Advancing Wearable Biosensors for Congenital Heart Disease: Patient and Clinician Perspectives: A Science Advisory From the American Heart Association.

Wearable biosensors (wearables) enable continual, noninvasive physiologic and behavioral monitoring at home for those with pediatric or congenital heart disease. Wearables allow patients to access their personal data and monitor their health. Despite substantial technologic advances in recent years, issues with hardware design, data analysis, and integration into the clinical workflow prevent wearables from reaching their potential in high-risk congenital heart disease populations. This science advisory reviews the use of wearables in patients with congenital heart disease, how to improve these technologies for clinicians and patients, and ethical and regulatory considerations. Challenges related to the use of wearables are common to every clinical setting, but specific topics for consideration in congenital heart disease are highlighted.

Cardiovascular Prevention Among Young Adults with Congenital Heart Disease.

PURPOSE OF REVIEW: There are over a million adults living with congenital heart disease (CHD) in the USA. There have been improvements in CHD management which have led to an expansion of the adult congenital heart disease (ACHD) population. There is a high prevalence of atherosclerotic cardiovascular disease (ASCVD) encountered in the aging ACHD population. This review focuses on the most recent literature regarding the primary prevention of ASCVD in young ACHD patients. RECENT FINDINGS: There are unique considerations for ASCVD risk reduction in ACHD patients. ASCVD may be as prevalent in ACHD compared in the general population. However, there may be a perceived shorter life expectancy in ACHD patients; therefore, primary prevention of ASCVD may not be considered important. Preventative strategies for ASCVD are underutilized in ACHD patients. As these patients are followed for a lifetime by cardiologists, we can truly pursue primary prevention in this aging population.

Laparoscopic Surgery Requiring Abdominal Insufflation in Patients With Congenital Heart Disease.

OBJECTIVE: To determine if patients with congenital heart disease are undergoing laparoscopic surgery requiring abdominal insufflation and to compare the outcomes of these procedures with those who underwent an open surgical approach. DESIGN, SETTING, PARTICIPANTS: This was a retrospective study using the National Inpatient Sample from 2006 to 2014. Individuals with congenital heart disease who underwent at least one of six selected surgical procedures (laparoscopic or open) were included in the study. Subgroup analysis was performed on patients with Fontan palliation. MEASUREMENTS AND MAIN RESULTS: The primary outcome was to determine the frequency with which congenital heart disease patients undergo laparoscopic surgery requiring abdominal insufflation compared with open surgery. Secondary outcomes included all-cause in-hospital mortality and in-hospital length of stay. Of the 5,527 patients included, nearly half underwent laparoscopic surgery (46.3%), and 128 (2.3%) had single-ventricle circulation. All-cause mortality was significantly higher for those who underwent open surgery compared with the laparoscopic approach (3.6% v 0.9%; odds ratio [OR], 4.0 [2.6-6.3]; p < 0.0001). Subgroup analysis of patients with Fontal palliation older than five years showed 30 (42%) underwent laparoscopic surgery and there was no mortality difference between the laparoscopic and open approaches (OR, 1.4 [0.2-21.3], p = 0.8). Length of stay was significantly shorter for patients undergoing laparoscopic compared with open surgery (median three days [interquartile range, two-five] v six days [three-13], p < 0.0001). CONCLUSIONS: Individuals with congenital heart disease are being offered laparoscopic surgery that requires abdominal insufflation. All-cause mortality and length of stay were higher for patients who underwent open surgical operations.

Patient evaluation of a virtual visit program for adults with congenital heart disease.

BACKGROUND: Improved longevity for adults with congenital heart disease (ACHD) necessitates regular, longitudinal care for this population. Telemedicine has emerged as a strategy to increase access to subspecialty care. We evaluated patient experience with a virtual visit program in the pre-COVID era to identify patient-centered benefits and limitations. METHODS: We enrolled patients for 30-minute synchronous videoconferencing virtual visits at our institution between October 2013 and March 2019. All patients were Massachusetts residents. Patients were surveyed and their characteristics were abstracted from electronic medical records. RESULTS: A total of 264 virtual visits were conducted among 174 patients with a median age of 40 years. Patients traveled a median of 70 miles for in-person visits. Many visits were to review patient data (47%), and most individuals had moderate complexity CHD (45%). Patients reported very high satisfaction with a median visit rating of 10. Patients mostly preferred virtual visits when considering convenience and cost. No difference in preference to in-person visits was reported when considering sharing private information, confidence that concerns would be addressed, and overall visit quality. In-person visits were still preferred for personal connections and showing a physical problem. CONCLUSION: We find that patients are highly satisfied with virtual visits. ACHD programs should consider blended virtual and in-person care. Long-term regulatory provisions will further improve care through the expansion of telemedicine in the post-COVID era.

Interventional Therapy Versus Medical Therapy for Secundum Atrial Septal Defect: A Systematic Review (Part 2) for the 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines.

Secundum atrial septal defect (ASD) is the most common adult congenital heart defect and can present with wide variation in clinical findings. With the intention of preventing morbidity and mortality associated with late presentation of ASD, consensus guidelines have recommended surgical or percutaneous ASD closure in adults with right heart enlargement, with or without symptoms. The aim of the present analysis was to determine if the protective effect of secundum ASD closure in adults could be qualified by pooling data from published studies. A systematic review and meta-analysis were performed by using EMBASE, MEDLINE (through PubMed), and the Cochrane Library databases to assess the effect of secundum ASD percutaneous or surgical closure in unoperated adults ≥18 years of age. Data were pooled across studies with the DerSimonian-Laird random-effects model or a Bayesian meta-analysis model. Between-study heterogeneity was assessed with Cochran's Q test. Bias assessment was performed with the Newcastle-Ottawa Scale and the Cochrane Risk of Bias Tool, and statistical risk of bias was assessed with Begg and Mazumdar's test and Egger's test. A total of 11 nonrandomized studies met the inclusion criteria, contributing 603 patients. Pooled analysis showed a protective effect of ASD closure on New York Heart Association functional class and on right ventricular systolic pressure, volumes, and dimensions. Two additional studies comprising 652 patients were reviewed separately for mortality outcome and primary outcome of interest because they did not meet the inclusion criteria. Those studies showed that ASD closure was associated with a weak protective effect on adjusted mortality rate but no significant impact on atrial arrhythmias in patients >50 years of age. Across all studies, there was significant heterogeneity between studies for nearly all clinical outcomes. The overall body of evidence was limited to observational cohort studies, the limitations of which make for low-strength evidence. Even within the parameters of the included studies, quality of evidence was further diminished by the lack of well-defined clinical outcomes. In conclusion, pooled data analysis on the impact of secundum ASD closure in adults was notably limited because of the lack of randomized controlled trials in patients with only secundum ASD. The few cohort studies in this population demonstrated improvement in functional status and right ventricular size and function as shown by echocardiogram. However, our findings suggest that at the time of this publication, insufficient data are available to determine the impact of ASD repair on mortality rate in adults.

Recognition and management of adults with Turner syndrome: From the transition of adolescence through the senior years.

Turner syndrome is recognized now as a syndrome familiar not only to pediatricians and pediatric specialists, medical geneticists, adult endocrinologists, and cardiologists, but also increasingly to primary care providers, internal medicine specialists, obstetricians, and reproductive medicine specialists. In addition, the care of women with Turner syndrome may involve social services, and various educational and neuropsychologic therapies. This article focuses on the recognition and management of Turner syndrome from adolescents in transition, through adulthood, and into another transition as older women. It can be viewed as an interpretation of recent international guidelines, complementary to those recommendations, and in some instances, an update. An attempt was made to provide an international perspective. Finally, the women and families who live with Turner syndrome and who inspired several sections, are themselves part of the broad readership that may benefit from this review.

Diagnosis and Management of Noncardiac Complications in Adults With Congenital Heart Disease: A Scientific Statement From the American Heart Association.

Life expectancy and quality of life for those born with congenital heart disease (CHD) have greatly improved over the past 3 decades. While representing a great advance for these patients, who have been able to move from childhood to successful adult lives in increasing numbers, this development has resulted in an epidemiological shift and a generation of patients who are at risk of developing chronic multisystem disease in adulthood. Noncardiac complications significantly contribute to the morbidity and mortality of adults with CHD. Reduced survival has been documented in patients with CHD with renal dysfunction, restrictive lung disease, anemia, and cirrhosis. Furthermore, as this population ages, atherosclerotic cardiovascular disease and its risk factors are becoming increasingly prevalent. Disorders of psychosocial and cognitive development are key factors affecting the quality of life of these individuals. It is incumbent on physicians who care for patients with CHD to be mindful of the effects that disease of organs other than the heart may have on the well-being of adults with CHD. Further research is needed to understand how these noncardiac complications may affect the long-term outcome in these patients and what modifiable factors can be targeted for preventive intervention.

Prediction of adverse events after catheter-based procedures in adolescents and adults with congenital heart disease in the IMPACT registry.

AIMS: We sought to identify factors associated with major adverse events (MAE) after cardiac catheterization in adolescents and adults with congenital heart disease (CHD), and create the first model to individualize risk discussions in this growing population. METHODS AND RESULTS: Improving Pediatric and Adult Congenital Treatment (IMPACT), a National Cardiovascular Data Registry, contains congenital catheterization data from over 87 hospitals in the United States. Demographics, pre-procedure, and procedural variables were collected for patients over age 10. Multivariable logistic regression was used to identify significant predictors of MAE, a composite of death, urgent surgery or procedure due to a catheterization complication, transfusion, embolic stroke, tamponade, extracorporeal membrane oxygenation or ventricular assist device placement, and device embolization, malposition or thrombosis requiring surgical intervention. A risk score was built based on the effect sizes of each predictor and validated in a split sample. A MAE occurred in 686 (2.5%) of the 27 293 index procedures meeting inclusion criteria. The independent multivariate predictors of MAE were older age, pre-procedural anticoagulation use, renal disease, lower haemoglobin, lower oxygen saturation, non-elective procedure, higher index procedure risk and having had no prior cardiac procedures. Being underweight or overweight had borderline significance and was added to the model. The C-statistic for the model was robust at 0.787 in the derivation and 0.773 in the validation cohort. CONCLUSION: The factors predicting adverse events after cardiac catheterization in adolescents and adults with CHD are different than in the general population. Validation of this model in other national or multi-institutional datasets is the next step.

Design and rationale of a prospective, collaborative meta-analysis of all randomized controlled trials of angiotensin receptor antagonists in Marfan syndrome, based on individual patient data: A report from the Marfan Treatment Trialists' Collaboration.

RATIONALE: A number of randomized trials are underway, which will address the effects of angiotensin receptor blockers (ARBs) on aortic root enlargement and a range of other end points in patients with Marfan syndrome. If individual participant data from these trials were to be combined, a meta-analysis of the resulting data, totaling approximately 2,300 patients, would allow estimation across a number of trials of the treatment effects both of ARB therapy and of ß-blockade. Such an analysis would also allow estimation of treatment effects in particular subgroups of patients on a range of end points of interest and would allow a more powerful estimate of the effects of these treatments on a composite end point of several clinical outcomes than would be available from any individual trial. DESIGN: A prospective, collaborative meta-analysis based on individual patient data from all randomized trials in Marfan syndrome of (i) ARBs versus placebo (or open-label control) and (ii) ARBs versus ß-blockers will be performed. A prospective study design, in which the principal hypotheses, trial eligibility criteria, analyses, and methods are specified in advance of the unblinding of the component trials, will help to limit bias owing to data-dependent emphasis on the results of particular trials. The use of individual patient data will allow for analysis of the effects of ARBs in particular patient subgroups and for time-to-event analysis for clinical outcomes. The meta-analysis protocol summarized in this report was written on behalf of the Marfan Treatment Trialists' Collaboration and finalized in late 2012, without foreknowledge of the results of any component trial, and will be made available online (http://www.ctsu.ox.ac.uk/research/meta-trials).

Distinct effects of losartan and atenolol on vascular stiffness in Marfan syndrome.

We conducted a randomized, double-blind trial of losartan (100 mg QD) versus atenolol (50 mg QD) for 6 months in adults with Marfan syndrome. Carotid-femoral pulse wave velocity (PWV), central augmentation index (AIx), aortic diameter and left ventricular (LV) function were assessed with arterial tonometry and echocardiography. Thirty-four subjects (18 female; median age 35 years, IQR 27, 45) were randomized. Central systolic and diastolic blood pressure decreased comparably with atenolol and losartan (p = 0.64 and 0.31, respectively); heart rate decreased with atenolol (p = 0.02), but not with losartan. PWV decreased in patients treated with atenolol (-1.15 ± 1.68 m/s; p = 0.01), but not in those treated with losartan (-0.22 ± 0.59 m/s; p = 0.15; between-group difference p = 0.04). In contrast, AIx decreased in the losartan group (-9.6 ± 8.6%; p < 0.001) but not in the atenolol group (0.9 ± 6.2%, p = 0.57; between-group difference p < 0.001). There was no significant change in aortic diameters or LV ejection fraction in either treatment group. In adults with Marfan syndrome, 6 months of treatment with atenolol improves PWV, whereas losartan reduces the AIx. By improving vascular stiffness via distinct mechanisms of action, there is physiologic value to considering the use of both medications in individuals with Marfan syndrome.

Transcatheter Pulmonary Valve Replacement With Balloon-Expandable Valves: Utilization and Procedural Outcomes From the IMPACT Registry.

BACKGROUND: Transcatheter pulmonary valve replacement (TPVR) has expanded and evolved since its initial commercial approval in the United States in 2010. OBJECTIVES: This study sought to characterize real-world practice, including patient selection, procedural outcomes, complications, and off-label usage. METHODS: Characteristics and outcomes for patients undergoing balloon-expandable TPVR were collected from the American College of Cardiology National Cardiovascular Data Registry IMPACT (Improving Pediatric and Adult Congenital Treatment) Registry. RESULTS: Between April 2016 and March 2021, 4,513 TPVR procedures were performed in patients with a median age of 19 years, 57% with a Melody (Medtronic Inc) and 43% with a SAPIEN (Edwards Lifesciences) valve. Most implanting centers performed <10 cases annually. One-third of transcatheter pulmonary valve implants were into homograft conduits, one-third were into bioprosthetic valves (BPVs), 25% were in native or patched right ventricular outflow tracts (RVOTs), and 6% were into Contegra (Medtronic Inc) conduits. Over the course of the study period, SAPIEN valve use grew from ∼25% to 60%, in large part because of implants in patients with a native/patched RVOT. Acute success was achieved in 95% of patients (95.7% in homografts, 96.2% in BPVs, 94.2% in native RVOTs, and 95.4% in Contegra conduits). Major adverse events occurred in 2.4% of procedures, more commonly in patients with a homograft (2.9%) or native RVOT (3.4%) than a prior BPV (1.4%; P = 0.004). CONCLUSIONS: This study describes novel population data on the use and procedural outcomes of TPVR with balloon-expandable valves. Over time, there has been increasing use of TPVR to treat regurgitant native RVOT anatomy, with the SAPIEN valve more commonly used for this application.

Collaborative Intelligence to catalyze the digital transformation of healthcare.

Collaborative intelligence reflects the promise and limits of leveraging artificial intelligence (AI) technologies in clinical care. It involves the use of advanced analytics and computing power with an understanding that humans bear responsibility for the accuracy, completeness and any inherent bias found in the training data. Clinicians benefit from using this technology to address increased complexity and information overload, support continuous care and optimized resource allocation, and to enact efforts to eradicate disparities in health care access and quality. This requires active clinician engagement with the technology, a general understanding of how the machine produced its insight, the limitations of the algorithms, and the need to screen datasets for bias. Importantly, by interacting, the clinician and the analytics will create trust based on the clinician's critical thinking skills leveraged to discern value of machine outputs within clinical context. Utilization of collaborative intelligence should be staged with the level of understanding and evidence. It is particularly well suited to low-complexity non-urgent care and to identifying individuals at rising risk within a population. Clinician involvement in algorithm development and the amassing of evidence to support safety and efficacy will propel adoption. Utilization of collaborative intelligence represents the natural progression of health care innovation, and if thoughtfully constructed and equitably deployed, holds the promise to decrease clinician burden and improve access to care.

Cardiovascular Risk Management in the South Asian Patient: A Review.

South Asians represent a growing percentage of the diverse population in the U.S. and are disproportionately impacted by a greater burden of aggressive and premature cardiovascular disease. There are multiple potential explanations for these findings including a high prevalence of traditional risk factors (particularly diabetes, dyslipidemia, and obesity), a genetic predisposition, and unique lifestyle factors. In this review, we discuss the cardiovascular risk stratification and disease management goals for South Asian adults. We review the pharmacologic and non-pharmacologic interventions studied in this population and discuss the role of specialized clinics and digital outreach to improve care for this vulnerable group of patients.

Isolated Coarctation of the Aorta: Current Concepts and Perspectives.

Current management of isolated CoA, localized narrowing of the aortic arch in the absence of other congenital heart disease, is a success story with improved prenatal diagnosis, high survival and improved understanding of long-term complication. Isolated CoA has heterogenous presentations, complex etiologic mechanisms, and progressive pathophysiologic changes that influence outcome. End-to-end or extended end-to-end anastomosis are the favored surgical approaches for isolated CoA in infants and transcatheter intervention is favored for children and adults. Primary stent placement is the procedure of choice in larger children and adults. Most adults with treated isolated CoA thrive, have normal daily activities, and undergo successful childbirth. Fetal echocardiography is the cornerstone of prenatal counseling and genetic testing is recommended. Advanced 3D imaging identifies aortic complications and myocardial dysfunction and guides individualized therapies including re-intervention. Adult CHD program enrollment is recommended. Longer follow-up data are needed to determine the frequency and severity of aneurysm formation, myocardial dysfunction, and whether childhood lifestyle modifications reduce late-onset complications.

Disparities in patient engagement with video telemedicine among high-video-use providers during the COVID-19 pandemic.

Aims: Known racial, ethnic, age, and socioeconomic disparities in video telemedicine engagement may widen existing health inequities. We assessed if telemedicine disparities were alleviated among patients of high-video-use providers at a large cardiovascular practice. Methods and results: All telemedicine visits from 16 March to 31 October 2020 and patient demographics were collected from an administrative database. Providers in the upper quintile of video use were classified as high-video-use providers. Descriptive statistics and a multivariable logistic model were calculated to determine the distribution and predictors of a patient ever having a video visit vs. only phone visits. A total of 24 470 telemedicine visits were conducted among 18 950 patients by 169 providers. Video visits accounted for 48% of visits (52% phone). Among telemedicine visits conducted by high-video-use providers (n = 33), ever video patients were younger (P < 0.001) and included 78% of Black patients vs. 86% of White patients (P < 0.001), 74% of Hispanic patients vs. 86% of non-Hispanic patients (P < 0.001), and 79% of public insurance patients vs. 91% of private insurance patients (P < 0.001). High-video-use provider patients had 9.4 (95% confidence interval 8.4-10.4) times the odds of having video visit compared to low-video-use provider patients. Conclusion: These results suggest that provider-focused solutions alone, including promoting provider adoption of video visits, may not adequately reduce disparities in telemedicine engagement. Even in the presence of successful clinical infrastructure for telemedicine, individuals of Black race, Hispanic ethnicity, older age, and with public insurance continue to have decreased engagement. To achieve equity in telemedicine, patient-focused design is needed.

Social determinants of telemedicine utilization in ambulatory cardiovascular patients during the COVID-19 pandemic.

Aims: The coronavirus disease 2019 (COVID-19) pandemic has resulted in the rapid uptake of telemedicine (TM) for routine cardiovascular care. To examine the predictors of TM utilization among ambulatory cardiology patients during the COVID-19 pandemic. Methods and results: In this single-centre retrospective study, all ambulatory cardiovascular encounters occurring between 16 March and 19 June 2020 were assessed. Baseline characteristics by visit type (in-person, TM phone, TM video) were compared using Chi-square and student t-tests, with statistical significance defined by P-value <0.05. Multivariate logistic regression was used to explore the predictors of TM vs. in-person care. A total of 8446 patients [86% Non-Hispanic (NH) White, 42% female, median age 66.8 ± 15.2 years] completed an ambulatory cardiovascular visit during the study period. TM phone (n = 4981, 61.5%) was the primary mode of ambulatory care followed by TM video (n = 2693, 33.2%). NH Black race [odds ratio (OR) 0.56, 95% confidence interval (CI): 0.35-0.94; P-value = 0.02], Hispanic ethnicity (OR 0.53, 95% CI: 0.29-0.98; P = 0.04), public insurance (Medicaid OR 0.50, 95% CI: 0.32-0.79; P = 0.003, Medicare OR 0.65, 95% CI: 0.47-0.89; P = 0.009), zip-code linked median household income of <$75 000, age >85 years, and patients with a diagnosis of heart failure were associated with reduced access to TM video encounters and a higher likelihood of in-person care. Conclusions: Significant disparities in TM video access for ambulatory cardiovascular care exist among the elderly, lower income, as well as Black and Hispanic racial/ethnic groups.

Outcomes of open and endovascular repair of Kommerell diverticulum.

OBJECTIVES: Kommerell diverticulum (KD) is a rare congenital vascular anomaly often associated with an aberrant subclavian artery (ASCA). Definitive indications for intervention remain unclear. We present open and endovascular (EV) operative outcomes in a large contemporary series and propose a management algorithm. METHODS: Between 2004 and 2020, 224 patients presented with ASCA and associated KD to our institution. Of the 43 (19.2%) patients who underwent operative repair, 31 (72.1%) had open surgical (OS) repair via thoracotomy and 12 (27.9%) had EV repair. Univariable and bivariable statistical analyses were conducted stratified by approach. The median follow-up time was 5.4 years (IQR, 2.9-9.7). RESULTS: Patients in EV group were older (68 years vs 47 years, P < 0.001) and had larger aneurysms (base diameter 3.2 cm vs 21.5 cm, P = 0.007). All patients with dysphagia lusoria were treated with open surgery (n = 20). Asymptomatic patients with incidentally detected KD (50% vs 16.1%), those with chest or back pain (50% vs 19.4%) and patients who presented with an aortic emergency (25% vs 6.5%) were more likely to be treated endovascularly (P = 0.001). Carotid-to-subclavian bypass was used in 38 (88.4%) patients. There were no operative mortalities. In-hospital mortality was similar between groups (3.2% vs 16.7%, P = 0.121). Mid-term mortality was higher in the EV group [4 (33.8%) vs 0, P < 0.001]. There were 2 (15.4%) postoperative strokes in the EV group. There were no statistically significant differences in other postoperative complications or hospital length of stay between groups. CONCLUSIONS: KD can be managed using open or EV approaches with low morbidity and mortality. Treatment strategy should depend on clinical presentation and patient factors.