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Aims As congenital heart disease (CHD) survivors age, they are confronted with elevated risk of cardiovascular morbidity and increasingly complex disease self-management demands. Given that stress is associated with poor physical and psychosocial outcomes, it is crucial to examine how disease-related stress changes over time in this population. However, this outcome has received little research attention to date. This study aimed to identify demographic and clinical predictors of change in disease-related stress over six years among CHD survivors. Methods and Results CHD survivors (N = 252, Mage = 25.6 ± 7.1, 52.9% female) completed the first 13 items of the Responses to Stress Questionnaire, adapted for use among CHD survivors, to assess disease-related stressors at study entry (T1) and six-year follow-up (T2). Age, gender, estimated family income, and New York Heart Association (NYHA) functional class at T1 were entered into mixed linear models to determine their impact on change in disease-related stress. Older age (p Conclusion The transition from adolescence to adulthood may be a period of increasing disease-related stress. Healthcare providers should consider screening adolescents for elevated disease-related stress during transition education and provide resources to bolster resilience.

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.

The epidemiology of congenital heart disease (CHD) has changed in the past 50 years as a result of an increase in the prevalence and survival rate of CHD. In particular, mortality in patients with CHD has changed dramatically since the latter half of the twentieth century as a result of more timely diagnosis and the development of interventions for CHD that have prolonged life. As patients with CHD age, the disease burden shifts away from the heart and towards acquired cardiovascular and systemic complications. The societal costs of CHD are high, not just in terms of health-care utilization but also with regards to quality of life. Lifespan disease trajectories for populations with a high disease burden that is measured over prolonged time periods are becoming increasingly important to define long-term outcomes that can be improved. Quality improvement initiatives, including advanced physician training for adult CHD in the past 10 years, have begun to improve disease outcomes. As we seek to transform lifespan into healthspan, research efforts need to incorporate big data to allow high-value, patient-centred and artificial intelligence-enabled delivery of care. Such efforts will facilitate improved access to health care in remote areas and inform the horizontal integration of services needed to manage CHD for the prolonged duration of survival among adult patients.

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…

The landscape of congenital heart disease has changed rapidly over the past few decades. The shift from pediatric to adult congenital heart disease care has stretched resources and the ability to provide high-quality access and delivery of care for the more than 1.5 million adults with congenital heart disease in the United States. Meeting the demand for delivering high-quality care requires a team-based approach, with each member highly specialized. This review describes the deficits and deficiencies in providing care for adults with congenital heart disease in the United States and a team-based approach to improving access and delivery of care.

Background Adults presenting with an unrepaired atrial septal defect and pulmonary arterial hypertension (ASD-PAH) are typically classified as “correctable” or “non-correctable”. The use of directed PAH medical therapy in non-correctable ASD-PAH leading to favorable closure candidacy, repair status and long-term follow-up is not well studied. We therefore sought to characterize response to PAH targeted therapy in ‘non-correctable’ ASD-PAH. Methods and results Nine North American tertiary care centers submitted retrospective data from adults with unrepaired ASD-PAH that did not meet recommendations for repair at initial presentation (1996–2017). Sixty-nine patients (women 51(74%), 40 ± 15 years, mean pulmonary artery pressure (mPA) 51 ± 13 mm Hg, pulmonary vascular resistance (PVR) 8.7 ± 4.9 Wood units, Qp:Qs 1.6 ± 0.4) were enrolled. All patients were prescribed PAH targeted therapy and late shunt repair occurred in 19(28%) (Women 15(29%) vs. Men 4(22%), p = 0.6). At late follow-up (4.4 ± 2.9 years) 6-minute walk test distance (6MWTD) was significantly better in the group that underwent repair (486 ± 89 m vs. 375 ± 139 m, p Conclusions This is the first and largest multicenter study evaluating the “treat-to-close” approach in non-correctable ASD-PAH. Our new data supports further study of this strategy in patients who have reversibility of PAH in response to targeted therapy. We demonstrate that in the carefully selected patient with non-correctable ASD-PAH, successful shunt repair is possible if post-therapy PVR is ≤6.5 Wood units. Patients who underwent repair had improved RV function following PAH targeted therapy. Divergent survival curves suggest that with further study, defect repair may affect medium-term to late survival.

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.

The landscape of congenital heart disease has changed rapidly over the past few decades. The shift from pediatric to adult congenital heart disease care has stretched resources and the ability to provide high-quality access and delivery of care for the more than 1.5 million adults with congenital heart disease in the United States. Meeting the demand for delivering high-quality care requires a team-based approach, with each member highly specialized. This review describes the deficits and deficiencies in providing care for adults with congenital heart disease in the United States and a team-based approach to improving access and delivery of care.

Objectives: We aimed to evaluate the utility of computed tomography angiography (CTA) for coronary compression (CC) prediction in patients with congenital heart disease undergoing balloon-expandable transcatheter pulmonary valve replacement (TPVR). Background: Coronary compression is a serious complication of TPVR, but the value of preprocedural CTAs to assess CC risk is largely unexplored. Methods: In all, 586 patients underwent TPVR between January 2009 and July 2018. Adults with a pre-TPVR CTA and children with a CTA performed less than one year prior to TPVR were included. Patients with poor CTA image quality or with aborted cases due to reasons other than CC were excluded. Sixty-six patients were finally included. Cardiac anatomy was assessed via multiplanar reconstruction of CTAs. Results: Coronary compression occurred in 9 (14%) of the 66 patients who underwent TPVR. Most CC cases (seven of nine) occurred in patients with conduits. Proximity of the right ventricular outflow tract (RVOT) landing zone to the coronary arteries and to the chest wall was a significant risk factor for compression ( P < .001 and P = .019, respectively). Compression risk increased significantly if patients had an RVOT to coronary artery distance of ≤3 mm ( P < .001) and an RVOT to chest wall distance of ≤8 mm ( P = .026). Anomalous course of coronary arteries was another significant univariate risk factor ( P = .003). Conclusions: Right ventricular outflow tract landing zone distance of ≤3 mm to a coronary artery, landing zone distance of ≤8 mm to the chest wall, and anomalous coronary arteries are associated with increased CC risk. Electrocardiogram gating may not be necessary if coronary arteries are opacified on CTAs. Larger studies are needed to explore and confirm these coronary artery compression risk factors.

Lapses in care during transition in adult CHD patients lead to increased morbidity and mortality. Previous studies have investigated predictors of poor follow-up in universal healthcare paradigms and select American populations. We studied patients with a wide spectrum of CHD severity within a single American centre to identify factors associated with successful internal transition and maintenance of care. Loss of follow-up was defined as no documented cardiac follow-up for ⩾3 years. Ambulatory cardiology patients aged 16–17 years with CHD were retrospectively enrolled and contacted. A survey assessing demographics, patients’ understanding of their CHD, medical status, and barriers to care was administered. On the basis of chart review of 197 enrolled patients, 74 demonstrated loss of follow-up (37.6%). Of 78 successfully contacted patients, 58 were surveyed, of whom a minority had loss of follow-up (n=16). The status of most patients with loss of follow-up was not known. Maintenance of care was associated with greater complexity of CHD (p

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...

The aging patient with severe congenital heart disease (CHD) faces many challenges: heart failure, arrhythmia, and in the Fontan patient, liver disease. Our goal was to define combined heart liver transplant (CHLT) and isolated orthotopic heart transplant (OHT) outcomes in U.S. adult CHD patients. The U.S. United Network for Organ Sharing (UNOS) thoracic and liver databases were queried for cardiac and CHD diagnoses, from inception-2014.In CHLT, CHD made up 22% of waitlist patients (non-CHD n=262 vs. CHD n=58), and 20% of transplanted patients (non-CHD n=137 vs. CHD n=27). Liver function tests in the non-CHD and CHD groups were similar and there was no difference in CHD and non-CHD survival (HR 0.93, CI: 0.36-2.38, p 0.48). In isolated OHT, CHD patients comprised 2% of those listed (non-CHD n=74,080 vs. CHD n=1599) and transplanted (non-CHD n=48,985 vs. CHD n=967) and had higher early (1year) mortality (HR 1.36, CI: 1.18-1.57, p0.0001), but better long-term survival (HR 0.66, CI; 0.57-0.76, p0.001) than non-CHD. Both groups benefitted from mechanical support when used (non-CHD HR 0.34, CI: 0.31-0.37 and CHD HR 0.14, CI: 0.03-0.58) and prior sternotomy had no effect on mortality in CHD (HR 0.63, CI: 0.15-2.58).Survival of CHD patients undergoing CHLT is no different than in non-CHD, encouraging consideration of CHLT when clinically appropriate. Short-term mortality is higher in CHD (vs. non-CHD) patients undergoing OHT, regardless of prior cardiac surgery status. Modifications to CHD classification within UNOS would help better understand CHD CHLT and OHT outcomes.

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.

Objective We describe the technical considerations of transcatheter implantation of the CardioMEMS™ HF System (Abbott, Abbott Park, IL) in adult patients with complex palliated congenital heart disease (CHD) and advanced heart failure (HF). Background Ambulatory pulmonary artery (PA) pressure monitoring with implantable hemodynamic monitors (IHMs) has been shown to reduce HF-related hospital admissions in non-CHD populations. HF is a common late cardiovascular complication in adult CHD necessitating better understanding of IHM application in this population. Methods We analyzed adults with complex CHD and advanced HF who were referred for CardioMEMS™ device implantation (2015-2018). Feasibility of device implantation, defined by successful device implantation and calibration, and procedural outcomes were evaluated. Results CardioMEMS™ was successfully implanted in all 14 adults (35.5 ± 9.2 years old, 72 ± 12 kg) with complex CHD (single ventricle/Fontan, n = 8 [57%]; d-transposition of the great arteries/atrial switch, n = 6 [43%]). The device was delivered via femoral venous access in 13 (93%) patients and implanted in the left PA in 12 (86%). A long sheath was used in 8 (57%) patients, including 5/6 with an atrial switch operation. There was one device migration that did not require retrieval. Conclusions Transcatheter implantation of an IHM is feasible in select complex adult CHD patients with advanced HF. Further studies evaluating integration of ambulatory hemodynamics and the impact on clinical care are needed. This technology has the potential to improve medical management of advanced HF in patients with Fontan and atrial switch physiologies and provide new insights into their ambulatory hemodynamics.

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.

The Fontan anatomy leads to elevated central venous pressure along with chronic venous congestion and low cardiac output; this is felt to be responsible for deterioration of exercise tolerance and functional capacity over time. Real-time hemodynamic evaluation of the Fontan anatomy has not been evaluated until now. Here, we report the technical aspects of the first two adult Fontan patients to undergo placement of an invasive hemodynamic monitor (IHM). We validate IHM readings with invasive pulmonary artery catheter derived hemodynamics in the Fontan and show successful home transmission of pulmonary artery hemodynamic tracings. This technology has the capacity to change current understanding of Fontan hemodynamics and treatment in patients with complex single-ventricle anatomy. © 2016 Wiley Periodicals, Inc.

Adults with congenital heart disease (ACHD) represent a diverse cohort with specific and wide-ranging healthcare needs especially within the intensive care unit (ICU). They require coordinated care with expertise from both pediatric and internal medicine realms in both primary and consultative roles. With data showing increased morbidity and mortality as compared to other populations, the details regarding care are especially important. Cardiologists with specific training in ACHD and a team of subspecialists able to care for specific needs are necessary to providing adequate care. Consultative services with multiple subspecialties should be available to deal with litany of other healthcare needs that may become relevant. With increased morbidity and mortality and as a growing population, this cohort has unique healthcare needs within the ICU. While no perfect ICU exists for ACHD patients, combining the expertise of pediatric and internal medicine knowledge with well-trained staff together constitutes the most ideal environment to care the ACHD population.

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

Adults with congenital heart disease (ACHD) often have aortopathies. These patients require specialized care and attention before, during, and after procedures are performed. With complex relationships involving differential blood flow, elevated pressures and resistances, as well as unique blood vessel anatomy, these patients require specialty care. Knowledge of medications to avoid, details of post-procedural monitoring, and sequelae associated with the inherent congenital heart disease and aortopathy mandate that critical care staff be aware of the clinical complexities associated with each patient’s specific anatomy and physiology.