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Background Ablation of atrial tachyarrhythmia in adults with congenital heart disease (ACHD) is challenging because of complex anatomy and high scar burden. We proposed that the addition of high-density mapping with the PentaRay® (Biosense Webster, Inc) mapping catheter (EAM+P) to 3-dimensional electroanatomic mapping (EAM) allows for rapid acquisition of high-resolution maps and shorter procedure times. Methods In this single-center, retrospective cohort study of patients with ACHD who underwent atrial arrhythmia ablation, patients were divided those who underwent ablation with EAM and those who underwent ablation with EAM+P. Results Fifteen ablations were performed in 13 patients using standard EAM, and 11 ablations were performed in 10 patients using EAM+P. There was no difference in mean age or complexity of congenital heart disease. The procedure duration was 1.5 times longer in the EAM than in the EAM+P group (P = .015). The dose area product was 12 times higher in the EAM than in the EAM+P group (P = .001). A higher number of venous access sites were used for EAM cases than for EAM+P cases (P = .008). Acute success rates of ablation and recurrence rates at 1 year were similar in the 2 groups. There were no procedure-related complications in either group. Conclusion This is the first study to evaluate the use of the PentaRay® high-density mapping catheter for ablation of atrial tachyarrhythmia in patients with ACHD. The use of the PentaRay® high-density mapping catheter results in shorter procedure time, decreased radiation exposure, and fewer venous access sites.

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.

Levo-transposition of the great arteries is a congenital heart disease characterized by atrioventricular and ventricular-arterial discordance. Aortic valve disease in levo-transposition of the great arteries patients is uncommon. We present a patient with levo-transposition of the great arteries and severe aortic stenosis who successfully underwent transcatheter aortic valve replacement and the diagnostic and procedural challenges involved. (Level of Difficulty: Intermediate.), Graphical abstract, Levo-transposition of the great arteries is a congenital heart disease characterized by atrioventricular and ventricular-arterial discordance. Aortic…

Background Rhythm disorders are the leading cause of morbidity and mortality in adults with congenital heart disease (ACHD). Infrequent or asymptomatic arrhythmias may not be detected by routine monitoring. Implantable loop recorders (ILRs), such as the Reveal LINQTM , have been useful in long-term monitoring for arrhythmias in adults with cryptogenic stroke. Objective We propose the Reveal LINQTM will detect arrhythmias, not documented by other monitoring modalities, resulting in change in management in ACHD patients. Methods This is a single center retrospective review of Reveal LINQTM use in ACHD patients from 2014-2017. Medical records were reviewed to determine cardiac diagnosis, indication for implant, ILR findings, and changes in management. Results Twenty-two patients, median age 25 years, underwent ILR implantation. ILR findings resulted in change in management in nine (41%) patients. One-third (3/9) of the patients with clinically relevant events were asymptomatic. Patients with Fontan palliation had the highest number of pertinent positive events (57%). ACHD physiologic class D patients were more likely to have a positive finding (P = .034) compared to other physiologic classes. Majority (75%) of patients with positive events had arrhythmias documented on ILR which were not demonstrated on prior Holter/event monitors. Pertinent negative event occurred in one patient with Fontan palliation (5%) who had syncope corresponding to sinus rhythm. Conclusion ILRs are a useful adjunct for arrhythmia monitoring in the ACHD population with clinically relevant events in 41% of patients. A special consideration for ILRs could be made for high-risk asymptomatic patients.

[Extract] The 2018 ACHD guideline is a full revision of the “2008 ACC/AHA Guidelines for the Management of Adults with Congenital Heart Disease” (S1.4-1), which was the first U.S. guideline to be published on the topic. This revision uses the 2008 ACHD guideline as a framework and incorporates new data and growing ACHD expertise to develop recommendations. Congenital heart disease (CHD) encompasses a range of structural cardiac abnormalities present before birth attributable to abnormal fetal cardiac development but does not include inherited disorders that may have cardiac manifestations such as Marfan syndrome or hypertrophic cardiomyopathy. Also not included are anatomic variants such as patent foramen ovale. Valvular heart disease (VHD) may be congenital, so management overlaps with the “2014 AHA/ACC Guidelines for the Management of Patients With Valvular Heart Disease” (S1.4-2), particularly for bicuspid aortic valve (BAV) disease. Where overlap exists, this document focuses on the diagnosis and treatment of congenital valve disease when it differs from acquired valve disease, whether because of anatomic differences, presence of concomitant lesions, or differences to consider given the relatively young age of patients with ACHD. This guideline is not intended to apply to children (

With the recent emergence of SARS-CoV-2 and COVID-19, healthcare facilities and personnel are expected to rapidly triage and care for patients with even the most complex medical conditions. Adults with congenital heart disease (ACHD) represent an often-intimidating group of complex cardiovascular disorders. Given that general internists and general cardiologists will often be asked to evaluate this group during the pandemic, we propose here an abbreviated triage algorithm that will assist in identifying the patient's overarching ACHD phenotype and baseline cardiac status. The strategy outlined allows for rapid triage and groups various anatomic CHD variants into overarching phenotypes, permitting care teams to quickly review key points in the management of moderate to severely complex ACHD patients., Highlights • ACHD patients may be at increased risk of poor outcomes with COVID-19. • Rapid triage of the ACHD patient is needed during the COVID-19 pandemic. • We propose a simplified algorithm for triage and care of COVID19+ ACHD patients.

Introduction: After the initial surgical repair of tetralogy of Fallot (rTOF), right ventricular outflow tract dysfunction is common, with pulmonary stenosis (PS), regurgitation, or both. Obese adults with rTOF have worse biventricular systolic function, and greater post-operative pulmonary valve replacement morbidity than non-obese patients. Transcatheter PVR (TPVR) is used increasingly, though no studies have examined the impact of body mass index (BMI) on morbidity and hemodynamics in adults with rTOF and subsequent TPVR. Hypothesis: BMI affects outcomes of transcatheter PVR in adults with TOF. Methods: This was a 10 yr, single center, retrospective review of adults (>18 yo) with rTOF who underwent TPVR. The cohort was split into 2 groups based on BMI at time of TPVR. Group A (Grp A): normal and overweight (BMI Results: There were 81 adults, 42 (52%) normal, 18 (22%) overweight (Grp A) and 21 (26%) obese (Grp B). Mean follow up was 6.4 + 3.1 yrs. With most recent post-TPVR echocardiogram, there was no significant difference in LV or RV size and function across groups. Compared to Grp A, Grp B patients were more likely to develop any degree of PS (mild, moderate, or severe) following TPVR (69% vs 94%, respectively, p = 0.032). An ROC curve analysis demonstrated BMI ≥ 26.3 had a low sensitivity (45%), but good specificity (89%) for predicting PS post TPVR. There was no difference between groups requiring re- intervention for PVR. Conclusion: This is the first study to show greater BMI affects valve function in adults with rTOF following TPVR. BMI >26 was associated with a greater risk of PS in follow up echocardiograms after TPVR. Obesity is an epidemic in the US and patient prosthesis mismatch may be an issue when considering TPVR. Further studies are indicated to determine the long-term effects of BMI on TPVR and the need for re-intervention in adults with TOF.

Introduction: Athletic cardiac adaptation is associated with structural changes that can overlap with disease states, unnecessarily limiting sports participation. Cardiovascular magnetic resonance (CMR) is useful in athlete’s heart and provides myocardial tissue characterization by T1 and T2 mapping. Hypothesis: CMR in competitive athletes will show abnormal T1 and T2 mapping due to intense exercise induced myocardial edema that can overlap with myocarditis. Methods: CMR data including left ventricular ejection fraction (LVEF) and T1/T2 maps were collected using standardized protocols on a 1.5 T scanner and compared between competitive athletes (N = 18, 83% male, median age 20 years), clinical myocarditis (N = 42, 71% male, median age 23 years) and controls (N = 35, 86% male, median age 22 years) between 2016-2020. T2 values of Results: Figure 1 (panel A) shows participating sport and indications for CMR in athletes. There were 11 athletes (61%) with elevated T2 values (>59 ms), of which 9 (82%) were without clinical myocarditis. Average T2, native T1, ECV, and LVEF are shown in panels B-E. T2 values were highest in myocarditis, followed by athletes and controls (p = 0.001). ECV was higher in myocarditis compared to athletes (p = 0.002). LGE was present in 8/18 athletes and 41/42 myocarditis patients. 6 athletes had follow-up CMR after a period of deconditioning, with 3 (50%) demonstrating an improvement in T2 values and LGE. Conclusions: To conclude, we demonstrate abnormalities on T2 mapping in athletes consistent with myocardial edema or inflammation. Changes in T2 may be related to intense training. Additional studies are required to prospectively evaluate athletes for normative T1 and T2 mapping values, relationship to training, and their correlation with LGE.

Introduction: Pregnancy is contraindicated in women with pulmonary hypertension (PH), yet many still decide to pursue pregnancy. Hypothesis: We hypothesized improved maternal mortality with PH at our center’s cardio-obstetrics program and sought to identify factors to estimate the risk of major adverse cardiac events (MACE). Methods: Pregnant women with right ventricular systolic pressure (RVSP) ≥35 mmHg or tricuspid regurgitant velocity > 2.8 m/s on transthoracic echocardiogram (TTE) were identified. Women with intermediate to high probability PH by ESC criteria (TTE or catheterization, n = 70) were classified using the 6 th World Society of PH definitions. Results: In 70 women with PH (30 ± 6 years-old, RVSP 52 ± 16 mmHg) there were 12 (17%) with WHO Group 1 PH, 45 (64%) with Group 2 PH, 4 (6%) with Group 3 PH and 9 (13%) with Group 5 PH (Figure A). Baseline characteristics were similar except: Group 1 PH had 83% on prostacyclin (PC) therapy, higher RVSP (78 ± 20 mmHg vs. Groups 2 (46 ± 9), 3 (44 ± 2 mmHg) and 5 PH (48 ± 10mmHg), p50 mmHg (95% CI 1.3-10, p = 0.02) and LVEF Conclusion: To conclude, in a large single center cohort we report 100% 1-year survival in Groups 1, 3, and 5 PH, with most Group 1 PH patients on PC therapy and under cardio-obstetrics care. We identify Group 2 PH as an under-recognized group for adverse outcomes in pregnancy, with NYHA FC, pre-eclampsia, RVSP >50 mmHg and LVEF

Introduction: A dilated main pulmonary artery (MPA) seen in congenital heart disease (CHD) or pulmonary arterial hypertension (PAH) is a rare cause of angina due to left main coronary artery (LMCA) compression. Case: A 49 year old male with no prior cardiac history presented with substernal chest pain. He reported exercise intolerance for several years. On exam he had an oxygen saturation of 85%, loud P2, fixed split second heart sound and ejection click. Electrocardiogram showed right ventricular (RV) hypertrophy and no significant ST changes. Laboratory data revealed a negative troponin. An enlarged main and right pulmonary artery (RPA) (Figure 1A) was seen on computed tomography (CT) chest angiography for pulmonary embolism (CTPE) and on echocardiography he had a severely dilated RV with decreased function (Figure 1B,1C). Cardiac catheterization showed a step up in saturations from 56 to 71%, Qp:Qs of 1.5 mean MPA pressure of 53 mmHg, pulmonary capillary wedge pressure of 11mmHg and pulmonary vascular resistance (PVR) of 7WU. A slit-like narrowing of LMCA ostium was seen on coronary angiography and intravascular ultrasound (Figure 1D). He was diagnosed with a sinus venosus defect, anomalous right upper pulmonary venous drainage and suspicion for extrinsic compression of LMCA by the dilated RPA that was confirmed on cardiac CT (Figure 1E-I). He was started on triple therapy for PAH with a significant reduction in PA pressures (mean 36 mmHg and PVR 2.1 WU). Percutaneous stenting of the LMCA was deferred and he underwent successful sinus venosus defect repair with a 5mm fenestrated patch, baffling of the anomalous pulmonary veins and a 32 mm interposition graft in the MPA with reduction plasty of MPA/RPA. Conclusion: This is an unusual presentation of unrepaired CHD with angina due to a compressed LMCA from a dilated pulmonary artery. Treatment of the underlying PAH as well as repair of the defect including reduction plasty of the MPA is a consideration for symptomatic relief.

Introduction: COVID19 pandemic has resulted in drastic changes in healthcare delivery with increase in use of telemedicine. There is limited data on patient experience with telemedicine in adults with congenital heart disease (ACHD). Hypothesis: We hypothesized that ACHD patients would prefer in-person clinic visits over telemedicine, and this would be especially true for patients > 65 years who may be at a higher risk of experiencing technical limitations. Methods: We conducted a nurse administered telephone survey (Figure 1A) based on Agency for Healthcare Research and Quality recommendations to assess patient experience after ACHD telemedicine visits from March 2020 to June 2020. Results: Of 216 patients who had ACHD telemedicine visits, 136 (63%) agreed to participate in the survey. Mean age was 45 + 18 years, majority (65%) being video encounters. Most (98%) said that the telemedicine visit was successful in addressing their healthcare needs. Only 21 (15%) patients reported technical issues. Most patients (76%) were likely to choose telemedicine in the future instead of an in-person clinic visit provided testing was done separately. Given a choice between in-person clinic visit and telemedicine: 103 (76%) preferred telemedicine, 25 (18%) preferred in-person clinic visits, and 8 (6%) had no preference (Figure1B). Of the 25 patients over 65 years, 19 (76%) would choose telemedicine over the in-person clinic, and only one patient reported technical difficulties. Conclusion: In conclusion, patients reported a positive experience with ACHD telemedicine, which was true even among elderly patients. Technical limitations were infrequent even amongst the elderly. Majority of patients felt that their healthcare needs were addressed and would consider telemedicine over clinic visits if given a choice. This data is vital as payors move away from supporting telemedicine and hospitals restructure, with and without ongoing COVID 19 concerns.

Introduction: Pulmonary hypertension (PH) diagnosed by elevated right ventricular systolic pressure (RVSP) is present in 6-11% of patients with sickle cell disease (SCD) and is associated with increased mortality. PH, independent of SCD, is associated with increased maternal mortality. While there are reports of adverse outcomes during pregnancy in patients with SCD, pregnancy outcomes of patients with SCD and PH have not been well studied. Methods: Using our institutional electronic data warehouse we searched for pregnant women who had an echocardiogram between November 1, 2011 and April 30, 2018, and met criteria for pulmonary hypertension with report of either RVSP > 35 mmHg or tricuspid regurgitant (TR) velocity of more than 2.8 m/s. We assessed maternal and fetal outcomes in this group of patients. Results: We identified 136 women with elevated RVSP, of which 7 (5%) had SCD (mean maternal age 29 ± 3 years, mean RVSP 47 ± 11 mmHg, mean TR velocity 3.1 ± 0.4 m/s). RVSP was known to be elevated before pregnancy in 5 (71%). There were no maternal deaths during pregnancy or up to 1-year post-partum. Thromboembolic complications occurred in 3 (43%); including 1 deep vein thrombosis with presumed pulmonary embolism (PE) at 20 weeks gestational age (GA), 1 catheter associated right atrial thrombus at 22 weeks GA, and 1 PE at 16 weeks GA. There were no heart failure complications. Cesarean section occurred in 5 (71%), all for obstetric indications. There were no fetal or neonatal deaths. Pre-eclampsia occurred in 2 (28%). The average gestational age was 35 ± 4 weeks. Preterm birth and low birth weight occurred in 3 (43%) patients. Conclusion: In conclusion, there was no maternal mortality or heart failure in pregnant women with SCD and elevated RVSP at our high-volume center, however 3 of 7 patients (43%) had thromboembolic complications. Further studies of women with PH and SCD are needed to assess the thromboembolic risk during pregnancy.

Pulmonary arterial hypertension (PAH) and novel coronavirus (SARS-CoV-2) disease COVID-19 are characterized by extensive endothelial dysfunction and inflammation leading to vascular remodeling and severe microthrombi and microvascular obliterative disease. It is hypothesized that those patients with underlying lung disease, like PAH, represent a high-risk cohort in this pandemic. However, reports of COVID-19 in this cohort of patient have been scaring and an observational survey showed that the disease was relatively well tolerated. We postulate that specific PAH vasodilator may offer some protection and/or advantage in the case of concomitant COVID-19. Here we review the literature describing mechanisms of action for each of the broad categories of PAH therapy, and offer potential hypothesis about why this therapy may impact outcomes in COVID-19.

This study investigates the use of cardiac magnetic resonance imaging in competitive athletes who recovered from coronavirus disease 2019 (COVID-10) to detect myocardial inflammation that would identify high-risk athletes for return to competitive play.

Importance Myocarditis is a leading cause of sudden death in competitive athletes. Myocardial inflammation is known to occur with SARS-CoV-2. Different screening approaches for detection of myocarditis have been reported. The Big Ten Conference requires comprehensive cardiac testing including cardiac magnetic resonance (CMR) imaging for all athletes with COVID-19, allowing comparison of screening approaches. Objective To determine the prevalence of myocarditis in athletes with COVID-19 and compare screening strategies for safe return to play. Design, Setting, and Participants Big Ten COVID-19 Cardiac Registry principal investigators were surveyed for aggregate observational data from March 1, 2020, through December 15, 2020, on athletes with COVID-19. For athletes with myocarditis, presence of cardiac symptoms and details of cardiac testing were recorded. Myocarditis was categorized as clinical or subclinical based on the presence of cardiac symptoms and CMR findings. Subclinical myocarditis classified as probable or possible myocarditis based on other testing abnormalities. Myocarditis prevalence across universities was determined. The utility of different screening strategies was evaluated. Exposures SARS-CoV-2 by polymerase chain reaction testing. Main Outcome and Measure Myocarditis via cardiovascular diagnostic testing. Results Representing 13 universities, cardiovascular testing was performed in 1597 athletes (964 men [60.4%]). Thirty-seven (including 27 men) were diagnosed with COVID-19 myocarditis (overall 2.3%; range per program, 0%-7.6%); 9 had clinical myocarditis and 28 had subclinical myocarditis. If cardiac testing was based on cardiac symptoms alone, only 5 athletes would have been detected (detected prevalence, 0.31%). Cardiac magnetic resonance imaging for all athletes yielded a 7.4-fold increase in detection of myocarditis (clinical and subclinical). Follow-up CMR imaging performed in 27 (73.0%) demonstrated resolution of T2 elevation in all (100%) and late gadolinium enhancement in 11 (40.7%). Conclusions and Relevance In this cohort study of 1597 US competitive athletes with CMR screening after COVID-19 infection, 37 athletes (2.3%) were diagnosed with clinical and subclinical myocarditis. Variability was observed in prevalence across universities, and testing protocols were closely tied to the detection of myocarditis. Variable ascertainment and unknown implications of CMR findings underscore the need for standardized timing and interpretation of cardiac testing. These unique CMR imaging data provide a more complete understanding of the prevalence of clinical and subclinical myocarditis in college athletes after COVID-19 infection. The role of CMR in routine screening for athletes safe return to play should be explored further.

Over the past decade, as the majority of patients with single ventricle anatomy who have undergone the Fontan operation reach adulthood, a newly recognized disease process, Fontan-associate...

Adults who underwent complex atrial baffling as children via Mustard or Senning procedures are at heightened risk for atrial arrhythmias. Antiarrhythmic therapies are typically ineffective in this population. Accordingly, our team of pediatric and adult electrophysiologists investigated the effectiveness of early invasive transbaffle-access techniques to perform early radiofrequency ablation at the source of these clinically significant arrhythmias. For this retrospective study, we selected 11 adult survivors of atrial baffling (mean age, 34 ± 9 yr) who underwent clinically indicated electrophysiologic study after no more than one trial of antiarrhythmic therapy. Using transbaffle-access techniques and 3-dimensional mapping of the venous atria, we found 12 inducible arrhythmias in 10 patients: intra-atrial reentrant tachycardia (n=6), atrioventricular nodal reentrant tachycardia (n=3), focal atrial tachycardia (n=2), and repetitive double firing of the atrioventricular node (n=1). Defining success as short- and midterm freedom from arrhythmia, we analyzed outcomes of radiofrequency ablation at 1 and 6 months. At 1 month, ablation was 100% successful. At 6 months, after 11 ablations in 9 patients, 5 patients had no clinical recurrence, 2 had improved arrhythmia control from minimal medical therapy, and 2 were to undergo repeat study for recurrent tachycardia. In the recurrence-free patients, arrhythmias during electrophysiology study matched the types found clinically before the study. To our knowledge, this is the largest one-year cohort of adult survivors of atrial baffling to have undergone study by a combined pediatric–adult electrophysiology team. We conclude that early invasive transbaffle access for ablating diverse atrial tachyarrhythmias was effective in these patients.

Congenital heart disease (CHD) is the most common birth defect, affecting about 0.8% of live births. Advances in recent decades have allowed >85% of children with CHD to survive to adulthood, creating a growing population of adults with CHD. Little information exists regarding survival, demographics, late outcomes, and comorbidities in this emerging group, and multiple barriers impede research in adult CHD. The National Heart, Lung, and Blood Institute and the Adult Congenital Heart Association convened a multidisciplinary working group to identify high-impact research questions in adult CHD. This report summarizes the meeting discussions in the broad areas of CHD-related heart failure, vascular disease, and multisystem complications. High-priority subtopics identified included heart failure in tetralogy of Fallot, mechanical circulatory support/transplantation, sudden cardiac death, vascular outcomes in coarctation of the aorta, late outcomes in single-ventricle disease, cognitive and psychiatric issues, and pregnancy.

Partial anomalous pulmonary venous return (PAPVR) is a rare congenital anomaly that results in a left-to-right shunt. Based on the shunt fraction, PAPVR has a wide spectrum of presentations. If a significant left-to-right shunt is left unrepaired, pulmonary vascular remodeling can occur resulting in the development of pulmonary arterial hypertension (PAH). Furthermore, if the condition is associated with an atrial septal defect (ASD), the patient can develop shunt reversal and Eisenmenger's syndrome in setting of severe PAH. Management plans include close observation, surgical repair, and treatment with pulmonary artery vasodilator therapies. Here, we present multiple cases of PAPVR to highlight the wide spectrum of presentations and the individualized treatment for each case. Keywords: PAPVR, PAH, CHD, ASD

Objective The current study aims to identify the rates of lapses in care and loss to follow-up before age one through age five for white and nonwhite congenital heart disease (CHD) survivors. Nonwhite CHD survivors were hypothesized to experience an earlier lapse in care and be lost to follow-up than whites. Design Patients were from a large pediatric hospital and had (1) at least one outpatient cardiology clinic visit or cardiac surgery visit before the age of one and (2) a diagnosis of moderate or complex structural CHD. Cardiology outpatient utilization rates were tracked from before age one through age five. Lapse in follow-up was defined as not having at least one outpatient cardiology visit per year, and loss to follow-up was not returning after a lapse in care by age five. Race was categorized as white and nonwhite. Covariates included sex, insurance type, noncardiology inpatient and outpatient hospital utilization, and CHD severity. Results The sample included 1034 patients. Overall, 75.7% experienced a lapse in care with only 41.6% of those returning by age five. Nonwhites experienced lapses in care at younger ages than whites. Nonwhites had a 53% increased risk of lapse in care. Medicaid patients and those with moderate CHD diagnoses also had an increased risk for lapse in care. Conclusions Lapse in care appears prevalent among CHD survivors by age five, with nonwhites demonstrating elevated risk. Future multisite prospective studies should include the assessment of parental knowledge, barriers to accessing care, and satisfaction with care.

Atrial switch operations for D-Transposition of the great arteries (D-TGA) were performed until the late 20th century. These patients have substantial rates of re-operation, particularly for baffle related complications. This study sought to analyze the efficacy of percutaneous transcatheter intervention (PTI) for baffle leak and/or stenosis in adult atrial switch patients. Adult patients with a prior atrial switch operation who underwent heart catheterization (2002-2014) at a tertiary adult congenital heart disease referral center were retrospectively analyzed. In 58 adults (30 ± 8 years, 75% men, 14% New York Heart Association (NYHA) functional class ≥2) who underwent 79 catheterizations, PTI was attempted in 50 (baffle leak (n = 10, 20%), stenosis (n = 27, 54%), or both (n = 13, 26%)). PTI was successful in 45 and 5 were referred for surgery due to complex anatomy. A total of 40 bare metal stents, 18 covered stents, 16 occlusion devices, 2 angioplasties, and 1 endovascular graft were deployed. In isolated stenosis, there was improvement in NYHA functional class after PTI (8 vs. 0 patients were NYHA FC > 2, p = 0.004), which was matched by improvement in maximal oxygen consumption on exercise testing (VO2) (25.1 ± 5.4 mL/kg/min vs. 27.9 ± 9 mL/kg/min, p = 0.03). There were no procedure-related deaths or emergent surgeries in this cohort. This single-center cohort is the largest reported series of adult atrial switch operation patients who have undergone PTI for baffle stenosis and/or leak. We demonstrate that PTI with an expert multi-disciplinary team is a safe and effective alternative to surgery in adult patients with an atrial switch operation.

Background Identify demographic and medical status indicators that account for variability in physical and emotional health-related quality of life (QoL) among young adults with congenital heart disease (CHD) as compared to traditional lesion severity categories. Methods Cross-sectional study of 218 young adult survivors of CHD (mean=25.7, SD=7.1years). Participants were recruited from pediatric and adult CHD clinics at a pediatric and an adult hospital. Stepwise linear regression examined the unique contribution of demographic (age; sex; estimated income) and medical status indicators (comorbid conditions; treatment modality; ventricular function/functional capacity) on QoL compared to traditional lesion severity categories (simple; moderate; complex). Results Lesion severity category accounted for a small portion of the variance in physical QoL (3%), but was not associated with emotional QoL. Lesion severity did not significantly contribute to the variability in physical QoL once other variables were entered. Having an estimated income of ≤$30,000, taking more than one cardiac-related medication, and having a New York Heart Association (NYHA) functional class designation>I was associated with poorer physical QoL and explained 23% of the variability. NYHA class was the only variable that explained a unique proportion of variance (7%) in emotional QoL, and having a NYHA class designation>I was associated with greater risk for poorer emotional functioning. Conclusions Findings suggested that several indicators readily available to treatment teams may provide important information about the risk for poor patient-reported outcomes of physical and emotional QoL among CHD survivors.

Background In order to determine the feasibility of tracking quality of care in adults with congenital heart disease (ACHD), we aimed to estimate the availability of relevant data in electronic medical records (EMR) used in North American ACHD centers. Methods Previously proposed quality indicators (QIs) were reviewed to consider what types of data would be required for each. ACHD program directors were surveyed about the nature of electronic data in existing EMRs. From the survey, the availability of data types needed for the denominator and numerator of each QI were estimated, and an overall estimate of data availability was calculated for each QI. These estimates were adjusted by the sensitivity of identifying the patients through administrative codes. Analysis was repeated for scenarios in which various data type estimates were hypothetically dropped by half to determine the overall impact of each data type. Results A total of 64 ACHD program directors responded to the survey. Of 55 QIs, average estimated data availability was 67%. QIs for tetralogy of Fallot had the highest estimated data availability (mean 88%), whereas those for atrial septal defect were lowest (mean 23%), reflecting both the need for interpretation of imaging studies and the lower reliability of billing codes for identification of ACHD patients. QIs with highest estimates were based largely on administrative data, which had the biggest impact on overall estimates. QIs needing interpretation of imaging findings had the lowest estimates, as well as certain overuse measures. Conclusions For a wide range of ACHD programs, data for proposed QIs based on administrative data are most likely to be obtainable through EMR. Data related to imaging interpretation or overuse measures are least likely. Our findings can inform future efforts to establish registry efforts or data reporting tools to track these indicators.

Objective Survivors of congenital heart disease (CHD) are at risk for life-threatening complications as they age. This study aimed to examine the association of knowledge of future health risks, perceived risk, and health behaviors among adolescents and adults with CHD. Methods CHD survivors ( N = 200, ages 15–39; 23% simple, 44% moderate, 33% complex lesions) completed measures of risk knowledge accuracy and perceived risk for developing complications, and reported physical activity and saturated fat intake. Results CHD survivors reported poor risk knowledge and consuming high-fat diets. Adolescents reported more physical activity than young adults. Greater risk knowledge was associated with lower fat intake, and participants who exercised more expected fewer future complications, and this difference remained statistically significant when accounting for education and age. Conclusions CHD survivors, regardless of age, have poor risk knowledge and diets. Survivors may benefit from emphasis on future health risks and health behaviors from both pediatric and adult providers.

Over the past decade, as the majority of patients with single ventricle anatomy who have undergone the Fontan operation reach adulthood, a newly recognized disease process, Fontan-associated liver disease (FALD), has emerged. FALD is an extracardiac complication that may lead to substantial comorbid disease and premature mortality. The risk factors, pathophysiology, longitudinal consequences, and therapeutic options related to FALD remain poorly defined. Although we recognize that Fontan circulatory properties are associated with extracardiac organ dysfunction, numerous gaps in our understanding of the nature of this relationship exist. Such extracardiac manifestations, in addition to other late complications of the circulation, can significantly affect quality of life and healthcare use. Therefore, to initiate a formal evaluation of FALD, the American College of Cardiology (ACC) sponsored a stakeholders meeting on October 1 to 2, 2015, in Washington, DC. The goal of the meeting was to bring together subspecialty experts in the fields of adult and pediatric hepatology, congenital cardiology (adult congenital and pediatric cardiology), heart failure/transplant, epidemiology, and cardiothoracic surgery, as well as patient advocates, patients, parents of children and young adults who have had the Fontan procedure, and research organizations and societies to discuss the current state of FALD. Topics included gaps in knowledge, optimal care, research opportunities and barriers, and sound practices to guide providers, patients, and families. This report summarizes findings from the stakeholders meeting and seeks to establish a platform for understanding and addressing FALD.

Pediatric cardiovascular services are responding to the dynamic changes in the medical environment, including the business of medicine. The opportunity to advance our pediatric cardiology field through collaboration is now realized, permitting us to define meaningful quality metrics and establish national benchmarks through multicenter efforts. In March 2016, the American College of Cardiology hosted the first Adult Congenital/Pediatric Cardiology Section Congenital Heart Community Day. This was an open participation meeting for clinicians, administrators, patients/parents to propose metrics that optimize patient care and outcomes for a state-of-the-art congenital heart center of the 21st century. Care center collaboration helps overcome the barrier of relative small volumes at any given program. Patients and families have become active collaborative partners with care centers in the definition of acute and longitudinal outcomes and our quality metrics. Understanding programmatic metrics that create an environment to provide outstanding congenital heart care will allow centers to improve their structure, processes and ultimately outcomes, leading to an increasing number of centers that provide excellent care. This manuscript provides background, as well listing of proposed specialty domain quality metrics for centers, and thus serves as an updated baseline for the ongoing dynamic process of optimizing care and realizing patient value.

The impact of pulmonary hypertension (PH) on survival in cystic fibrosis (CF) remains unclear.To determine the influence of PH on survival in the CF population.The United Network for Organ Sharing database was queried from 1987 to 2013 to identify first-time lung transplant candidates who were tracked from wait list entry date until death or censoring to determine influence of PH. Using right heart catheterization measurements, mild PH was defined as mean pulmonary artery pressure greater than or equal to 25 mm Hg and severe greater than or equal to 35 mm Hg.Of 2,781 CF patients, 2,100 were used for univariate analysis, 764 for Kaplan-Meier survival function, 687 for multivariate Cox models, and 576 and 132 for matching on the propensity of mild PH and severe PH, respectively. Univariate Cox analysis found significant differences in survival for mild PH (hazard ratio [HR], 1.747; 95% confidence interval [CI], 1.387-2.201; P0.001) and severe PH (HR, 2.299; 95% CI, 1.639-3.225; P0.001). Further assessment by multivariate Cox models identified significant risk for death associated with mild PH (HR, 1.757; 95% CI, 1.367-2.258; P0.001) and severe PH (HR, 2.284; 95% CI, 1.596-3.268; P0.001). Cox regression stratified on matched pairs of PH cases and control subjects confirmed the risk for death for mild PH (HR, 1.919; 95% CI, 1.290-2.85; P = 0.001) and severe PH (HR, 4.167; 95% CI, 1.709-10.157; P = 0.002).The manifestation of PH is associated with significantly increased risk for death in CF patients with advanced lung disease.

We report a case of platypnea-orthodeoxia syndrome resulting from multiple inter-atrial communications with right-toleft shunting in a 73 year old woman. Our patient presented with progressive shortness of breath over the last eighteen months. She was on 10 liters of high flow oxygen at time of presentation to our hospital and was bed bound owing to severe dyspnea. Her history of chronic pulmonary emboli despite therapeutic coumadin levels and ulcerative colitis initially led to an exhaustive hepatopulmonary work-up. On clinical exam, she was noted to have normal oxygen saturation with lying down, but with sitting up or walking she developed significant hypoxia and respiratory distress. With no evidence of lung parenchymal or vascular diseases, right heart catheterization was performed and revealed normal right sided pressures. Intracardiac echocardiography confirmed a typical appearing patent foramen ovale (PFO) with atrial septal aneurysm and bidirectional shunting, and a small inferior secundum atrial septal defect (ASD). Although the ASD was congenital, aortic dilation with ageing could have altered the location of the atrial septum which facilitated laminar blood flow from the inferior vena cava to the left atrium. We proceeded to close both defects with an Amplatzer Cribiform Occluder. Her symptoms dramatically improved 48 hours following closure and she was ambulating on room air at time of discharge. One month follow-up was notable for no recurrence of symptoms. PFOs and small ASDs are generally innocuous but could cause right to left shunting even in the absence of elevated right sided pressures. Excluding other causes of platypnea-orthodeoxia, and closing the atrial communications can lead to resolution of symptoms. Key words: Multiple Atrial Communications, Platypnea-Orthodeoxia Syndrome.

In recent years, athletic participation has more than doubled in all major demographic groups, while simultaneously, children and adults with established heart disease desire participation in sports and exercise. Despite conferring favorable long-term effects on well-being and survival, exercise can be associated with risk of adverse events in the short term. Complex individual cardiovascular (CV) demands and adaptations imposed by exercise present distinct challenges to the cardiologist asked to evaluate athletes. Here, we describe the evolution of sports and exercise cardiology as a unique discipline within the continuum of CV specialties, provide the rationale for tailoring of CV care to athletes and exercising individuals, define the role of the CV specialist within the athlete care team, and lay the foundation for the development of Sports and Exercise Cardiology in the United States. In 2011, the American College of Cardiology launched the Section of Sports and Exercise Cardiology. Membership has grown from 150 to over 4,000 members in just 2 short years, indicating marked interest from the CV community to advance the integration of sports and exercise cardiology into mainstream CV care. Although the current athlete CV care model has distinct limitations, here, we have outlined a new paradigm of care for the American athlete and exercising individual. By practicing and promoting this new paradigm, we believe we will enhance the CV care of athletes of all ages, and serve the greater athletic community and our nation as a whole, by allowing safest participation in sports and physical activity for all individuals who seek this lifestyle.

Objective This study aimed to quantitatively compare findings of emotional functioning across studies of adolescents and adults with congenital heart disease (CHD) through meta-analysis.

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

Summary Background Although pulmonary arterial hypertension (PAH) is a potential co-morbidity in cystic fibrosis (CF), right heart catheterization (RHC) is not commonly performed in this patient population until referral for lung transplantation. Material and methods An non-randomized observational pilot study was performed after an exercise protocol with an upright stationary bicycle was added to RHC performed in patients with CF undergoing evaluation for lung transplantation (LT). Results Twelve consecutive patients with advanced lung disease due to CF referred for LT completed RHC with exercise protocol. Transthoracic echocardiography (TTE) prior to the RHC did not identify evidence of PAH or significant structural abnormalities; right and left ventricular systolic function were normal. non-randomized RHC in this same cohort found 75% (9/12) had PAH with an elevation of the mean pulmonary artery pressure (PAP) at rest with a mean (±SD) of 27.8 ± 4.9 mmHg that significantly increased during exercise to 47.2 ± 5.4 mmHg, p = 0.0025. For the last 6 patients, pulmonary vascular resistance was calculated during exercise, with a significant increase from 3.15 ± 0.3 to 12.8 ± 0.6 Wood Units (p = 0.0313) comparing measurements at rest to exercise. Conclusion RHC at rest and during exercise was safely and effectively performed in patients with CF referred for LT. Furthermore, central hemodynamic measurements found significant worsening of PAH during exercise in a small cohort of CF patients with advanced lung disease.

BACKGROUND Disease-related stressors for survivors of congenital heart disease (CHD) have been qualitatively described but not quantified nor examined in relationship to important patient-reported outcomes (PROs). OBJECTIVE The aims of this study are to (1) identify the types and degree of disease-related stress experienced by CHD survivors based on age, functional status, and sex, (2) examine differences in stress and PROs by age, functional status, and sex, and (3) determine the unique contribution of perceived stress to variability in PROs. METHODS A cross-sectional study of 173 adolescents and emerging and young adults who were recruited from both pediatric and adult CHD clinics was conducted. Participants rated the degree to which they found various aspects of CHD stressful and completed PROs of health-related quality of life and emotional distress. Differences in perceptions of stress across predictors were determined using analyses of variance and χ analyses. The relative contribution of perceived stress predicting PROs was examined using stepwise linear regression. RESULTS Two items emerged as being stressful for almost half of the sample, including concerns about future health and having scars or other signs of medical procedures. Adolescents reported less perceived stress than emerging or young adults, and survivors with even mild functional limitations reported higher perceived stress than did those without any symptoms. Perceptions of stress significantly contributed to variability in PROs above and beyond other predictors and was the only variable to explain unique variance in emotional distress. CONCLUSIONS Having even mild functional impairment may have significant deleterious consequences on PROs via increased perceptions of stress. Stress may be modifiable using cognitive behavioral therapy.

Aims There is a fivefold increase in the frequency of intracranial aneurysm (IA) in adults with coarctation of the aorta (CoA). Current guidelines for management of adults with CoA recommend computed tomography angiography (CTA) or magnetic resonance imaging of the intracranial vessels. However, this recommendation has not been universally accepted. The purpose of our study was to prospectively perform CTA of the intracranial vessels in adults with CoA to evaluate the prevalence and identify high-risk features of this complication. Methods and results From January 2008 to February 2011, adults ≥18 years of age with CoA were prospectively enrolled in a screening program with CTA of the intracranial vessels. Analyses of prognostic variables were performed with both Fisher's exact and two sample t-test. Forty-three patients (58% female, 33.55 ± 10.21 years) with CoA completed CTA of the intracranial vessels. Five patients (11%) were found to have IA. Patients with IA were older than those without (45.6 ± 8.17 vs. 30.89 ± 7.89, P = 0.0003). There were no statistically significant differences detected between measurements of fasting lipid profiles, C-reactive protein, brain natriuretic peptide, and homocysteine levels among CoA patients with and without IA (P = not significant). Conclusion Prospective screening of adults with CoA confirmed the increased prevalence of IA but also identified increased age as the sole risk factor. These data suggested that screening is justified particularly in the fourth and fifth decades of life. Further studies are required that focus on the development, natural history, and treatment of IA.

Background With advances in cardiac care, patients with congenital heart disease, including single ventricle (SV) physiology, now survive into adulthood. These patients often suffer from congestive heart failure (CHF) with overexpression of serum biomarkers. Strain and strain rate (SR) may better describe the myocardial mechanics of a failing SV. Our objective was to determine the correlation between strain/SR and biomarkers in adult patients with SV and CHF. Methods Adult patients (age ≥8 years) with a SV were enrolled. Strain/SR in a 16-segment model of a SV was measured using 2D speckle echocardiography. Serum levels of interleukin 6, interleukin 8, matrix metalloproteinase 9, procollagen I C-terminal peptide (PCIP), cross-linked carboxy-terminal telopeptide of type I collagen (ICTP), pro-B-type natriuretic peptide, nitrotryrosine, tissue growth factor beta (TGF-β), tumor necrosis factor alpha, vascular endothelial growth factor, and creatinine (Cr) were measured. Patients underwent a complete 6 Minute Walk Test (MWT). Pearson correlation coefficient was used. P