left ventricular mass index regression and cardiovascular events. Persistent hypertension was common after repair of native COA in adults and was associated with suboptimal left ventricular mass index regression and cardiovascular events. These results suggest that optimal blood pressure control with medical therapy after COA repair may result in improved clinical outcomes.

To review our experience with balloon testing prior to atrial septal defect (ASD) closure in adults with left ventricular (LV) diastolic dysfunction.ASD closure in patients with LV diastolic dysfunction may precipitate LV failure. Temporary ASD occlusion has been used in this scenario but data are limited.Retrospective review of 27 patients age ≥ 50 years undergoing temporary ASD balloon occlusion between 2000 and 2018 for suspected LV diastolic dysfunction or elevated LV end-diastolic pressure (LVEDP).Median age was 72 years (IQR 66.7; 75.2). Atrial fibrillation was seen in 58% of patients, hypertension in 58%, and coronary artery disease in 26%; 52% were females. Median ASD size was 13 mm (10; 18) and Qp/Qs 1.8 (1.6; 2.2). Median LVEDP was 14 mmHg (12; 22); pulmonary artery wedge pressure (PAWP) 12 mmHg (9; 16.5) and left atrial pressure (LAP) 13.5 mmHg (8; 16.3). After a median of 5 min (3; 10) of balloon occlusion, patients with baseline LVEDP ≥15 mmHg had more significant increases in LVEDP (9 [6; 12] vs. 2 mmHg [0.5; 5]; p = 0.03) and LAP/PAWP (10.5 [8.3; 16.3] vs. 1.5 mmHg [-1.5; 3]; p = 0.0003) than those with baseline LVEDP15 mmHg. None of those with a baseline LVEDP15 mmHg had a LAP/PAWP15 mmHg during balloon testing compared to 92% of patients with a baseline LVEDP ≥15 mmHg.LVEDP might be used to predict LAP post-ASD closure. Comorbidities typically associated with LV diastolic dysfunction are common in these patients and should be considered in their management.

Transcatheter stent therapy provides similar acute reduction in coarctation of aorta (COA) gradient and systolic blood pressure (SBP) as compared with surgery. However, there are limited data comparing mid-term outcomes after transcatheter vs surgical therapy for COA. The purpose of this study was to compare temporal changes in Doppler COA gradient and SBP after transcatheter stent therapy versus surgical therapy for COA.A retrospective study of COA patients (≥18 years old) undergoing transcatheter stent therapy or surgical therapy at Mayo Clinic in Rochester, Minnesota from 2000-2018 was performed. Linear regression analyses were used to compare temporal changes in Doppler gradient and SBP between the 2 groups. Propensity matching was used to adjust for between-group differences in clinical and anatomic characteristics.A total of 44 and 128 patients underwent transcatheter and surgical therapy, respectively; there were no significant between-group differences in the anatomy of the thoracic aorta. Both groups had similar acute reduction in Doppler peak gradient (P=.66), mean gradient (P=.41), SBP (P=.22), and upper-to-lower extremity SBP (ULE-SBP) gradient (P=.69). The median follow-up was 46 months (interquartile range, 27-81 months) and 63 months (interquartile range, 41-94 months) in the transcatheter and surgical groups, respectively. There were no significant between-group differences in the temporal change in Doppler peak gradient (P-interaction=.06), mean gradient (P-interaction=.15), SBP (P-interaction=.20), and ULE-SBP gradient (P-interaction=.51).These favorable short- and mid-term outcome data support the use of transcatheter therapy as an alternative to surgery in adults with COA. Further studies are required to determine if these favorable outcomes are maintained on long-term follow-up.

Cardio-obstetrics has emerged as an important multidisciplinary field that requires a team approach to the management of cardiovascular disease during pregnancy. Cardiac conditions during pregnancy include hypertensive disorders, hypercholesterolemia, myocardial infarction, cardiomyopathies, arrhythmias, valvular disease, thromboembolic disease, aortic disease, and cerebrovascular diseases. Cardiovascular disease is the primary cause of pregnancy-related mortality in the United States. Advancing maternal age and preexisting comorbid conditions have contributed to the increased rates of maternal mortality. Preconception counseling by the multidisciplinary cardio-obstetrics team is essential for women with preexistent cardiac conditions or history of preeclampsia. Early involvement of the cardio-obstetrics team is critical to prevent maternal morbidity and mortality during the length of the pregnancy and 1 year postpartum. A general understanding of cardiovascular disease during pregnancy should be a core knowledge area for all cardiovascular and primary care clinicians. This scientific statement provides an overview of the diagnosis and management of cardiovascular disease during pregnancy.

Background Fontan-associated liver disease (FALD) is universal in patients with a Fontan circulation. Hepatocellular carcinoma (HCC) is one of its severe expressions, and, though rare, frequently fatal. The purpose of this study was to describe the clinical presentation, risk factors, and outcomes of HCC in patients with a Fontan circulation. Methods A multicenter case series of Fontan patients with a diagnosis of HCC formed the basis of this study. The case series was extended by published cases and case reports. Clinical presentation, tumor characteristics, laboratory and hemodynamic findings as well as treatment types and outcomes, were described. Results Fifty-four Fontan patients (50% female) with a diagnosis of HCC were included. Mean age at HCC diagnosis was 30 ± 9.4 years and mean duration from Fontan surgery to HCC diagnosis was 21.6 ± 7.4 years. Median HCC size at the time of diagnosis was 4 cm with a range of 1 to 22 cm. The tumor was located in the right hepatic lobe in 65% of the patients. Fifty-one percent had liver cirrhosis at the time of HCC diagnosis. Fifty percent of the patients had no symptoms related to HCC and alpha-fetoprotein was normal in 26% of the cases. Twenty-six patients (48%) died during a median follow-up duration of 10.6 (range 1–50) months. Conclusions HCC in Fontan patients occurs at a young age with a 1-year survival rate of only 50%. Meticulous liver surveillance is crucial to detect small tumors in the early stage.

Limited data are available on long-term outcome after repair of partial atrioventricular septal defects (pAVSD) in adults. We sought to review our experience. Between January 1, 1957 and December 31, 2016, 179 adult patients [median age (IQR) 34 (18, 72) years] underwent primary repair of pAVSD. The most common associated defects were secundum atrial septal defect (n = 38) and ventricular septal defect (VSD) (n = 7). Left atrioventricular valve (LAVV) zone of apposition (ZOA) was complete in 47 patients and LAVV regurgitation (≥moderate) was present in 73 patients. Autologous pericardium (n = 79, 45%) and polytetrafluoroethylene felt (n = 56, 32%) were mainly used for pAVSD closure. Repair techniques for LAVV regurgitation included: ZOA suture closure (n = 142), suture annuloplasty (n = 10) and posterior band annuloplasty (n = 9). Six had LAVV replacement. There were 61 deaths over a median follow-up of 21 years (IQR 10, 38), with only 4 early deaths. In a limited subset of patients with 80 paired measurements (n = 40), median right ventricular systolic pressure declined from 43 mm Hg (IQR 35-51) to 33 mm Hg (IQR 30-44) postoperatively (P0.001), and this improvement was sustained over long-term follow-up (P = 0.513). A total of 34 patients underwent a reoperation (recurrent LAVV regurgitation, n = 26; left ventricular outflow-tract obstruction, n = 7; LAVV stenosis, n = 4; patch dehiscence, n = 1) with cumulative incidence of 6% and 16% at 10 and 15 years, respectively. Repair of pAVSD in adults can be done safely with low early mortality and good long-term outcomes. Postrepair reduction of pulmonary artery pressure is significant. Despite the low re-operation rates, long-term surveillance remains essential.

Placement of an epicardial pacemaker system is often preferred over an endocardial system in patients who have undergone a Fontan operation, but data are limited on how these two systems perform over time in patients with Fontan palliation. We performed a retrospective review of adults with Fontan palliation who had pacemaker implantation and interrogation data at Mayo Clinic from 1994 to 2014. Lead parameters, pacing mode, and polarity were collected at the earliest device interrogation report. Clinic notes and device interrogation reports were reviewed at implantation, 6 months, and yearly after implantation to determine impedance, capture threshold (CT), and energy threshold (ET). There were 87 patients with 168 leads in the study cohort. The mean follow-up time was 7.7 years (6 months-19 years). There were 143 epicardial leads (57 atrial and 86 ventricular) and 25 endocardial leads (20 atrial and 5 ventricular). There was no difference in the baseline lead parameters between epicardial and endocardial leads for impedance (610 ± 259 versus 583 ± 156 Ω, p = 0.93), CT (2.0 ± 1.3 versus 1.8 ± 1.3 V, p = 0.28), or ET (7.1 ± 12.5 versus 6.8 ± 18.1 µJ, p = 0.29). Compared to endocardial leads, ventricular epicardial leads were associated with temporal decrease in impedance and increase in ET. Regarding clinical outcomes, epicardial leads had higher rates of failure but similar generator longevity in comparison to endocardial leads. Ventricular epicardial leads were associated with temporal decrease in impedance and increase in ET. Epicardial leads had a higher rate of failure but similar generator longevity compared to endocardial leads.

It is unknown whether systemic right ventricular (SRV) dysfunction confers increased risk for sudden death in congenitally corrected transposition of the great arteries (ccTGA). We sought to define risk of ventricular arrhythmias and sudden death attributable to systemic right ventricular dysfunction in ccTGA.The study cohort is comprised of adult patients with a diagnosis of ccTGA. Clinical information and clinical outcomes including ICD therapy, incidence of VT/VF, and cause of death were reviewed.129 patients with ccTGA were followed for 7.2 ± 3.4 years. Mean SRV ejection fraction (SRVEF) was 39% (n = 56 with an EF35%). Significant documented arrhythmia (sustained VT requiring defibrillation or cardiac arrest due to VT/VF) occurred in 13/56 patients (23%) with SRV EF35% compared with 2/73 (3%) with SRV EF35% (p 0.001). There was no significant difference in age at death, death from documented arrhythmia, or death from all cause between patients with SRV EF35% and35%. However, risk of sudden death was higher in patients with SRV EF35% (16% vs 1%, p = 0.002). The combined clinical endpoint of ICD therapy, clinically significant VT/VF, and sudden death was significantly higher in patients with SRV EF35% than in patients with SRV EF35%, independent of the presence of LV EF35% (p 0.001).Dysfunction of the systemic RV in ccTGA increases the risk of sudden death and clinically significant ventricular tachyarrhythmias. Further study is needed to determine if ventricular tachyarrhythmias comprise the primary cause of sudden death in these patients and if ICDs offer any significant mortality benefit.

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 (V o 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 V o 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 V o 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 V o 2 was 22.9 ± 4.1 mL/kg/min (63 ± 11 percent predicted), SVI was 43 ± 15 mL/m2, and CI was 2.9 ± 0.9 L/min/m2. Peak V o 2 correlated with SVI (r = 0.30, P o 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 V o 2 and chronotropic index improved. Conclusions Overall, there was a temporal decline in peak V o 2 that correlated with decline in Doppler SVI. In the patients who had Fontan conversion operation, there was a temporal improvement in peak V o 2 that correlated with improvement in chronotropic index.

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.

The optimal management strategy for atrial tachyarrhythmia in the Fontan population is unknown.Retrospective review of 264 adult Fontan patients with atrial tachyarrhythmia evaluating 3 clinically adopted scenarios: antiarrhythmic drug (AAD) therapy, catheter ablation (CA), and Fontan conversion (FC). These patients were followed up at Mayo Clinic from 1994 to 2014. The study objective was to compare freedom from atrial tachyarrhythmia recurrence (AR) and occurrence of composite adverse events (stroke, heart failure hospitalization, death, or heart transplant) between treatment groups.The age of atrial tachyarrhythmia onset was 25 ± 4 years, time from Fontan operation was 13 ± 6 years, follow-up was 74 ± 18 months, atriopulmonary Fontan was 215 (81%), and atrial flutter/intra-atrial reentry tachycardia was 173 (65%). In those managed with AAD (n = 110), freedom from AR was 7% at 60 months. Catheter ablation (n = 31) was associated with an acute procedural success of 94%, and freedom from AR was 41% at 60 months. Fontan conversion (n = 33) resulted in a perioperative mortality of 3%, and freedom from AR was 51% at 60 months. Fontan conversion and CA were similar with regard to AR (P = .14) and significantly better compared with AAD (P.0001). Adverse events were found to occur more frequently in the patients with AR (P.0001) and the patients treated with AAD only (P.0001).Catheter ablation and FC operations are associated with less recurrence of atrial tachyarrhythmia compared with AAD. Atrial tachyarrhythmias are more likely to recur in patients with a longer history of the arrhythmia and are associated with more adverse events. Early referral to a specialty center for these interventions should be considered.

The fate of the left ventricle (LV) after aortic valve replacement (AVR) in mixed aortic valve disease (MAVD) is unknown.Patients with moderate-severe MAVD, ejection fraction ≥50%, and no coronary artery disease who underwent AVR were identified. Moderate-severe MAVD was defined as a combination of ≥moderate aortic stenosis and ≥moderate aortic regurgitation. Assessment for LVD was performed at 1 and 5years after AVR. The purpose of the study was to determine prevalence and predictors of early and late left ventricular dysfunction (LVD) defined as ejection fraction50% at 1 and 5years post-AVR. The severity of LV hypertrophy was assessed using LV mass index (LVMI), while relative wall thickness (RWT) was used to determine the type of hypertrophy. RWT was calculated as (2×posterior wall thickness)/LV end-diastolic dimension (LVEDD). A RWT score ≥0.42 and0.42 indicates concentric and eccentric hypertrophy respectively.Patients with MAVD (n=179); age 63±8years, males 134 (75%); underwent AVR at Mayo Clinic, 1994-2010. Early LVD occurred in 38(21%). Predictors of early LVD were LVMI/LVEDD3.1 (HR 1.83, CI 1.59-1.98); RWT0.46 (HR 2.16, CI 1.21-4.99); and older age (HR 1.62, CI 1.23-3.02). Assessment of LV function was performed in 124 patients at 5-years post-AVR, and late LVD was present in 29(23%). Predictors of late LVD were LVMI/LVEDD3.1 (HR 1.77, CI 1.24-2.01) and RWT0.46 (HR 1.65, CI 1.29-2.24). All-cause mortality occurred in 21(12%), and was more common in patients with LVMI/LVEDD3.1 (P=0.043) and RWT0.46 (P=0.029). Patients with postoperative LVD showed less regression of LV mass after AVR even after controlling for blood pressure.LVD can occur after AVR even in the setting of normal preoperative LV function and absence of coronary artery disease. Preoperative LV mass was predictive of LVD and should be taken into consideration when determining the timing of AVR.

Ambulatory blood pressure monitoring (ABPM) is more sensitive than resting blood pressure (BP) assessment in detecting hypertension in patients with coarctation of aorta (COA). However, it is not known whether ABPM provides a better measure of left ventricular (LV) pressure load and LV remodeling

Coarctation of aorta (COA) results in chronic left ventricular (LV) pressure overload and LV hypertrophy, and the goal of COA intervention is to relieve LV pressure overload ([1][1]). The guidelines recommend the use of several COA severity indices as indications for intervention ([2][2],[3][3]).

Objectives There is a paucity of data about mixed aortic valve disease (MAVD) in patients with bicuspid/unicuspid aortic valve (BAV). This study sought to describe the outcomes of patients with moderate/severe MAVD. Methods We queried our database for patients with BAV and moderate/severe MAVD seen between 1994 and 2013. We excluded patients with baseline New York Heart Association (NYHA) III/IV symptoms, left ventricular ejection fraction 50 mm, and significant disease of other valves. The purpose of the study was to determine the freedom from NYHA III/IV symptoms and aortic valve replacement (AVR). Results We identified 138 patients with moderate/severe MAVD; mean age was 51 ± 12 years; 112 (81%) were males; and follow-up was 8.5 ± 4 years. Ninety-two patients (67%) underwent AVR within 3.7 ± 2.5 years. Mechanical prostheses were implanted in 73 patients (79%); 22 patients (26%) and 36 patients (39%) had concomitant coronary artery bypass graft and aorta replacement during AVR respectively. There were no surgical deaths. Freedom from AVR was 84%, 51%, and 20% at 1, 5 and 10 years respectively. Predictors of AVR were age at presentation (hazard ratio [HR] 5.22; confidence interval [CI] 3.10 to 6.64) for every decade increase in age; and having severe stenosis or regurgitation at the time of presentation (HR 1.32; CI 1.05 to 3.16). Conclusions Age and disease severity should be incorporated in the risk assessment of BAV patients with MAVD, and patients with both risk factors should be monitored closely.

Background A direct comparison of outcomes between moderate mixed aortic valve disease (MAVD) and isolated aortic stenosis (AS) or aortic regurgitation (AR) has not been performed, making evidence-based recommendations difficult in patients with MAVD. Objectives This study sought to determine adverse event (AE) occurrence (the primary endpoint), defined as New York Heart Association functional class III/IV symptoms, aortic valve replacement, or cardiac death, and to compare AE rates between MAVD and isolated AS or AR. Methods Asymptomatic patients were identified with moderate MAVD and an ejection fraction ≥50% and were followed at Mayo Clinic from 1994 to 2013. Moderate MAVD was defined as a combination of moderate AS and moderate AR. Age- and sex-matched control groups were selected with isolated moderate AR (n = 117), moderate AS (n = 117), or severe AS (n = 117). Results At 9.1 ± 4.2 years of follow-up, patients with moderate MAVD (n = 251) had a mean age of 63 ± 11 years, 73% were male, and 38% had bicuspid valve. AE occurred in 193 (77%) patients in this group, including symptom development (69%), aortic valve replacement (67%), and cardiac death (4%). Predictors of AE were older age (hazard ratio [HR]: 1.71 per decade; 95% confidence interval [CI]: 1.38 to 1.97 per decade; p = 0.001), and relative wall thickness >0.42 (HR: 2.01; 95% CI: 1.86 to 2.33; p = 0.002). AE rates were similar in the MAVD and severe AS group (71% vs. 68% at 5 years; p = 0.49), but were significantly higher compared with the moderate AS and AR groups. Conclusions MAVD patients had outcomes comparable to those with severe AS, and preserved ejection fraction and should be monitored closely for symptoms.

Aims The aim of this article is to determine freedom from adverse events (AE) defined as symptoms, aortic valve replacement (AVR), or death in the mixed aortic valve disease (MAVD) population. Methods and results We reviewed patients with moderate/severe MAVD followed at Mayo Clinic from 1994–2013. Only asymptomatic patients with normal ejection fraction and trileaflet aortic valve were included. Cox proportional-hazard models and Kaplan–Meier method were used. We identified 213 patients with moderate/severe MAVD; mean age was 69 years (±11) and 67% were males. An AE endpoint was reached in 172 patients, and 69% of these AEs occurred in moderate MAVD (peak velocity of 3–3.9 m/s). Mean follow-up was 10.1 ± 3 years, and mean time to AE was 2.9 ± 2.1 years. A mechanical prosthesis was implanted in 71/151 (47%), and 54/151 (36%) had concomitant coronary artery bypass grafting and/or aorta replacement during AVR. Early surgical mortality was 0.7%. Freedom from AE endpoint was 42% [confidence interval (CI) 39–45%] and 30% (CI 27–33%) at 3 and 5 years, respectively. Predictors of AE were peak aortic velocity [hazard ratio (HR) 2.73; CI 1.97–2.84, P < 0.0001] for every 1 m/s difference and having severe stenosis or severe regurgitation at presentation (HR 2.58; CI 2.01–4.44, P = 0.001). Conclusion Moderate MAVD had high rate of AEs comparable to severe isolated aortic stenosis and should be followed as such. Patients with severe MAVD should be evaluated at least every 6 months because half of them will become symptomatic and require AVR within 1 year.

PURPOSE OF REVIEW Adults with congenital heart disease now outnumber children with these syndromes in developed countries. This has seen a surge in the care required for these patients, and the development of an entirely new realm of cardiology. Arrhythmia is one of the most common causes of morbidity and mortality in this group, and this review highlights current approaches to recognition and management. RECENT FINDINGS Atrial arrhythmias are especially common in this group of patients, while pacemaker or implantable cardioverter defibrillator implantation and cardiac ablation are also frequently necessary. The presentation and management of these entities present salient differences for the clinician--for both acute and chronic care--and more recently a national societal consensus statement has attempted to encapsulate the best approach. Without any level of evidence A, all recommendations are based on data derived from nonrandomized studies or only expert/consensus opinion. This review is aimed at providing current opinion on optimum clinical care in this arena in lieu of this publication and the more novel corroborative clinical studies. SUMMARY Recognition and appropriate management of arrhythmia in adults with congenital heart disease frequently differ from those patients with a normal heart or acquired heart disease. Early diagnosis and proper treatment are essential in this complex patient category.

Congenitally corrected transposition (ccTGA) is a rare form of congenital heart disease characterized by atrioventricular and ventriculoarterial discordance. Patients with ccTGA usually have associated congenital cardiovascular conditions; less than 1% have no associated lesions. Generally, ccTGA is identified during infancy or childhood with features of heart failure or cyanosis when there are associated lesions such as ventricular septal defect and/or pulmonic stenosis. Presentation later in life generally occurs when there are either mild or no associated lesions. Presentation during adulthood may be prompted by symptoms or signs of cardiovascular disease or due to abnormal findings on cardiac testing. Management of patients with ccTGA depends on presentation, symptoms, and associated defects. In this review, we will focus on the management of adult patients with ccTGA.

Objective Patients with unoperated single ventricle (SV) rarely survive into adulthood with good functional status and may develop Eisenmenger9s syndrome (ES). We report outcomes of a 30-year cohort of such patients. Methods Adult patients with unoperated SV were identified by searching the Mayo Clinic medical record from 1984 to 2014. Clinical data were collected and compared between patients with pulmonary stenosis (PS) and ES. Results 24 patients were identified (median peak-age 56 (31–77) years (11 ES, 13 PS); 22 had left ventricular morphology. 50-year transplant-free survival was 65% (95% CI 43 to 81). Median age at death was 55 years (31–77 years); 15 deaths (62%) occurred before oral pulmonary vasodilators were commercially available. Two-thirds of the cohort demonstrated preserved New York Heart Association functional class and median EF was 60% (49% to 62%). The majority of patients to survive into the fifth decade exhibited anatomy of double-inlet LV (DILV) with PS. Conclusion Selected patients with unoperated SV with PS and ES can survive with good functional class up to the eighth decade with good medical management. DILV/PS appears to be the ideal phenotype for advanced survival. Our outcomes may be considered when such patients with SV having ‘balanced’ physiology are evaluated for Fontan palliation. However, additional prospective study will be necessary to verify this assertion.

This is the first scientific statement from the American Heart Association on maternal resuscitation. This document will provide readers with up-to-date and comprehensive information, guidelines, and recommendations for all aspects of maternal resuscitation. Maternal resuscitation is an acute event that involves many subspecialties and allied health providers; this document will be relevant to all healthcare providers who are involved in resuscitation and specifically maternal resuscitation.

The number of adults with congenital heart disease (CHD) has increased markedly over the past few decades as a result of astounding successes in pediatric cardiac care. Nevertheless, it is now well understood that CHD is not cured but palliated, such that life-long expert care is required to optimize outcomes. All countries in the world that experience improved survival in CHD must face new challenges inherent to the emergence of a growing and aging CHD population with changing needs and medical and psychosocial issues. Founded in 1992, the International Society for Adult Congenital Heart Disease (ISACHD) is the leading global organization of professionals dedicated to pursuing excellence in the care of adults with CHD worldwide. Recognizing the unique and varied issues involved in caring for adults with CHD, ISACHD established a task force to assess the current status of care for adults with CHD across the globe, highlight major challenges and priorities, and provide future direction. The writing committee consisted of experts from North America, South America, Europe, South Asia, East Asia, and Oceania. The committee was divided into subgroups to review key aspects of adult CHD (ACHD) care. Regional representatives were tasked with investigating and reporting on relevant local issues as accurately as possible, within the constraints of available data. The resulting ISACHD position statement addresses changing patterns of worldwide epidemiology, models of care and organization of care, education and training, and the global research landscape in ACHD.

Objective To evaluate the utility of magnetic resonance elastography (MRE) in screening patients for hepatic fibrosis, cirrhosis, and hepatocellular carcinoma after the Fontan operation. Patients and Methods Hepatic MRE was performed in conjunction with cardiac magnetic resonance imaging in patients who had undergone a Fontan operation between 2010 and 2014. Liver stiffness was calculated using previously reported techniques. Comparisons to available clinical, laboratory, imaging, and histopathologic data were made. Results Overall, 50 patients at a median age of 25 years (range, 21-33 years) who had undergone a Fontan operation were evaluated. The median interval between Fontan operation and MRE was 22 years (range, 16-26 years). The mean liver stiffness values were increased: 5.5±1.4 kPa relative to normal participants. Liver stiffness directly correlated with liver biopsy-derived total fibrosis score, time since operation, mean Fontan pressure, γ-glutamyltransferase level, Model for End-Stage Liver Disease score, creatinine level, and pulmonary vascular resistance index. Liver stiffness was inversely correlated with cardiac index. All 3 participants with hepatic nodules exhibiting decreased contrast uptake on delayed postcontrast imaging and increased nodule stiffness had biopsy-proven hepatocellular carcinoma. Conclusion The association between hepatic stiffness and fibrosis scores, Model for End-Stage Liver Disease scores, and γ-glutamyltransferase level suggests that MRE may be useful in detecting (and possibly quantifying) hepatic cirrhosis in patients after the Fontan operation. The correlation between stiffness and post-Fontan time interval, mean Fontan pressure, pulmonary vascular resistance index, and reduced cardiac index suggests a role for long-term hepatic congestion in creating these hepatic abnormalities. Magnetic resonance elastography was useful in detecting abnormal nodules ultimately diagnosed as hepatocellular carcinoma. The relationship between stiffness with advanced fibrosis and hepatocellular carcinoma provides a strong argument for additional study and broader application of MRE in these patients.

The management of women with mechanical heart valves during pregnancy remains difficult and controversial. There is no ideal anticoagulation regimen for this unique population, as there are inherent risks and benefits of each approach for both mother and fetus. There has been limited data available to guide the clinician in the optimal treatment strategy for an individual patient. The AHA/ACC Guidelines for the Management of Patients with Valvular Heart Disease created class recommendations for this population based upon current evidence, which is summarized in this review.

There are limited data about outcomes after aortic valve replacement (AVR) in patients with mixed aortic valve disease (MAVD) [(1)][1]. We conducted a retrospective review of all adults with moderate-to-severe MAVD who underwent AVR at Mayo Clinic from January 1, 1994 to December 31, 2014 to

Cone reconstruction for tricuspid valve repair has revolutionized the surgical treatment of Ebstein anomaly. We present the case of a 58-year-old woman with atypical Ebstein anomaly and right ventricular apical hypoplasia who was spared from palliative shunt physiology by our use of cone reconstruction. Compared with other techniques, cone reconstruction of the tricuspid valve more closely replicates normal valvular anatomy and function. This surgical procedure can be applied to many anatomic variations of Ebstein anomaly, as in our patient's apparently unique instance of atypical Ebstein anomaly with right ventricular apical hypoplasia.

The relationship between temporal progression of magnetic resonance elastography derived liver stiffness (MRE-LS) and progression of Fontan associated liver disease (FALD) is unknown. To assess this relationship, we hypothesized that progression of MRE-LS correlated with progression FALD severity and clinical outcomes.Retrospective review of Fontan patients who had1 liver MRE, 2010-2016. Annual change in MRE-LS was the quotient of the difference between baseline and subsequent MRE-LS, and the interval between scans.22 patients were enrolled; median age 29(19-38) years, 14 (64%) males and 10 (46%) with atriopulmonary Fontan. Baseline and subsequent MRE-LS values were 5.4 ± 1.1 kPa and 5.8 ± 0.9 kPa" for clarity, interval between scans was 25 ± 5 months, and annual change in MRE-LS was 0.3 ± 0.2 kPa. Temporal change in MRE-LS correlated with temporal changes in model for end-stage liver disease (MELD) score (r = 0.84, p 0.001) and model for end-stage liver disease excluding international normalized ratio (MELD-XI) score (r = 0.75, p = 0.001). The study cohort was divided into 2 groups using the mean annual change in MRE-LS as the cut point. Groups A and B comprised of patients with annual increase in MRE-LS ≥0.3 kPa (n = 6) and0.3 kPa (n = 16) respectively. Composite adverse event endpoint (death, heart-liver transplant listing, palliative care, hospitalization, paracentesis) was more common in Group A (4 of 6, 67%) compared to Group B (3 of 16, 19%), p = 0.13 although this did not reach statistical significance due to small sample size.Progression of MRE-LS correlated with clinical deterioration as measured by worsening liver disease severity scores and the occurrence of adverse events.

Aims Determine the role of diastolic function indices in pre-operative and post-operative risk stratification in patients with moderate mixed aortic valve disease (MAVD). Methods and results A retrospective study was conducted of asymptomatic patients with moderate MAVD (a combination of moderate aortic stenosis and moderate aortic regurgitation) and an ejection fraction of 50% or more who were followed up at Mayo Clinic from 1 January 2004, to 31 December 2013. A pre-requisite for inclusion in the study was assessment of diastolic function involving at least three of the following indices: tissue Doppler early diastolic velocity (e'), mitral inflow early velocity (E), tricuspid regurgitation velocity, and left atrial volume index. Primary endpoints were aortic valve replacement (AVR) or cardiac death while secondary endpoints were cardiovascular adverse events (CAEs) after AVR. We defined CAEs as stroke, heart failure hospitalization, severe left ventricular dysfunction, and cardiac death. There were 214 patients (age 61 ± 8 years, men 146 [68%]) followed for 6.1 ± 2.3 years during which 162 (76%) AVRs and 11 (5%) cardiac deaths occurred. The multivariable risk factors for cardiac death or AVR were relative wall thickness (RWT) > 0.42 [hazard ratio (HR), 1.88 [95% CI, 1.28-2.59]; P = 0.001] and average E/e' >14 (HR, 1.94 [95% CI, 1.29-3.01]; P = 0.02). Freedom from CAE after AVR was significantly lower in the patients with baseline RWT >0.42 or mean E/e' >14 than the other patients: 79% (95% CI 74-83%) vs. 94% (95% CI 89-98%) at 3 years (P = 0.03). Conclusion The presence of RWT >0.42 or E/e' >14 identifies a high-risk patient subset whose risk for cardiovascular morbidities persists even after AVR.

Today, most female children born with congenital heart disease will reach childbearing age. For many women with complex congenital heart disease, carrying a pregnancy carries a moderate to high risk for both the mother and her fetus. Many such women, however, do not have access to adult congenital heart disease tertiary centers with experienced reproductive programs. Therefore, it is important that all practitioners who will be managing these women have current information not only on preconception counseling and diagnostic evaluation to determine maternal and fetal risk but also on how to manage them once they are pregnant and when to refer them to a regional center with expertise in pregnancy management.

Society Guidelines PACES/HRS Expert Consensus Statement on the Recognition and Management of Arrhythmias in Adult Congenital Heart Disease Developed in Partnership Between the Pediatric and Congenital Electrophysiology Society (PACES) and the Heart Rhythm Society (HRS). Endorsed by the Governing Bodies of PACES, HRS, the American College of Cardiology (ACC), the American Heart Association (AHA), the European Heart Rhythm Association (EHRA), the Canadian Heart Rhythm Society (CHRS), and the International Society for Adult Congenital Heart Disease (ISACHD) Paul Khairy, MD, PhD, FRCPC (Chair),* George F. Van Hare, MD, FACC, FHRS (Co-Chair),y Seshadri Balaji, MBBS, PhD,y Charles I. Berul, MD, FHRS,y Frank Cecchin, MD, FACC,z Mitchell I. Cohen, MD, FACC, FHRS,y Curt J. Daniels, MD, FACC,** Barbara J. Deal, MD, FACC,y Joseph A. Dearani, MD, FACC,* Natasja de Groot, MD, PhD,{ Anne M. Dubin, MD, FHRS,y Louise Harris, MBChB, FHRS, Jan Janousek, MD, PhD,{ Ronald J. Kanter, MD, FHRS,y Peter P. Karpawich, MD, FACC, FAHA, FHRS,y James C. Perry, MD, FACC, FHRS,* Stephen P. Seslar, MD, PhD,y Maully J. Shah, MBBS, FHRS,y Michael J. Silka, MD, FACC, FAHA,x John K. Triedman, MD, FACC, FHRS,y Edward P. Walsh, MD, FACC, FHRS,y and Carole A. Warnes, MD, FRCP, FACC, FAHA** Pediatric and Congenital Electrophysiology Society (PACES) representative; yHeart Rhythm Society (HRS) representative; zAmerican College of Cardiology (ACC) representative; xAmerican Heart Association (AHA) representative; {European Heart Rhythm Association (EHRA) representative; Canadian Heart Rhythm Society (CHRS) representative; International Society for Adult Congenital Heart Disease (ISACHD) representative

Recognition and Management of Arrhythmias in Adult Congenital Heart Disease: Executive Summary Developed in partnership between the Pediatric and Congenital Electrophysiology Society (PACES) and the Heart Rhythm Society (HRS). Endorsed by the governing bodies of PACES, HRS, the American College of Cardiology (ACC), the American Heart Association (AHA), the European Heart Rhythm Association (EHRA), the Canadian Heart Rhythm Society (CHRS), and the International Society for Adult Congenital Heart Disease (ISACHD)

BackgroundPatients with protein-losing enteropathy (PLE) following the Fontan operation have a reported 50% mortality at 5 years after diagnosis.ObjectivesThe aim of this study was to review outcomes in patients with PLE following the Fontan operation.MethodsFrom 1992 to 2010, 42 patients (55% male) with PLE following the Fontan operation were identified from clinical databases at the Mayo Clinic. Data were collected retrospectively.ResultsMean age at PLE diagnosis was 18.9 ± 11.0 years. Initial Fontan operation was performed at 10.1 ± 10.8 years of age. Mean time from Fontan operation to PLE diagnosis was 8.4 ± 14.2 years. Survival was 88% at 5 years. Decreased survival was seen in patients with high Fontan pressure (mean >15 mm Hg; p = 0.04), decreased ventricular function (ejection fraction 2 at diagnosis (p = 0.04). Patients who died had higher pulmonary vascular resistance (3.8 ± 1.6 Wood units [WU] vs. 2.1 ± 1.1 WU; p = 0.017), lower cardiac index (1.6 ± 0.4 l/min/m2 vs. 2.7 ± 0.7 l/min/m2; p < 0.0001), and lower mixed venous saturation (53% vs. 66%; p = 0.01), compared with survivors. Factors were assessed at the time of PLE diagnosis. Treatments used more frequently in survivors with PLE included spironolactone (21 [68%]), octreotide (7 [21%]), sildenafil (6 [19%]), fenestration creation (15 [48%]), and relief of Fontan obstruction (7 [23%]).ConclusionsPLE remains difficult to treat; however, in the current era, survival has improved with advances in treatment. Further study is needed to better understand the mechanism of disease and ideal treatment strategy.

Cyanotic heart disease with central right-to-left shunt predisposes patients to paradoxical embolus, which can lead to development of brain abscess. Early detection is key to reduction of morbidity and mortality. A 39-year-old Ethiopian male without prior medical history presented with five days

Eric S. Williams, MD, MACC, Chair Jonathan L. Halperin, MD, FACC, Co-Chair James A. Arrighi, MD, FACC Eric H. Awtry, MD, FACC Eric R. Bates, MD, FACC Salvatore Costa, MD, FACC Lori Daniels, MD, FACC Akshay Desai, MD, FACC Douglas E. Drachman, MD, FACC Susan Fernandes, LPD, PA-C Rosario

Objectives The objective of our study was to review the long-term outcomes of patients undergoing surgical repair of aortic coarctation. Background Surgical repair of aortic coarctation has been performed at the Mayo Clinic, Rochester, Minnesota, for over 60 years. Methods Between 1946 and 2005, 819 patients with isolated coarctation of the aorta underwent primary operative repair. Medical records were reviewed and questionnaires mailed to the patients. Results Mean age at repair was 17.2 ± 13.6 years. The majority (83%) had pre-operative hypertension. Operations included simple and extended end-to-end anastomosis (n = 632), patch angioplasty (n = 72), interposition grafting (n = 49), bypass grafting (n = 30), and subclavian flap or “other” (n = 35). Overall early mortality ( 20 yrs) and pre-operative hypertension were associated with decreased survival (p Conclusions Primary repair of isolated coarctation of the aorta was performed with a low rate of mortality. However, long-term survival was reduced compared with that in an age- and sex-matched population, and many patients required further reoperation. These findings emphasize that patients with aortic coarctation need early recognition and intervention, as well as lifelong informed follow-up.

Limited data exist regarding the outcomes of non-cardiac procedures (NCPs) in adult patients after Fontan operations (Fontan patients).To compare procedural outcomes after NCPs in Fontan patients with outcomes for two matched control groups: patients with repaired congenital heart disease and biventricular circulation (CHD-BiV) and patients with no heart disease (NHD). We defined cyanosis as oxygen saturation90% and procedural hypoxia as saturation80% or a decrease in saturation10% from baseline.There were 538 NCPs in 154 Fontan patients (mean age, 30 years) performed in 1990-2015. Sedation and anaesthesia types were monitored anaesthesia care (256,48%), general anaesthesia (51,9%), minimal sedation (105,20%), local anaesthesia (75,14%) and regional anaesthesia (51,9%). Ninety-three complications occurred in 79 procedures (15%) and included arrhythmia requiring intervention (9), hypotension (14), bradycardia (8), hypoxia (38), heart failure requiring intravenous diuretics (2), acute kidney injury (3), bleeding requiring blood transfusion (1), unplanned procedures for dialysis catheter placement (2), readmission (2), unplanned hospitalisation for hypoxia (8) and unplanned transfer to intensive care unit (1). Baseline cyanosis was the only multivariable risk factor for complications (HR, 1.87 (95% CI 1.14 to 3.67), p=0.04). Procedural complications were more common in the Fontan group (18%) than in the CHD-BiV (5%) and NHD groups (1.4%) (p=0.001).Complications after NCPs were more common in Fontan patients, and baseline cyanosis was a risk factor for complications. All-cause mortality was low and may be related to the multidisciplinary care approach used for Fontan patients at our centre.

The growing population of adults with congenital heart disease (CHD) poses challenges for cardiac surgeons, general cardiologists and CHD sub specialists. The patients themselves, often believing themselves to be 'totally corrected' following operative repair, also face challenges with re-operations and lifelong cardiac problems. This review examines the challenges of the past, present and future for both medical providers and patients.

There is a paucity of data about mixed aortic valve disease (MAVD) in patients with bicuspid/unicuspid aortic valve (BAV). This study sought to describe the outcomes of patients with moderate/severe MAVD.We queried our database for patients with BAV and moderate/severe MAVD seen between 1994 and 2013. We excluded patients with baseline New York Heart Association (NYHA) III/IV symptoms, left ventricular ejection fraction50%, aortic dimension50 mm, and significant disease of other valves. The purpose of the study was to determine the freedom from NYHA III/IV symptoms and aortic valve replacement (AVR).We identified 138 patients with moderate/severe MAVD; mean age was 51 ± 12 years; 112 (81%) were males; and follow-up was 8.5 ± 4 years. Ninety-two patients (67%) underwent AVR within 3.7 ± 2.5 years. Mechanical prostheses were implanted in 73 patients (79%); 22 patients (26%) and 36 patients (39%) had concomitant coronary artery bypass graft and aorta replacement during AVR respectively. There were no surgical deaths. Freedom from AVR was 84%, 51%, and 20% at 1, 5 and 10 years respectively. Predictors of AVR were age at presentation (hazard ratio [HR] 5.22; confidence interval [CI] 3.10 to 6.64) for every decade increase in age; and having severe stenosis or regurgitation at the time of presentation (HR 1.32; CI 1.05 to 3.16).Age and disease severity should be incorporated in the risk assessment of BAV patients with MAVD, and patients with both risk factors should be monitored closely.