Mental Health Care for Children with Heart Disease and Their Families: Practical Approaches and Considerations for the Pediatric and Pediatric Cardiology Clinician.

Mental health conditions are a common comorbidity among children living with heart disease. Children with congenital heart disease are more likely to have a mental health condition than their unaffected peers or peers with other chronic illnesses, and mental health risk persists across their lifetime. While poorer mental health in adults with congenital heart disease is associated with worse overall health outcomes, the association between mental health and cardiac outcomes for children with heart disease remains unknown. Despite this, it is suspected that mental health conditions go undiagnosed in children with heart disease and that many affected children and adolescents do not receive optimal mental health care. In this article, we review mental health in congenital heart disease across the lifespan, across domains of care, and across diagnoses. Further directions to support mental health care for children and adolescents with heart disease include practical screening and access to timely referral and mental health resources.

Disparities in resource utilisation by families of children with cardiac conditions.

OBJECTIVES: There are limited data documenting sources of medical information that families use to learn about paediatric cardiac conditions. Our study aims to characterise these resources and to identify any disparities in resource utilisation. We hypothesise there are significant variations in the resources utilised by families from different educational and socio-economic backgrounds. METHODS: A survey evaluating what resources families use (websites, healthcare professionals, social media, etc.) to better understand paediatric cardiac conditions was administered to caretakers and paediatric patients at Morgan Stanley Children's Hospital. Patients with a prior diagnosis of CHD, cardiac arrhythmia, and/or heart failure were included. Caretakers' levels of education (fewer than 16 years vs. 16 years or more) and patients' medical insurance types (public vs. private) were compared with regard to the utilisation of resources. RESULTS: Surveys completed by 137 (91%) caretakers and 27 (90%) patients were analysed. Websites were utilised by 72% of caretakers and 56% of patients. Both private insurance and higher education were associated with greater reported utilisation of websites, healthcare professionals, and personal networks (by insurance p = 0.009, p = 0.001, p = 0.006; by education p = 0.022, p < 0.001, p = 0.018). They were also more likely to report use of electronic devices (such as a computer) compared to those with public medical insurance and fewer than 16 years of education (p < 0.001, p < 0.001, respectively). CONCLUSION: Both levels of education and insurance status are associated with the utilisation of informative resources and digital devices by families seeking to learn more about cardiac conditions in children.

Return to Activity After SARS-CoV-2 Infection: Cardiac Clearance for Children and Adolescents.

CONTEXT: Sedentary behavior and inability to participate in organized physical activity has negatively affected the physical and mental health of children and adolescents; however, cardiac injury and associated risk for sudden cardiac death with return to activity remains a major concern. Guidelines have been proposed for return to activities; however, these fail to address the needs of younger children and those participating in more casual activities. Guidance is needed for primary care providers to facilitate safe return to everyday activity and sports and to help direct appropriate laboratory, electrocardiographic, and anatomical assessment. EVIDENCE ACQUISITION: Review of computerized databases of available literature on SARS-CoV-2 infection in children and postinfection sequelae, risk factors for sudden cardiac death, and previous return to play recommendations. STUDY DESIGN: Clinical guidelines based on available evidence and expert consensus. LEVEL OF EVIDENCE: Level 4. RESULTS: In this report, we review the literature on return to activity after SARS-CoV-2 infection and propose recommendations for cardiac clearance for children and adolescents. Though severe disease and cardiac injury is less common in children than in adults, it can occur. Several diagnostic modalities such as electrocardiography, echocardiography, cardiac magnetic resonance imaging, and serologic testing may be useful in the cardiac evaluation of children after SARS-CoV-2 infection. CONCLUSION: Gradual return to activity is possible in most children and adolescents after SARS-CoV-2 infection and many of these patients can be cleared by their primary care providers. Providing education on surveillance for cardiopulmonary symptoms with return to sports can avoid unnecessary testing and delays in clearance.

Adaptations of paediatric cardiology practice during the COVID-19 pandemic.

During the initial surge of the COVID-19 pandemic in the spring and summer of 2020, paediatric heart centres were forced to rapidly alter the way patient care was provided to minimise interruption to patient care as well as exposure to the virus. In this survey-based descriptive study, we characterise changes that occurred within paediatric cardiology practices across the United States and described provider experience and attitudes towards these changes during the pandemic. Common changes that were implemented included decreased numbers of procedures, limiting visitors and shifting towards telemedicine encounters. The information obtained from this survey may be useful in guiding and standardising responses to future public health crises.

Effect of In utero Non-steroidal anti-inflammatory drug therapy for severe ebstein anomaly or tricuspid valve dysplasia (NSAID therapy for fetal ebstein anomaly)

Abstract: Ebstein anomaly (EA) and tricuspid valve dysplasia (TVD) are rare congenital malformations associated with nearly 50% mortality when diagnosed in utero. The diseases often produce severe tricuspid regurgitation (TR) in the fetus and in some cases, pulmonary regurgitation (PR) and circular shunting ensue. Since the ductus arteriosus (DA) plays a critical role in the circular shunt and may be constricted by transplacental non-steroidal anti-inflammatory drugs (NSAIDs), we sought to assess the effect of NSAIDs on fetuses with EA/TVD. We reviewed mothers of singleton fetuses with EA/TVD and PR, indicative of circular shunting, who were offered NSAIDs at multiple centers from 2010-2018. Initial dosing consisted of indomethacin, followed by ibuprofen in most cases. Twenty-one patients at 10 centers were offered therapy 4 at a median gestational age (GA) of 30.0 weeks (range: 20.9-34.9). Most (15/21=71%) mothers received NSAIDs, and 12/15 (80%) achieved DA constriction after a median of 2.0 days (1.0-6.0). All fetuses with DA constriction had improved PR; 92% had improved Doppler patterns. Median GA at pregnancy outcome was 36.1 weeks (30.7-39.0) in fetuses with DA constriction vs. 33 weeks (23.3-37.3) in fetuses who did not receive NSAIDs or achieve DA constriction (p=0.040). Eleven of 12 patients (92%) with DA constriction survived to live-birth, whereas 4/9 patients (44%) who did not receive NSAIDs or achieve DA constriction survived (p=0.046). In conclusion, our findings demonstrate the proof of concept that NSAIDs mitigate circular shunt physiology by DA constriction and improve PR among fetuses with severe EA/TVD. Although the early results are encouraging, further investigation is necessary to determine safety and efficacy.

Effect of In Utero Non-Steroidal Anti-Inflammatory Drug Therapy for Severe Ebstein Anomaly or Tricuspid Valve Dysplasia (NSAID Therapy for Fetal Ebstein anomaly).

Ebstein anomaly (EA) and tricuspid valve dysplasia (TVD) are rare congenital malformations associated with nearly 50% mortality when diagnosed in utero. The diseases often produce severe tricuspid regurgitation (TR) in the fetus and in some cases, pulmonary regurgitation (PR) and circular shunting ensue. Since the ductus arteriosus (DA) plays a critical role in the circular shunt and may be constricted by transplacental nonsteroidal anti-inflammatory drugs (NSAIDs), we sought to assess the effect of NSAIDs on fetuses with EA/TVD. We reviewed mothers of singleton fetuses with EA/TVD and PR, indicative of circular shunting, who were offered NSAIDs at multiple centers from 2010 to 2018. Initial dosing consisted of indomethacin, followed by ibuprofen in most cases. Twenty-one patients at 10 centers were offered therapy at a median gestational age (GA) of 30.0 weeks (range: 20.9 to 34.9). Most (15/21 = 71%) mothers received NSAIDs, and 12 of 15 (80%) achieved DA constriction after a median of 2.0 days (1.0 to 6.0). All fetuses with DA constriction had improved PR; 92% had improved Doppler patterns. Median GA at pregnancy outcome (live-birth or fetal demise) was 36.1 weeks (30.7 to 39.0) in fetuses with DA constriction versus 33 weeks (23.3 to 37.3) in fetuses who did not receive NSAIDs or achieve DA constriction (p = 0.040). Eleven of 12 patients (92%) with DA constriction survived to live-birth, whereas 4 of 9 patients (44%) who did not receive NSAIDs or achieve DA constriction survived (p = 0.046). In conclusion, our findings demonstrate the proof of concept that NSAIDs mitigate circular shunt physiology by DA constriction and improve PR among fetuses with severe EA/TVD. Although the early results are encouraging, further investigation is necessary to determine safety and efficacy.

ACC/AHA/ASE/HRS/ISACHD/SCAI/SCCT/SCMR/SOPE 2020 Appropriate Use Criteria for Multimodality Imaging During the Follow-Up Care of Patients With Congenital Heart Disease: A Report of the American College of Cardiology Solution Set Oversight Committee and Appropriate Use Criteria Task Force, American Heart Association, American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, Society of Cardiovascular Computed Tomography, Society for Cardiovascular Magnetic Resonance, and Society of Pediatric Echocardiography.

The American College of Cardiology (ACC) collaborated with the American Heart Association, American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, Society of Cardiovascular Computed Tomography, Society for Cardiovascular Magnetic Resonance, and the Society of Pediatric Echocardiography to develop Appropriate Use Criteria (AUC) for multimodality imaging during the follow-up care of patients with congenital heart disease (CHD). This is the first AUC to address cardiac imaging in adult and pediatric patients with established CHD. A number of common patient scenarios (also termed "indications") and associated assumptions and definitions were developed using guidelines, clinical trial data, and expert opinion in the field of CHD.1 The indications relate primarily to evaluation before and after cardiac surgery or catheter-based intervention, and they address routine surveillance as well as evaluation of new-onset signs or symptoms. The writing group developed 324 clinical indications, which they separated into 19 tables according to the type of cardiac lesion. Noninvasive cardiac imaging modalities that could potentially be used for these indications were incorporated into the tables, resulting in a total of 1,035 unique scenarios. These scenarios were presented to a separate, independent panel for rating, with each being scored on a scale of 1 to 9, with 1 to 3 categorized as "Rarely Appropriate," 4 to 6 as "May Be Appropriate," and 7 to 9 as "Appropriate." Forty-four percent of the scenarios were rated as Appropriate, 39% as May Be Appropriate, and 17% as Rarely Appropriate. This AUC document will provide guidance to clinicians in the care of patients with established CHD by identifying the reasonable imaging modality options available for evaluation and surveillance of such patients. It will also serve as an educational and quality improvement tool to identify patterns of care and reduce the number of Rarely Appropriate tests in clinical practice.

Cardiac workup and monitoring in hospitalised children with COVID- 19.

Approximately, 1.7 million individuals in the United States have been infected with SARS-CoV-2, the virus responsible for the novel coronavirus disease-2019 (COVID-19). This has disproportionately impacted adults, but many children have been infected and hospitalised as well. To date, there is not much information published addressing the cardiac workup and monitoring of children with COVID-19. Here, we share the approach to the cardiac workup and monitoring utilised at a large congenital heart centre in New York City, the epicentre of the COVID-19 pandemic in the United States.

Echocardiographic surveillance in children after tetralogy of Fallot repair: Adherence to guidelines?

BACKGROUND: Longitudinal clinical surveillance by transthoracic echocardiography (TTE) is an established practice in children with repaired tetralogy of Fallot (TOF). Non-Invasive Imaging Guidelines recommends a list of reporting elements that should be addressed during routine TTE in this population. In this study, we assessed the adherence to these recommendations. METHODS: This was a multi-center (n = 8) retrospective review of TTE reports in children ≤11 years of age who have had complete TOF repair. We included 10 patients from each participating center (n = 80) and scored 2 outpatient follow-up TTE reports on each patient. The adherence rate was based on completeness of TTE reporting elements derived from the guidelines. RESULTS: We reviewed 160 TTE reports on 80 patients. Median age was 4.4 months (IQR 1.5-6.6) and 3.6 years (IQR 1.3-6.4) at the time of complete surgical repair and first TTE report, respectively. The median adherence rate to recommended reporting elements was 61% (IQR 53-70). Of the 160 reports, 9 (7%) were ≥80% adherent and 40 (25%) were ≥70% adherent. Quantitative measurements of right ventricular outflow tract (RVOT), right ventricular (RV) size and function, and branch pulmonary arteries were least likely to be reported. CONCLUSIONS: Overall adherence to the most recent published imaging guidelines for surveillance of children with repaired TOF patients was suboptimal, especially for reporting of RVOT, RV size and function, and branch pulmonary arteries. Further studies are needed to explore the barriers to adherence to guidelines and most importantly, whether adherence is associated with clinical outcomes.

The landscape of cardiovascular care in pediatric cancer patients and survivors: a survey by the ACC Pediatric Cardio-Oncology Work Group.

OBJECTIVE: To enhance the understanding of cardiovascular care delivery in childhood cancer patients and survivors. STUDY DESIGN: A 20-question survey was created by the Pediatric Cardio-oncology Work Group of the American College of Cardiology (ACC) Cardio-oncology Section to assess the care, management, and surveillance tools utilized to manage pediatric/young adult cardio-oncology patients. The survey distribution was a collaborative effort between Cardio-oncology Section and membership of the Adult Congenital and Pediatric Cardiology Section (ACPC) of the ACC. RESULTS: Sixty-five individuals, all self-identified as physicians, responded to the survey. Most respondents (n = 58,89%) indicated childhood cancer patients are regularly screened prior to and during cancer therapy at their centers, predominantly by electrocardiogram (75%), standard echocardiogram (58%) and advanced echocardiogram (50%) (i.e. strain, stress echo). Evaluation by a cardiologist prior to/during therapy was reported by only 8(12%) respondents, as compared to post-therapy which was reported by 28 (43%, p < 0.01). The most common indications for referral to cardiology at pediatric centers were abnormal test results (n = 31,48%) and history of chemotherapy exposure (n = 27,42%). Of note, during post-treatment counseling, common cardiovascular risk-factors like blood pressure (31,48%), lipid control (22,34%), obesity & smoking (30,46%) and diet/exercise/weight loss (30,46%) were addressed by fewer respondents than was LV function (72%). CONCLUSIONS: The survey data demonstrates that pediatric cancer patients are being screened by EKG and/or imaging prior to/during therapy at most centers. Our data, however, highlight the potential for greater involvement of a cardiovascular specialist for pre-treatment evaluation process, and for more systematic cardiac risk factor counseling in posttreatment cancer survivors.

Effectiveness of echocardiography simulation training for paediatric cardiology fellows in CHD.

Simulation is used in many aspects of medical training but less so for echocardiography instruction in paediatric cardiology. We report our experience with the introduction of simulator-based echocardiography training at Weill Cornell Medicine for paediatric cardiology fellows of the New York-Presbyterian Hospital of Columbia University and Weill Cornell Medicine. Knowledge of CHD and echocardiographic performance improved following simulation-based training. Simulator training in echocardiography can be an effective addition to standard training for paediatric cardiology trainees.

Scimitar Syndrome in Children and Adults: Natural History, Outcomes, and Risk Analysis.

BACKGROUND: Scimitar syndrome involves congenital anomalous pulmonary venous return to the inferior vena cava. Optimal management remains controversial. We describe the natural history of disease, nonsurgical and surgical outcomes, and risk factors for poor outcomes at our institution. METHODS: Patients with anomalous pulmonary venous return to the inferior vena cava documented on echocardiography at our institution between January 1994 and January 2015 were reviewed retrospectively. The study protocol IRB-AAAO1805 was approved. RESULTS: Forty-seven patients were identified, including 20 infants with significant associated congenital heart defects (42.6%, including 7 with single ventricle physiology), and 10 infants (21.3%) and 16 noninfants (34.0%) with isolated scimitar syndrome. Median follow-up was 3.55 years. Noninfants exhibited lower incidences of right pulmonary artery hypoplasia (p < 0.001), aortopulmonary collaterals (p = 0.004), and scimitar vein obstruction at the caval confluence (p = 0.032). Eighteen patients (38.3%) underwent surgical repair for scimitar syndrome. Overall mortality after baffle repair or scimitar vein reimplantation was 37.5% (3 of 8) for infants and 0% (0 of 6) for noninfants (p = 0.209). Overall mortality for medically managed infants was 46.7% (7 of 15) compared with 0% (0 of 8) for noninfants (p = 0.052). Multivariable analyses identified infantile onset as an independent risk factor for stenosis or obstruction after repair (hazard ratio 9.34, p = 0.048), and single ventricle physiology as an independent risk factor for mortality among unrepaired patients (hazard ratio 29.8, p = 0.004). CONCLUSIONS: The severity of scimitar syndrome depends on presenting age and associated congenital heart disease. Nonsurgical and surgical outcomes are suboptimal for infantile disease, which is a risk factor for stenosis after repair. Single ventricle physiology is associated with poor prognosis.

Drug-Coated Balloon Angioplasty: A Novel Treatment for Pulmonary Artery In-Stent Stenosis in a Patient with Williams Syndrome.

A 20-month-old boy with Williams syndrome had undergone multiple surgical and catheter-based interventions for resistant peripheral pulmonary arterial stenoses with eventual bilateral stent placement and conventional balloon angioplasty. He persistently developed suprasystemic right ventricular (RV) pressure. Angioplasty with a drug-coated balloon (DCB) was performed for in-stent restenosis and to remodel his distal pulmonary vessels bilaterally. This resulted in immediate improvement in the in-stent stenosis and resultant decrease in RV pressure. Follow-up catheterization two months later continued to show long-lasting improvement in the in-stent stenosis. We hypothesize that the anti-proliferative effects of DCBs may be of benefit in the arteriopathy associated with Williams syndrome. We report this as a novel use of a DCB in the pulmonary arterial circulation in a patient with Williams syndrome.

When Is It Better to Wait? Surgical Timing and Recurrence Risk for Children Undergoing Repair of Subaortic Stenosis.

Recurrence of subaortic stenosis (SAS) after surgery is common in children. The effects of patient characteristics and surgical timing on disease recurrence are largely unknown. We performed a retrospective study, assessing the relative effects of patient age and left ventricular outflow tract (LVOT) gradient on the need for reoperation for recurrent SAS. We included all children <20 years of age who underwent initial surgical resection of SAS at our center, January 2003-December 2013. Stratified logistic regression was performed, considering the effects of patient demographics, clinical characteristics, echocardiographic parameters, and operative technique, and clustering standard errors by surgeon. The multivariable model was used to simulate predicted probabilities of recurrent SAS for children at varying ages and baseline LVOT gradients. Sixty-three patients (38 males) underwent initial operation for SAS. Patients were followed for a median of 3.7 years (IQR 1.2-7.1). Twenty-one percent of patients (n = 13) underwent reoperation for SAS. Twelve were male. For every 10 mmHg increase in preoperative peak gradient in boys, the odds of reoperation for SAS doubled (OR 2.01, CI 1.5-2.72, p < 0.001), and for every additional 6 months of age, the odds of reoperation decreased by 14% (OR 0.86, CI 0.84-0.88, p < 0.001). Both younger age and higher preoperative outflow tract gradient are independently associated with risk of reoperation. Clinicians should consider the age and rate of LVOT gradient change-and not just the absolute gradient-in determining initial surgical timing.

Comparison of method of conception in fetuses undergoing echocardiography at a tertiary referral center.

OBJECTIVES: We compared the proportion of conception with and without in vitro fertilization (IVF) in fetuses with and without congenital heart disease (CHD). METHODS: This was a retrospective review of fetal echocardiograms at Columbia University from 2007 to 2010, to identify the mode of conception. RESULTS: Echocardiography was performed on 2828 fetuses, and 2761 (97.6%) had the method of conception documented. CHD was diagnosed in 22.4%, consisting predominantly of complex CHD. The proportion of IVF conception was lower in fetuses with CHD (6.9% CHD vs 10.3% no CHD, OR = 0.65 [95% CI 0.46-0.92], p = 0.01). IVF fetuses were conceived by elder mothers and were more likely part of a multiple gestation than those without IVF. In a multivariate model controlling for maternal age and multiple gestation, IVF was not associated with CHD diagnosis (OR = 1.1 [95% CI 0.77-1.7], p = 0.51). CONCLUSION: At a tertiary referral center, fetuses with CHD were not more likely to be conceived by IVF after controlling for maternal age and multiple gestation. These results differ from those of several previous reports, which may be related to our study population, and the exclusion of isolated atrial shunts and patent ductus arteriosus, which are normal fetal findings.

Fetal echocardiographic measurements and the need for neonatal surgical intervention in Tetralogy of Fallot.

BACKGROUND: This study aimed to evaluate fetal echocardiographic measurements at the time of the first fetal echocardiogram as predictors of neonatal outcome for tetralogy of Fallot (TOF). METHODS: The study reviewed all infants with a prenatal diagnosis of TOF from January 2004 to June 2011. Aortic valve (AoV), pulmonary valve (PV), main pulmonary artery (MPA), left and right pulmonary artery diameters, and ductus arteriosus flow were evaluated on fetal echocardiograms, and associations between the fetal echocardiogram and the neonatal echocardiogram measurements and outcomes were assessed. RESULTS: The study identified 67 TOF patients who had an initial fetal echocardiogram at a mean gestational age of 25.0 ± 5.2 weeks. Patients with absent PV syndrome or major aortopulmonary collaterals were excluded from the study, as were those without anterograde pulmonary blood flow at the first fetal echocardiogram. Of the remaining 44 patients, 10 were ductal dependent and required neonatal surgery. Infants who were ductal dependent had lower fetal PV (-5.38 ± 2.95 vs. -3.51 ± 1.66; p < 0.05) and MPA (-3.94 ± 1.66 vs. -2.87 ± 1.04; p < 0.05) z-scores. A fetal PV z-score of -5 predicted ductal dependence with 78 % sensitivity and 87 % specificity, and a PV z-score of -3 showed 100 % sensitivity and 34 % specificity (p < 0.001). Fetuses with a reversed left-to-right flow across the ductus arteriosus (DA) were more likely to be ductal dependent (odds ratio, 25; p < 0.001) than those who had normal ductal flow. CONCLUSIONS: In TOF, fetal PV and MPA z-scores and direction of the DA blood flow predict neonatal ductal dependence. Patients with fetal PV z-scores lower than -3 or any left-to-right flow at the level of the DA should be admitted to a center where prostaglandin is available.

Primary arterial switch operation as a strategy for total correction of Taussig-Bing anomaly: a 21-year experience.

BACKGROUND: Studies of the arterial switch operation for Taussig-Bing anomaly demonstrate significant rates of reintervention and mortality, particularly after initial palliation to delay complete repair. We aimed to describe the long-term outcomes of our 21-year practice of single-stage arterial switch operation for all patients with Taussig-Bing anomaly. METHODS AND RESULTS: A retrospective study was performed, and 43 patients with Taussig-Bing anomaly were identified between 1990 and 2011. Median age at arterial switch operation was 7 (range, 2-192) days, and median operative weight was 3.2 (1.4-6.2) kg. Aortic arch obstruction was present in 30 patients (70%). Hospital mortality was 7% (n=3). Follow-up was available for 37 hospital survivors at a mean of 8.1 (± 6.3) years. Late mortality was 2% (n=1). At follow-up, all patients were in New York Heart Association functional class I. Freedom from transcatheter or surgical reintervention was 73% at 1 year, 64% at 5 years, and 60% at 10 years. Eleven patients underwent 13 catheter reinterventions on the pulmonary arteries (n=8) or aortic arch (n=5). Seven patients underwent 11 reoperations, including relief of right ventricular outflow tract obstruction (n=5), pulmonary arterioplasty (n=3), recoarctation repair (n=2), and tricuspid valve repair (n=1). By multivariate analysis, a preoperative aortic valve annulus z score of ≤-2.5 was associated with reintervention (hazard ratio, 7.66 [95% confidence interval, 1.29-45.6], P=0.03). CONCLUSIONS: Although reintervention is common, primary correction of Taussig-Bing anomaly with arterial switch operation can be achieved in all patients with low mortality and good long-term outcomes.