Coronavirus Disease 2019 (COVID-19) Pandemic Implications in Pediatric and Adult Congenital Heart Disease.

The corona virus disease -2019 (COVID-19) is a recently described infectious disease caused by the severe acute respiratory syndrome corona virus 2 with significant cardiovascular implications. Given the increased risk for severe COVID-19 observed in adults with underlying cardiac involvement, there is concern that patients with pediatric and congenital heart disease (CHD) may likewise be at increased risk for severe infection. The cardiac manifestations of COVID-19 include myocarditis, arrhythmia and myocardial infarction. Importantly, the pandemic has stretched health care systems and many care team members are at risk for contracting and possibly transmitting the disease which may further impact the care of patients with cardiovascular disease. In this review, we describe the effects of COVID-19 in the pediatric and young adult population and review the cardiovascular involvement in COVID-19 focusing on implications for patients with congenital heart disease in particular.

Cancer Risk in Congenital Heart Disease-What Is the Evidence?

As life expectancy in patients with congenital heart disease (CHD) has improved, the risk for developing noncardiac morbidities is increasing in adult patients with CHD (ACHD). Among these noncardiac complications, malignancies significantly contribute to the disease burden of ACHD patients. Epidemiologic studies of cancer risk in CHD patients are challenging because they require large numbers of patients, extended follow-up, detailed and validated clinical data, and appropriate reference populations. However, several observational studies suggest that cancer risks are significantly elevated in patients with CHD compared with the general population. CHD and cancer share genetic and environmental risk factors. An association with exposure to low-dose ionizing radiation secondary to medical therapeutic or diagnostic procedures has been reported. Patients with Down syndrome, as well as, to a lesser extent, deletion of 22q11.2 and renin-angiotensin system pathologies, may manifest both CHD and a predisposition to cancer. Such observations suggest that carcinogenesis and CHD may share a common basis in some cases. Finally, specific conditions, such as Fontan circulation and cyanotic CHD, may lead to multisystem consequences and subsequently to cancer. Nonetheless, there is currently no clear consensus regarding appropriate screening for cancer and surveillance modalities in CHD patients. Physicians caring for patients with CHD should be aware of this potential predisposition and meet screening recommendations for the general population fastidiously. An interdisciplinary and global approach is required to bridge the knowledge gap in this field.

A Pilot Study of Inspiratory Muscle Training to Improve Exercise Capacity in Patients with Fontan Physiology.

While the Fontan procedure has improved life expectancy, patients with single ventricle physiology have impaired exercise capacity due to limited increase in pulmonary blood flow during activity. Enhancing the "thoracic pump" using inspiratory muscle training (IMT) may ameliorate this impairment. Adult nonsmokers with Fontan physiology were recruited through Boston Children's Hospital's outpatient clinic. Participants underwent cardiopulmonary exercise testing and pulmonary function testing, followed by 12 weeks of IMT and then repeat testing. The primary endpoint was change in % predicted peak oxygen consumption (VO2). Secondary endpoints were changes in other exercise metrics. Eleven patients (6 male) were enrolled. Median ages at time of enrollment and Fontan completion were 28.8 years (25.7, 45.5) and 7.8 years (3.9, 16.5), respectively. Average baseline maximal inspiratory pressure (MIP) was normal; only 2 patients had MIP <70% predicted. Peak work rate improved significantly from baseline after 12 weeks of IMT (116.5 ± 45.0 to 126.8 ± 47.0 W, P = 0.019). Peak VO2 tended to improve (baseline 68.1 ± 14.3, change + 5.3 ± 9.6% predicted, P = 0.12), as did VE/VCO2 slope (34.1 ± 6.7 to 31.4 ± 3.6, P = 0.12). There was no change in peak tidal volume or MIP. In a small cohort of Fontan patients with mostly normal MIP, IMT was associated with significant improvement in peak work rate and a trend toward higher peak VO2 and improved ventilatory efficiency. Larger studies are needed to determine if this reflects true lack of effect or whether this pilot study was underpowered for effect size, and whether IMT is more narrowly useful for patients with impaired MIP.

Electronic medical record integration with a database for adult congenital heart disease: Early experience and progress in automating multicenter data collection.

BACKGROUND: The adoption of electronic health records (EHR) has created an opportunity for multicenter data collection, yet the feasibility and reliability of this methodology is unknown. The aim of this study was to integrate EHR data into a homogeneous central repository specifically addressing the field of adult congenital heart disease (ACHD). METHODS: Target data variables were proposed and prioritized by consensus of investigators at five target ACHD programs. Database analysts determined which variables were available within their institutions' EHR and stratified their accessibility, and results were compared between centers. Data for patients seen in a single calendar year were extracted to a uniform database and subsequently consolidated. RESULTS: From 415 proposed target variables, only 28 were available in discrete formats at all centers. For variables of highest priority, 16/28 (57%) were available at all four sites, but only 11% for those of high priority. Integration was neither simple nor straightforward. Coding schemes in use for congenital heart diagnoses varied and would require additional user input for accurate mapping. There was considerable variability in procedure reporting formats and medication schemes, often with center-specific modifications. Despite the challenges, the final acquisition included limited data on 2161 patients, and allowed for population analysis of race/ethnicity, defect complexity, and body morphometrics. CONCLUSION: Large-scale multicenter automated data acquisition from EHRs is feasible yet challenging. Obstacles stem from variability in data formats, coding schemes, and adoption of non-standard lists within each EHR. The success of large-scale multicenter ACHD research will require institution-specific data integration efforts.

Left and right ventricular diastolic function in adults with surgically repaired tetralogy of Fallot: a multi-institutional study.

BACKGROUND: Our purpose was to assess the prevalence and clinical implications of left ventricular (LV) and right ventricular (RV) diastolic dysfunction (DD) in patients with repaired tetralogy of Fallot (TOF). METHODS: Adults with repaired TOF at 11 North American congenital heart disease centres were included. Of 556 patients analyzed, 325 had sufficient Doppler data to evaluate for LV DD, defined as mitral lateral e' < 10 cm/s and E/e' ratio > 9. Abnormal RV diastolic function was defined as tricuspid E/A ratio of 0.8 to 2.1 with E/e' ratio > 6 or a tricuspid ratio E/A > 2.1 with a deceleration time < 120 milliseconds, and 105 patients were found to have sufficient Doppler data. RESULTS: Abnormal LV diastolic Doppler indices were prevalent in 13.8% and associated with hypertension, dyslipidemia, 3 or more cardiac operations, and a history of ventricular tachycardia. Abnormal RV indices were prevalent in 52.4% and associated with hypertension, dyslipidemia, diabetes mellitus, and 3 or more cardiac operations. Ventricular tachycardia was more common in those with abnormal RV diastolic function. CONCLUSION: Further prospective studies are needed to evaluate these associations with DD and the impact of treatment risk factors on diastolic function and arrhythmia burden.

Aortic root dilatation in adults with surgically repaired tetralogy of fallot: a multicenter cross-sectional study.

BACKGROUND: Although aortic root pathology has been described in patients with tetralogy of Fallot, the scope of the problem remains poorly defined. We sought to determine the prevalence and predictors of aortic root dilatation in adults with repaired tetralogy of Fallot. METHODS AND RESULTS: A multicenter cross-sectional study was conducted with standardized reassessment of echocardiographic parameters in 474 adults (≥18 years) with surgically repaired tetralogy of Fallot or pulmonary atresia with ventricular septal defect. The aortic root was measured in a parasternal long-axis view, in diastole, at the level of the sinus of Valsalva. Prevalence and predictors of an absolute diameter ≥40 mm and of an observed-to-expected ratio >1.5 were assessed. The aortic root dimension was ≥40 mm in 28.9% (95% confidence interval, 26.9%-30.9%). In multivariate analyses, the only independently associated factor was male sex (odds ratio, 4.48; 95% confidence interval, 1.55-12.89; P=0.006). The prevalence of an observed-to-expected aortic root dimension ratio >1.5 was 6.6% (95% confidence interval, 5.3%-7.9%). It was associated with pulmonary atresia and moderate or severe aortic regurgitation in univariate analyses, but no independent predictor was identified. The side of the aortic arch was not associated with aortic root dimension. The prevalence of moderate or severe aortic regurgitation was 3.5% (95% confidence interval, 2.7%-4.2%). CONCLUSIONS: Although nearly one third of adults with repaired tetralogy of Fallot have an aortic root diameter ≥40 mm, the prevalence of a dilated aortic root, when defined by an indexed ratio of observed-to-expected values, is low. Similarly, moderate or severe aortic regurgitation is uncommon.

Prevalence of left ventricular systolic dysfunction in adults with repaired tetralogy of fallot.

Left ventricular (LV) systolic dysfunction has been observed in patients with repaired tetralogy of Fallot (TOF), although its clinical associations are unknown. Adults with repaired TOF were identified from 11 adult congenital heart disease centers. Clinical history was reviewed. Patients with pulmonary atresia were excluded. Echocardiograms were reanalyzed to estimate LV ejection fraction. LV function was defined as normal (LV ejection fraction ≥ 55%) or mildly (45% to 54%), moderately (35% to 44%), or severely (< 35%) decreased. Right ventricular (RV) and LV dimensions and Doppler parameters were remeasured. Function of all valves was qualitatively scored. Of 511 patients studied, LV systolic dysfunction was present in 107 (20.9%, 95% confidence interval 17.4 to 24.5). Specifically, 74 (14.4%) had mildly decreased and 33 (6.3%) had moderately to severely decreased systolic function. Presence of moderate to severe LV dysfunction was associated with male gender, LV enlargement, duration of shunt before repair, history of arrhythmia, QRS duration, implanted cardioverter-defibrillator, and moderate to severe RV dysfunction. Severity or duration of pulmonary regurgitation was not different. In conclusion, LV systolic dysfunction was found in 21% of adult patients with TOF and was associated with shunt duration, RV dysfunction, and arrhythmia.

Arrhythmia burden in adults with surgically repaired tetralogy of Fallot: a multi-institutional study.

BACKGROUND: The arrhythmia burden in tetralogy of Fallot, types of arrhythmias encountered, and risk profile may change as the population ages. METHODS AND RESULTS: The Alliance for Adult Research in Congenital Cardiology (AARCC) conducted a multicenter cross-sectional study to quantify the arrhythmia burden in tetralogy of Fallot, to characterize age-related trends, and to identify associated factors. A total of 556 patients, 54.0% female, 36.8+/-12.0 years of age were recruited from 11 centers. Overall, 43.3% had a sustained arrhythmia or arrhythmia intervention. Prevalence of atrial tachyarrhythmias was 20.1%. Factors associated with intraatrial reentrant tachycardia in multivariable analyses were right atrial enlargement (odds ratio [OR], 6.2; 95% confidence interval [CI], 2.8 to 13.6), hypertension (OR, 2.3; 95% CI, 1.1 to 4.6), and number of cardiac surgeries (OR, 1.4; 95% CI, 1.2 to 1.6). Older age (OR, 1.09 per year; 95% CI, 1.05 to 1.12), lower left ventricular ejection fraction (OR, 0.93 per unit; 95% CI, 0.89 to 0.96), left atrial dilation (OR, 3.2; 95% CI, 1.5 to 6.8), and number of cardiac surgeries (OR, 1.5; 95% CI, 1.2 to 1.9) were jointly associated with atrial fibrillation. Ventricular arrhythmias were prevalent in 14.6% and jointly associated with number of cardiac surgeries (OR, 1.3; 95% CI, 1.1 to 1.6), QRS duration (OR, 1.02 per 1 ms; 95% CI, 1.01 to 1.03), and left ventricular diastolic dysfunction (OR, 3.3; 95% CI, 1.5 to 7.1). Prevalence of atrial fibrillation and ventricular arrhythmias markedly increased after 45 years of age. CONCLUSIONS: The arrhythmia burden in adults with tetralogy of Fallot is considerable, with various subtypes characterized by different profiles. Atrial fibrillation and ventricular arrhythmias appear to be influenced more by left- than right-sided heart disease.

Electrocardiogram screening of infants for long QT syndrome: survey of pediatric cardiologists in North America.

OBJECTIVES: The aim of this study was to evaluate current opinions toward screening infants for long QT syndrome (LQTS) by electrocardiogram (ECG) among pediatric cardiologists in North America. BACKGROUND: Research from Italy shows that ECG screening of infants for LQTS is cost-effective. METHODS: E-mail invitations were sent to 1045 pediatric cardiologists in North America listed in the American Academy of Pediatrics directory. The survey was Internet-based with multiple choice questions. Two repeat e-mail reminders were sent after the initial invitation. RESULTS: Three sixty-three (35%) responses were returned. Among the respondents, 40% had more than 20 years of clinical experience, 32% had 10 to 20 years, 21% had 5-10 years, and 6% had less than 5 years. Thirty-one percent of respondents agreed and 41% disagreed that screening LQTS may decrease the incidence of sudden infant death syndrome. When asked if an ECG between 2 and 4 weeks of life can be used to screen newborns for LQTS, 47% agreed and 33% disagreed. To the question: "pediatricians should offer families the option of 12-lead ECG at baby's 2-week visit for detecting an uncommon but potentially lethal disease," 27% agreed and 49% disagreed. When asked if there should be a mandate for ECG screening of all newborns, 11% agreed and 69% disagreed. The support for a mandate of ECG screening decreases with increasing number of years of experience (P = .03). CONCLUSIONS: Most pediatric cardiologists in North America remain skeptical about ECG screening of infants for LQTS. Among pediatric cardiologists, current support for ECG screening at pediatrician's offices is low, and only 10% would agree to a mandate for ECG screening.

Changes in hospitalization patterns among patients with congenital heart disease during the transition from adolescence to adulthood.

OBJECTIVES: This study was designed to evaluate hospitalization patterns of congenital heart disease (CHD) patients surrounding the transition from adolescence to adulthood. BACKGROUND: Few population data exist on hospitalizations among adolescent and adult CHD patients. METHODS: Patients ages 12 to 44 years with CHD were selected from the 2000 to 2003 California hospital discharge database. Patient demographics, hospitalization patterns, emergency department (ED) admissions, CHD complexity, and insurance patterns were described. Data were analyzed in 3-year age increments and compared between patients over and under age 21. Predictors of admission via the ED were determined using multivariate regression analysis. RESULTS: There were 9,017 hospitalizations at 368 hospitals. For patients ages 12 to 20 years, 12 hospitals accounted for 70% of hospitalizations; for patients ages 21 to 44 years, 25 hospitals accounted for only 44.8% of cases. Regarding insurance, 53% of admissions were private, 44% public, and <4% were self-pay. Sixty-five percent of patients had complex CHD and 19% had a cardiac procedure during hospitalization. The proportion of patients admitted via the ED nearly doubled surrounding the transition to adulthood. The positive predictors of admission via the ED included public insurance, self-pay, and age >17 years, whereas having a procedure and being female decreased the likelihood. CONCLUSIONS: Congenital heart disease hospitalizations occur at a wide variety of hospitals and disperse as patients enter adulthood. Those without private insurance and >17 years old are at higher risk of being admitted via the ED. These findings require further investigation to examine access to care and possible disparities, as they are important for future healthcare planning.

Variations in adult congenital heart disease training in adult and pediatric cardiology fellowship programs.

OBJECTIVES: The purpose of this study was to evaluate adult congenital heart disease (CHD) training among U.S. cardiology fellowship programs. BACKGROUND: Although training recommendations for caring for adults with CHD exist, the educational patterns and numbers of specialists remain unknown. METHODS: We surveyed U.S. directors of 170 adult cardiology and 45 pediatric cardiology (PC) fellowship programs. Adult program surveys contained 1 single-response and 10 multiple-choice questions; pediatric program surveys contained 1 single-response and 13 multiple-choice questions. RESULTS: Ninety-four adult cardiology fellowship directors (55%) and 34 PC directors (76%) responded. Of adult programs, 70% were in university hospitals and 40% were associated with PC groups. Those with PC-affiliation had more adult CHD clinics (p < 0.02) and more adult CHD inpatient (p < 0.02) and outpatient (p < 0.002) visits than those without PC affiliation. Most PC programs were in children's hospitals (38%) or children's hospitals within adult hospitals (50%). Eighty-two percent had associated adult cardiology programs. Pediatric programs followed adult CHD patients in various care settings. Over one-third of adult and pediatric programs had < or = 3 lectures annually regarding adult CHD. Nine adult and 2 pediatric programs offered adult CHD fellowships, and only 31 adult and 11 pediatric fellows pursued advanced CHD training in the last 10 years. CONCLUSIONS: Adult CHD didactic and clinical experiences for cardiology fellows vary widely. Few programs offer advanced CHD training, and the number of specially trained physicians is unlikely to meet projected workforce requirements. Adult cardiology programs with PC affiliation have increased CHD experience and might provide good educational models.