Utility of Conventional but Late Pulmonary Artery Banding in Complex Cyanotic Congenital Heart Disease in a Toddler - A Single Case Scenario.

Newborns with untreated single ventricles develop pulmonary vascular diseases early in their lives. At that age, during the first eight weeks after birth, clinicians perform pulmonary artery (PA) banding to reduce the blood flow to the lung, decreasing the likelihood of future high vascular resistance or pressure. PA banding is also considered an initial stage in the process of single ventricle palliation procedures. We report a case of a 16-month-old toddler (7 kg) with room air saturation of 82%, diagnosed with tricuspid valve atresia, large atrial and ventricular septal defect, and hypoplastic right ventricle with severe pulmonary arterial hypertension. The baby underwent a successful surgical procedure of PA banding and was discharged after 13 days of hospital stay with a room air saturation of 89%. This case highlighted the benefit of PA banding beyond the stipulated period.

Initial Experience with Telemedicine for Interstage Monitoring in Infants with Palliated Congenital Heart Disease.

Infants with staged surgical palliation for congenital heart disease are at high-risk for interstage morbidity and mortality; home monitoring programs have mitigated these risks. In 2019, we instituted telemedicine (TM) in our established Infant Single Ventricle Monitoring Program. All consecutive patients discharged following neonatal operation/intervention were monitored until subsequent stage 2 surgical palliation. We offered TM (synchronous video) visits as part of regularly scheduled follow-up, replacing at least one in-person primary care visit with a TM cardiologist visit. We tracked emergency department (ED) visits, hospitalizations, how TM identified clinical concerns, and whether use of TM prevented unnecessary ED visits or expedited in-person assessment. We assessed caregiver and clinician satisfaction. Between 8/2019 and 5/2020, we conducted 60 TM visits for 29 patients. Of 31 eligible patients, 2 families (6.9%) declined. Median monitoring time was 199 days (range 75-264) and median number of TM visits/patient was 2 (range 1-5). In 6 visits (10%), significant clinical findings were identified which avoided an ED visit. Five TM visits led to expedited outpatient assessments, of which 1 patient required hospitalization. There were no missed events or deaths. Median ED visits/patient/month were significantly lower compared to the same calendar period of the prior year (0.0 (0-2.5) vs. 0.4 (0-3.7), p = 0.0004). Caregivers and clinicians expressed high levels of satisfaction with TM. TM for this high-risk population is feasible and effective in identifying clinical concerns and preventing unnecessary ED visits. TM was particularly effective during the COVID-19 pandemic, allowing for easy adaptation of care to ensure patient safety in this fragile cohort.

SARS-Cov-2 infection in patients with univentricular heart after fontan operation

Background and Aim: COVID-19 pandemic caused by SARS-Cov-2 coronavirus affects all groups of patients. Although pediatric pop-ulation seems to be less affected with milder or asymptomatic course of SARS-CoV-2 infection, there are few groups of patients with potential high risk of severe or fatal course of coronavirus dis-ease. These include children with congenital heart defects. The aim of this study was to evaluate the course of SARS-Cov-2 infection in patients with univentricular heart after Fontan operation. Method(s): From September 2020 to May 2021 (before vccination started in pediatric population in Poland) we screen all 38 Fontan patients admitted to Cardiology Department, Polish Mother's Memorial Hospital Research Institute for SARS-Cov2 antibodies. Result(s): We found positive SARS-Cov-2 antibodies in 21 unvac-cinated Fontan patients (55% of all hospitalized Fontan patients), 15 boys (71%) and 6 girls in the age 3-22 years (mean 11 years). 14 patients (67%) had hypoplastic left heart syndrome. Course of SARS-CoV-2 infection: asymptomatic course in 11(52%) patients, fever in 7 (33%) patients, cough 4 (19%) patients, diar-rhoea in 2 patients, loss of smell and taste-1 patient. One, 18 years old patient suffered from Covid fog symptoms (impairment of sus-tained attention and memory problems), he hasn't notice any SARS-Cov-2 symptoms but the level of antiobodies was high. Only 3 patients were hospitalized in acute SARS Cov2 infection: 2 due beacause of need for intravenous rehydratation during severe diarrhoea, 1 because of JET (junctional ectopic tachycardia) during fever. There was no case of PIMS (pediatric inflammatory multi-system syndrome) in study group. Medications used in study group: aspirin in 19 (90 %), warfarin in 2, spironolactone in 18 (86%), sildenafil in 9 (43%), angiotensyn-converting enzyme inhibitors in 17 (81%), beta-blockers in 4 (19%) of patients. Conclusion(s): 1. In our study severe congenital heart defect such as univentricular heart was not a risk factor of severe course of SARS-Cov-2 infection. 2. Absence of PIMS in analized group of patients may be connected with changed immunologic response in Fontan patients and chronic use of ASA (acetylsalicylic acid). 3. The impact of SARS CoV 2 infection on patients with congenital heart defects needs further studies.

Physical activity of single ventricle patients during the covid-19 pandemic

Background and Aim: It is well known that Fontan patients tend to a sedentary lifestyle with low physical activity levels and their objec-tive exercise capacity is reduced. Currently the COVID-19 pandemic has a significant impact on the physical activity levels of the whole population. This study investigates physical activity lev-els of Fontan patients during the COVID-pandemic, and their relationship to exercise capacity, heart rates, cardiac function at cardiovascular magnetic resonance (CMR), and biomarkers. Method(s): CMR, exercise testing, 24h-ECG, and blood samples were prospectively performed in 38 Fontan patients, of which 15 were females (40%) and 18 (47%) had a single left ventricle (LV). Time interval from Fontan operation was in median (IQR) 10 (8-15) years. Physical activity was assessed by accelerom-eter during 7 consecutive days of regular school/work. Moderate intensity physical activity was defined as gt;2296 counts/minute and vigorous physical activity as gt;4012 counts/minute. Patients with moderate-to-vigorous physical activity (MVPA) below 60minutes per day were categorized as inactive as recom-mended by the WHO. Parameters of exercise capacity included maximal oxygen uptake, maximum work rate, and maximal heart rate. Result(s): Daily MVPA was in median (IQR) 40.2 (27.6-56.5) minutes and 7/18 (39%) patients reached the recommended 60minutes/day of MVPA. Daily minutes of MVPA did not cor-relate with gender, age, single left ventricle, years from Fontan sur-gery, mean heart rate, ventricular volumes, and ejection fraction at CMR, cardiac biomarkers, or exercise capacity. Conclusion(s): Only 39% of the Fontan patients meet the recommen-dation for daily MVPA. The MVPA during the pandemic in our patients' cohort is lower than the one reported in Fontan patients before the pandemic. These current changes in behavioral habits may increase the risk for habitual sedentary lifestyles in Fontan patients.

Caring for High Risk Cyanotic Infants During a Global Pandemic: A Single Center Experience with COVID-19 and the Use of Telemedicine for Interstage Home Monitoring

Background: Pediatric single ventricle patients have seen dramatic improvements in outcomes over the past several decades. This is largely attributed to the development of home monitoring programs for interstage patients - cyanotic infants who are at highest risk. In today's current COVID-19 pandemic, the use of telemedicine has allowed providers to care for these patients and support their families effectively while minimizing the risk of COVID-19 exposure. This is particularly important in this fragile patient population for whom infection with a respiratory virus could prove life threatening. Methods: Our single center study reviewed the charts of 9 patients followed by our single ventricle team through 11 months of the COVID-19 pandemic in the United States from March 1, 2020 to January 31, 2021. We reviewed the charts of cyanotic infants who were either single ventricle patients following stage 1 palliation or two ventricle patients who underwent a palliative procedure and were awaiting complete two ventricle repair. All patients discharged from the hospital and enrolled in our digital home monitoring program were included. Records were retrospectively reviewed for total number of outpatient visits (inperson versus telemedicine), adverse events, unplanned hospital readmissions, and unplanned procedures. These results were then compared to pre-pandemic data from 2018-2019. Results: Nine infants were followed for a total of 58 months during the COVID-19 pandemic. This cohort was compared to the 9 infants followed for total of 70 months from 2018-2019. During the pandemic there were 2.8 in-person visits for every 1 telemedicine visit. Patients were seen in-person on average every 6 weeks compared to every 2-3 weeks pre-pandemic. Our results demonstrated zero adverse events with the use of telemedicine compared to 1 adverse event (cerebral vascular accident) during the 2 prior years. There were a total of 16 unplanned readmissions pre-pandemic compared to 8 during the pandemic (50% decrease). Three unplanned procedures were performed pre-pandemic compared to 1 during our study period. One patient was diagnosed with acute COVID-19 infection and managed conservatively via telemedicine with full recovery. Conclusion: To our knowledge, this is the only case series reporting on the use of telemedicine during the COVID-19 pandemic in the high risk cyanotic infant population. Our center utilized telemedicine effectively with a decrease in adverse events, unplanned procedures, and unplanned admissions. Telemedicine visits allowed for identification of issues requiring hospital readmission including feeding difficulties, poor weight gain, and decreased oxygen saturations as well as conservative management of one patient with COVID-19. Thus, telemedicine may continue to be an integral part of the management of high risk cyanotic infants who are enrolled in our high risk infant home monitoring program, including monitoring of interstage single ventricle patients. This is especially true as the COVID-19 pandemic persists.

Detrimental Intrauterine Stress Exposure and Post-natal Outcomes in Prenatally Diagnosed Congenital Heart Disease

Background: Prenatal diagnosis of congenital heart disease (CHD) is a stressful event that leads to anxiety, depression and traumatic stress in expectant parents. Cortisol is elevated in times of stress, and when present in mothers, crosses the placenta and leads to suppression of the fetus' own production of cortisol. When those neonates go on to require stressful procedures in the first months of life, some are unable to effectively mount a cortisol mediated stress response which can lead to poor outcomes and even death. We sought to investigate the relationship between maternal stress during pregnancy, and neonatal outcomes. Methods: We conducted a retrospective chart review of pregnancies complicated by a fetal diagnosis of critical CHD (including transposition of the great arteries, tetralogy of Fallot, total anomalous pulmonary venous return, and coarctation) who were born between 5/1/2019 and 5/1/2021. Maternal data included demographics and medical comorbidities. Composite maternal prenatal stress score (PSS) was calculated based upon 1) prenatal mental health diagnoses, 2) housing/food insecurity 3) income insecurity, 4) social support/child care, 5) legal involvement, 6) transportation issues and 7) other stressors. Categories ranged from 0 (no concerns) to 3 (significant concerns). Infant charts were reviewed for postnatal, and post-operative outcomes including infection, inotropic support, and exogenous steroid treatment. Results: 41 maternal-fetal dyads met inclusion criteria. Demographic and catheter based intervention at a median of 8 (2-54) days of life. 13 patients had single ventricle anatomy (8 initial surgical palliation, 5 catheterization). Mothers with higher composite PSS were more likely to have infants that required steroids after CHD surgery compared to mothers with lower scores (p=.01) (figure 1). Surgical patients needing bypass were more likely to require post-operative steroids than those not requiring bypass (18/22 vs 0/4, p<.005). None of the catheter-based interventions (including those with high risk single ventricle anatomy) required steroids (p <.0001). Maternal individual stress sub-categories, severity of prenatal CHD diagnosis, and counseling during the COVID-19 era did not correlate with steroid treatment. Finally, PSS did not correlate with individual outcomes such as birthweight, inotropic support, infection or hypoglycemia. Conclusion: Maternal prenatal stress is multifactorial and higher composite maternal prenatal stress scores are correlated with post-bypass steroid requirements, suggesting that a stressful intrauterine environment can be associated with worse postoperative outcomes for the neonate.

Adults with Congenital Heart Disease during the COVID-19 Era: One-Year Tertiary Center Experience

Background: Adult patients with congenital heart disease (ACHD) might be at high risk of Coronavirus disease-2019 (COVID-19). This study aimed to report on a one-year tertiary center experience regards COVID-19 infection in ACHD patients. Methods: This is a one-year (March-2020 to March-2021) tertiary-center retrospective study that enrolled all ACHD patients;COVID-19 positive patients’ medical records, and management were reported. Results: We recorded 542 patients, 205 (37.8%) COVID-19-positive, and 337 (62.2%) COVID-19-negative patients. Palliated single ventricle and Eisenmenger syndrome patients were more vulnerable to COVID-19 infection (P < 0.05*). Cardiovascular COVID-19 complications were arrhythmias in 47 (22.9%) patients, heart failure in 39 (19.0%) patients, cyanosis in 12 (5.9%) patients, stroke/TIA in 5 (2.4%) patients, hypertension and infective endo-carditis in 2 (1.0%) patients for each, pulmonary hypertension and pulmonary embolism in 1 (0.5%) patient for each. 11 (5.4%) patients were managed with home isolation, 147 (71.7%) patients required antibiotics, 32 (15.6%) patients required intensive care unit (ICU), 8 (3.9%) patients required inotropes, 7 (3.4%) patients required mechanical ventilation, and 2 (1.0%) patients required extracorporeal membrane oxygenation (ECMO). Thromboprophylaxis was given to all 46 (22.4%) hospitalized patients. American College of Cardiology/American Heart Association classification revealed that complex lesions, and FC-C/D categories were more likely to develop severe/critical symptoms, that required mechanical ventilation and ECMO (P < 0.05*). Mortality was reported in 3 (0.6%) patients with no difference between groups (P = 0.872). 193 (35.6%) patients were vaccinated. Conclusions: COVID-19 infection in ACHD patients require individualized risk stratification and management. Eisenmenger syndrome, single ventricle palliation, complex lesions, and FC-C/D patients were more vulnerable to severe/critical symptoms that required ICU admission, mechanical ventilation, and ECMO. The vaccine was mostly tolerable.

Developing a sense of self-reliance: caregivers of infants with single-ventricle heart disease during the interstage period.

BACKGROUND: Caring for infants after the first-stage palliative surgery for single-ventricle heart disease bring challenges beyond the usual parenting responsibilities. Current studies fail to capture the nuances of caregivers' experiences during the most critical "interstage" period between the first and second surgery. OBJECTIVES: To explore the perceptions of caregivers about their experiences while transitioning to caregiver roles, including the successes and challenges associated with caregiving during the interstage period. METHODS: Constructivist Grounded Theory methodology guided the collection and analysis of data from in person or telephonic interviews with caregivers after their infants underwent the first-stage palliative surgery for single-ventricle heart disease, and were sent to home for 2-4 months before returning for their second surgery. Symbolic interactionism informed data analyses and interpretation. RESULTS: Our sample included 14 parents, who were interviewed 1-2 times between November, 2019 and July, 2020. Most patients were mothers (71%), Latinx (64%), with household incomes <$30K (42%). Data analysis led to the development of a Grounded Theory called Developing a Sense of Self-Reliance with three categories: (1) Owning caregiving responsibilities despite grave fears, (2) Figuring out how "to make it work" in the interstage period, and (3) Gaining a sense of self-reliance. CONCLUSIONS: Parents transitioned to caregiver roles by developing a sense of self-reliance and, in the process, gained self-confidence and decision-making skills. Our study responded to the key research priority from the AHA Scientific Statement to address the knowledge gap in home monitoring for interstage infants through qualitative research design.

Adapting Interstage Home Monitoring with the use of Telemedicine During the COVID-19 Pandemic.

Pediatric single ventricle patients have seen dramatic improvements in overall outcomes over the past several decades. This is attributed to the development of home monitoring programs for interstage patients. In today's current COVID-19 pandemic, the use of telemedicine has allowed providers to care for these patients and support their families effectively while minimizing the risk of COVID-19 exposure. Our single-center study reviewed the charts of nine patients followed by our single ventricle team through the COVID-19 pandemic. Patients discharged from the hospital and enrolled in our digital home monitoring program were included. Records were retrospectively reviewed for total number of outpatient visits, adverse events, unplanned hospital readmissions, and unplanned procedures. These results were then compared to outcomes from 2018 to 2019. In-person visits averaged every 6 weeks compared to every 2-3-week pre-pandemic. Zero adverse events reported with the use of telemedicine compared to one adverse event pre-pandemic. There was a 50% decrease in unplanned readmissions and 60% decrease in unplanned procedures during our study period. One patient was diagnosed with acute COVID-19 infection and managed conservatively via telemedicine with full recovery. To our knowledge, this is the only case-control study reporting the use of telemedicine during the COVID-19 pandemic in the interstage population. Although not statistically significant, we report a decrease in total adverse events, unplanned procedures, and unplanned admissions. Telemedicine visits allowed for identification of issues requiring hospital readmission as well as conservative management of one patient with COVID-19.

Fatal MIS-C in a child with palliated single-ventricle physiology

INTRODUCTION: We report a case of MIS-C, confirmed on autopsy, in a toddler with hypoplastic left heart syndrome (HLHS). DESCRIPTION: The patient was a 15 month-old male with HLHS (aortic and mitral atresia) with a superior cavopulmonary anastomosis, and complete heart block with a pacemaker, admitted for respiratory distress. He had known exposure to multiple COVID+ family members, a positive SARS-COV-2 RT-PCR, fevers, elevated inflammatory markers, skin and mucosal changes, and multi-system organ dysfunction, evolving into severe, irreversible multiple organ failure. He met laboratory and clinical criteria for MIS-C. He was treated with high-dose Methylprednisolone, IVIG, Anakinra, and convalescent plasma, but continued to clinically deteriorate. Upon death, the family elected for autopsy. Significant autopsy findings included: acute ischemia of the papillary muscles;peripheral lung infarcts and hemophagocytosis in the bone marrow. The hemophagocytosis in the bone marrow has been noted on several of the small number of autopsies performed on children with fatal MIS-C. DISCUSSION: The patient met WHO and CDC criteria for MIS-C during his hospital stay. Both criteria require fever, elevated inflammatory markers without alternative cause, evidence of prior COVID-19 infection/exposure, and multi-organ involvement. While the pathophysiology of MIS-C remains unknown, the syndrome clinically and immunologically overlaps with other hyperferritinemic syndromes, such as hemophagocytic lymphohistiocytosis and macrophage activating syndrome, where immune-mediated multi-organ injury results from a dysregulated innate immune response-related cytokine storm. The finding of marrow hemophagocytosis on this patient's autopsy adds to the small body of literature on autopsies of children with fatal MIS-C, which also note hemophagocytosis in the marrow and/or spleen. The immune profile and pathology of patients with MIS-C suggests that Macrophages have a significant role in MIS-C. This case thus adds additional credence to this important role of Macrophages, which in turn can further direct studies into its management, to prevent similar deaths.