Review of Cardiac Involvement in Multisystem Inflammatory Syndrome in Children.

Coronavirus disease 2019 (COVID-19) is an infectious disease caused by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) with substantial cardiovascular implications. Although infection with SARS-CoV-2 is usually mild in children, some children later develop a severe inflammatory disease that can have manifestations similar to toxic shock syndrome or Kawasaki disease. This syndrome has been defined by the US Centers for Disease Control and Prevention as multisystem inflammatory syndrome in children. Although the prevalence is unknown, >600 cases have been reported in the literature. Multisystem inflammatory syndrome in children appears to be more common in Black and Hispanic children in the United States. Multisystem inflammatory syndrome in children typically occurs a few weeks after acute infection and the putative etiology is a dysregulated inflammatory response to SARS-CoV-2 infection. Persistent fever and gastrointestinal symptoms are the most common symptoms. Cardiac manifestations are common, including ventricular dysfunction, coronary artery dilation and aneurysms, arrhythmia, and conduction abnormalities. Severe cases can present as vasodilatory or cardiogenic shock requiring fluid resuscitation, inotropic support, and in the most severe cases, mechanical ventilation and extracorporeal membrane oxygenation. Empirical treatments have aimed at reversing the inflammatory response using immunomodulatory medications. Intravenous immunoglobulin, steroids, and other immunomodulatory agents have been used frequently. Most patients recover within days to a couple of weeks and mortality is rare, although the medium- and long-term sequelae, particularly cardiovascular complications, are not yet known. This review describes the published data on multisystem inflammatory syndrome in children, focusing on cardiac complications, and provides clinical considerations for cardiac evaluation and follow-up.

Lodewyk H.S. van Mierop (March 31, 1927-October 17, 2021): a true giant.

We honour a great man and a true giant. Lodewyk H.S. van Mierop (March 31, 1927 - October 17, 2021), known as Bob, was not only a Paediatric Cardiologist but also a dedicated Scientist. He made many significant and ground-breaking contributions to the fields of cardiac anatomy and embryology. He was devoted as a teacher, spending many hours with medical students, Residents, and Fellows, all of whom appreciated his regularly scheduled educational sessions. Those of us who were fortunate to know and spend time with him will always remember his great mind, his willingness to share his knowledge, and his ability to encourage spirited and fruitful discussions. His life was most productive, and he will long be remembered by many through his awesome and exemplary scientific contributions.His legacy continues to influence the current and future generations of surgeons and all providers of paediatric and congenital cardiac care through the invaluable archive he established at University of Florida in Gainesville: The University of Florida van Mierop Heart Archive. Undoubtedly, with these extraordinary contributions to the fields of cardiac anatomy and embryology, which were way ahead of his time, Professor van Mierop was a true giant in Paediatric Cardiology. The invaluable archive he established at University of Florida in Gainesville, The University of Florida van Mierop Heart Archive, has been instrumental in teaching medical students, Residents, Medical Fellows, and Surgical Fellows. Only a handful of similar archives exist across the globe, and these archives are the true legacy of giants such as Dr. van Mierop. We have an important obligation to leave no stone unturned to continue to preserve these archives for the future generations of surgeons, physicians, all providers of paediatric and congenital cardiac care, and, most importantly, our patients.

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.

Artefactual atrial flutter due to interference from a portable media device.

Portable media devices are widely used by today's youth. When used in hospitals, these devices can produce artefactual arrhythmias on telemetry.

Mind the Gap! Working Toward Gender Equity in Pediatric and Congenital Heart Disease: Present and Future.

Evidence from medicine and other fields has shown that gender diversity results in better decision making and outcomes. The incoming workforce of congenital heart specialists (especially in pediatric cardiology) appears to be more gender balanced, but past studies have shown many inequities. Gender-associated differences in leadership positions, opportunities presented for academic advancement, and recognition for academic contributions to the field persist. In addition, compensation packages remain disparate if evaluated based on gender with equivalent experience and expertise. This review explores these inequities and has suggested individual and institutional changes that could be made to recruit and retain women, monitor the climate of the institution, and identify and eliminate bias in areas like salary and promotions.

Managing High Cardiac Output Failure in a Patient With Hereditary Hemorrhagic Telangiectasias During Pregnancy.

Hemorrhagic telangiectasias is a rare genetic vascular disorder that may complicate pregnancy. We report a case of a pregnant hemorrhagic telangiectasias patient with innumerable hepatic arteriovenous malformations that developed high output cardiac failure necessitating delivery. Postpartum, the patient was treated with bevacizumab that resulted in clinical improvement.

Preprocedural Anxiety in Adults With Congenital Heart Disease: The PANIC Study.

Background: Preprocedural anxiety may have detrimental effects both cognitively and physiologically. Objectives: The objective of this study was to determine the association between state (situational) and trait (persistent in everyday life) anxiety and differences between the adult congenital heart disease (ACHD) and acquired heart disease populations. Methods: The State-Trait Anxiety Inventory and financial stress scale were administered to adults with acquired and CHD at 4 tertiary referral centers in the United States prior to cardiac catheterization. Student's t-test and least absolute shrinkage and selection operator regression analyses were used to assess differences in anxiety between groups and identify the optimal model of predictors of anxiety. Results: Of the 291 patients enrolled, those with CHD (n = 91) were younger (age 41.3 ± 16.3 years vs 64.7 ± 11.3 years, P < 0.001), underwent more cardiac surgeries (P < 0.001), and had higher levels of trait anxiety (t[171] = 2.62, P = 0.001, d = 0.33). There was no difference in state anxiety between groups (t[158.65] = 1.37, P = 0.17, d = 0.18). State anxiety was singularly associated with trait anxiety. Trait anxiety was negatively associated with age and positively associated with state anxiety and financial stress. Patients with CHD of great complexity were more trait (F[2,88] = 4.21, P = 0.02) and state anxious (F[2,87] = 4.59, P = 0.01), though with relatively small effect size. Conclusions: Trait anxiety levels are higher in the ACHD population and directly associated with state anxiety. Specialists caring for ACHD patients should not only recognize the frequency of trait anxiety but also high-risk subgroups that may benefit from psychological or social interventions to reduce preprocedural anxiety.

Assessment of parental decision making in congenital heart disease, cardiomyopathy and heart transplantation: an observational study analysing decisional characteristics and preferences.

OBJECTIVE: We explore shared decision making (DM) in guardians of children with heart disease by assessing the desired weight of influence on DM and factors that may alter the relative weight of parent or medical team influence. METHODS: Guardians of patients <21 years and admitted >1 week in the paediatric cardiac intensive care unit (PCICU) were recruited. Twelve vignettes were designed including technical (antibiotic selection, intubation, peripherally inserted central catheter placement, ventricular assist device placement, heart transplant, organ rejection, heart rhythm abnormalities and resuscitation effort) and non-technical vignettes (cessation of life-sustaining therapies, depression treatment, obesity and palliative care referral). Participants responded to questions on DM characteristics and one question querying preference for relative weight of parent or medical team influence on DM. RESULTS: Of 209 participants approached, 183 were included. Most responded with equal desire of medical team and parental influence on DM in all vignettes (range 41.0%-66.7%). Technical scenarios formed one cluster based on DM characteristics, compared with non-technical scenarios. Factors that increase the relative weight of parental influence on DM include desired input and involvement in big-picture goals (OR 0.274, CI [0.217 to 0.346]; OR 0.794, CI [0.640 to 0.986]). Factors that increase the relative weight of medical team influence on DM include perception of medical expertise needed (OR 1.949 [1.630 to 2.330]), urgency (OR 1.373 [1.138 to 1.658]), benefit (OR 1.415 [1.172 to 1.710]), number of PCICU admissions (OR 1.134 [1.024 to 1.256]) and private insurance (OR 1.921 [1.144 to 3.226]). CONCLUSION: Although factors may alter the weight of influence on DM, most parents desire equal parental and medical team influence on DM.

Effectiveness of palivizumab prophylaxis in infants and children in Florida.

PURPOSE: Palivizumab effectiveness data on respiratory syncytial virus (RSV) infections are limited to trial settings and vary considerably between selected high-risk populations. This study aimed to evaluate effectiveness in a community-based sample. METHODS: We conducted a cohort study of children with ≥ 3 months Florida Medicaid fee-for-service eligibility between 1998 and 2004 who also had matching birth certificates. Children entered the cohort at the beginning of the RSV season, after a minimum of 60 days in ambulatory care, and were followed until the earliest of the following: season end, second birthday, loss of eligibility, hospitalization, or death. Study endpoint was the first RSV-related hospitalization. To evaluate the presence of confounding, a second endpoint, hospitalizations for pneumonia or bronchiolitis secondary to specified bacterial or viral pathogens other than RSV, was used. Palivizumab exposure defined as first use (day 1-30 of first dose), subsequent use (days 1-30 of each subsequent dose), and former use (days 31-60 after any dose if delays or no readministration occurred) was compared with non-use with a Cox regression model, adjusting for confounders. RESULTS: Hazard ratios (HRs) for RSV hospitalizations were 0.89 (95%CI, 0.71-1.12), 0.56 (95%CI, 0.46-0.69), and 0.71 (95%CI, 0.51-0.97) for first, subsequent, and former use, respectively. HRs for hospitalization because of non-RSV infections were 1.31 (95%CI, 1.04-1.65), 1.03 (95%CI, 0.86-1.23), and 1.05 (95%CI, 0.78-1.41), indicating residual confounding for first but not for subsequent and former use. CONCLUSION: In this community-based study, palivizumab was associated with a reduction in severe RSV infections of a magnitude comparable to the lower clinical trial efficacy estimates. Protection appears to extend beyond the currently recommended monthly dosing schedule.

An algorithm to identify preterm infants in administrative claims data.

PURPOSE: To develop and validate an algorithm to identify preterm infants in the absence of birth certificates within Medicaid data. METHODS: Medicaid fee-for-service claims data from Florida (FL) and Texas (TX) were linked to vital statistics data for infants who were continuously eligible during the first 3 months following birth or died within that period. Prematurity was defined as less than 34 weeks gestational age. Using FL as exploratory dataset and vital statistics birth data as gold standard, we developed a logistic regression model from diagnostic and procedure codes commonly associated with preterm care, creating a prematurity score for each infant. A score cutoff was selected that maximized sensitivity while maintaining a positive predictive value (PPV) ≥ 90%. Confirmatory analyses were conducted in the TX datasets. RESULTS: The prevalence of prematurity was 5.2% (95%CI: 5.1-5.2) and 4.5% (95%CI: 4.4-4.6) in FL and TX, respectively. Using only gestational age International Classification of Disease version 9, Clinical Modification (ICD-9-CM) codes (765.20-765.27) associated with inpatient claims achieved sensitivity of 25.7% (FL) and 12.5% (TX), specificity of 99.9% (FL) and (TX), and PPV of 91.7% (FL) and 84.8% (TX). The model had excellent discriminatory validity with a c-statistic of 0.928 (95%CI: 0.925-0.931). The selected cutoff point achieved sensitivity of 52.6%, specificity of 99.8%, and PPV of 91.7% in FL. In TX, sensitivity was 46.8%, specificity was 99.9%, and PPV was 82.2%. CONCLUSION: Identification of prematurity based on gestational age ICD-9-CM codes is not sensitive. The prematurity score has superior construct validity and allows more comprehensive identification of preterm infants in the absence of birth certificates.

DiGeorge syndrome: consider the diagnosis.

DiGeorge syndrome (DGS) is caused by a chromosomal microdeletion at 22q11.2 that results in impaired development of the pharyngeal pouch system. Patients with DGS may have developmental abnormalities of craniofacial structures, parathyroid glands, thymus and cardiac outflow tract. Doctors have been routinely testing for DGS in newborns with conotruncal cardiac anomalies since the late 1990s; before then, however, they relied on complex diagnostic criteria and the disease was often missed. Adults born with conotruncal defects before the late 1990s may have undiagnosed DGS. We present one such case: a 35-year-old woman with a cardiac diagnosis of tetralogy of Fallot and unilateral absence of a pulmonary arter who was found to have DGS. Identifying DGS in adults is important both for disease management and genetic counselling. Our case emphasises the importance of screening for DGS in adults who were born with conotruncal cardiac abnormalities before widespread neonatal testing became common.

Artificial Intelligence in Congenital Heart Disease: Current State and Prospects.

The current era of big data offers a wealth of new opportunities for clinicians to leverage artificial intelligence to optimize care for pediatric and adult patients with a congenital heart disease. At present, there is a significant underutilization of artificial intelligence in the clinical setting for the diagnosis, prognosis, and management of congenital heart disease patients. This document is a call to action and will describe the current state of artificial intelligence in congenital heart disease, review challenges, discuss opportunities, and focus on the top priorities of artificial intelligence-based deployment in congenital heart disease.

Rationale, design and methodology of APPROACH-IS II: International study of patient-reported outcomes and frailty phenotyping in adults with congenital heart disease.

BACKGROUND: In recent years, patient-reported outcomes (PROs) have received increasing prominence in cardiovascular research and clinical care. An understanding of the variability and global experience of PROs in adults with congenital heart disease (CHD), however, is still lacking. Moreover, information on epidemiological characteristics and the frailty phenotype of older adults with CHD is minimal. The APPROACH-IS II study was established to address these knowledge gaps. This paper presents the design and methodology of APPROACH-IS II. METHODS/DESIGN: APPROACH-IS II is a cross-sectional global multicentric study that includes Part 1 (assessing PROs) and Part 2 (investigating the frailty phenotype of older adults). With 53 participating centers, located in 32 countries across six continents, the aim is to enroll 8000 patients with CHD. In Part 1, self-report surveys are used to collect data on PROs (e.g., quality of life, perceived health, depressive symptoms, autonomy support), and explanatory variables (e.g., social support, stigma, illness identity, empowerment). In Part 2, the cognitive functioning and frailty phenotype of older adults are measured using validated assessments. DISCUSSION: APPROACH-IS II will generate a rich dataset representing the international experience of individuals in adult CHD care. The results of this project will provide a global view of PROs and the frailty phenotype of adults with CHD and will thereby address important knowledge gaps. Undoubtedly, the project will contribute to the overarching aim of improving optimal living and care provision for adults with CHD.

Health Care Policy and Congenital Heart Disease: 2020 Focus on Our 2030 Future.

The congenital heart care community faces a myriad of public health issues that act as barriers toward optimum patient outcomes. In this article, we attempt to define advocacy and policy initiatives meant to spotlight and potentially address these challenges. Issues are organized into the following 3 key facets of our community: patient population, health care delivery, and workforce. We discuss the social determinants of health and health care disparities that affect patients in the community that require the attention of policy makers. Furthermore, we highlight the many needs of the growing adults with congenital heart disease and those with comorbidities, highlighting concerns regarding the inequities in access to cardiac care and the need for multidisciplinary care. We also recognize the problems of transparency in outcomes reporting and the promising application of telehealth. Finally, we highlight the training of providers, measures of productivity, diversity in the workforce, and the importance of patient-family centered organizations in advocating for patients. Although all of these issues remain relevant to many subspecialties in medicine, this article attempts to illustrate the unique needs of this population and highlight ways in which to work together to address important opportunities for change in the cardiac care community and beyond. This article provides a framework for policy and advocacy efforts for the next decade.

Palivizumab utilization and compliance: trends in respiratory syncytial virus prophylaxis in Florida.

OBJECTIVES: To analyze adherence to guidelines to prevent respiratory syncytial virus hospitalization and to a monthly immunoprophylaxis schedule in the absence of prior authorization requirements. STUDY DESIGN: Among Florida Medicaid fee-for-service recipients 0 to 2 years of age from the 1998/1999 season through the 2004/2005 season with available birth certificates, we identified indications for palivizumab prophylaxis based on claims data. At least 4 doses of palivizumab in the 5 core season-months were considered full season coverage. RESULTS: Of 302 101 children-seasons, 6089 were associated with 24 469 doses of palivizumab. In the 2004/2005 season, 73.6% of children with chronic lung disease received immunoprophylaxis, 67.6% children with gestational age <32 weeks, 37% with congenital heart disease, 26.4% with cystic fibrosis, and 19.4% with severe immunodeficiency. Multiple indications increased the likelihood for prophylaxis from 34.9% to 80.4%. Full season coverage was consistent across indications at approximately 70%. From the 1998/1999 season through the 2004/2005 season, 8038 doses were administered during 2051 children-seasons without any indication; mostly (69.6%) where premature children had exceeded the recommended age range for prophylaxis. CONCLUSIONS: High utilization rates were found in children with multiple indications, and compliance with a monthly schedule was consistently high. One third of doses were administered outside of guidelines, suggesting suboptimal utilization of resources in the absence of prior authorization.

Pre-existing cardiovascular conditions and pharmacological treatment of adult ADHD.

BACKGROUND: Stimulants and atomoxetine should generally not be used or used only with caution in adults with pre-existing cardiovascular conditions. The extent to which pre-existing cardiovascular conditions influence initiation of these ADHD medications in adults is not known. METHODS: We performed a retrospective cohort study of privately insured adults with new ADHD treatment episodes. Pre-existing cardiovascular conditions were assessed by the presence of ICD-9-CM codes for congenital abnormalities, atherosclerosis, cardiac disease, and cerebrovascular disease in the 12 months before the index ADHD diagnosis. The primary outcome was new initiation of a stimulant or atomoxetine in the 3 months after the index date. Multivariate logistic regression was used to predict the likelihood of treatment initiation with stimulants or atomoxetine based on pre-existing cardiovascular conditions, patient demographic characteristics, clinical mental disorder comorbidities, other psychotropic drug use, and provider type. RESULTS: Of 8752 patients with a new ADHD treatment episode, 917 (10.5%) had evidence of >or=1 pre-existing cardiovascular condition. Stimulants were started by 40.8% of patients with and 53.0% of patients without pre-existing cardiovascular conditions (Adjusted Odds Ratio, AOR 0.71; 95%CI 0.61-0.82). Pre-existing cardiovascular conditions reduced the likelihood of initiating stimulant treatment in younger but not in older patients (p-value for age x cardiovascular condition interaction = 0.0002). Initiation of atomoxetine treatment was not affected by pre-existing cardiovascular conditions (AOR 1.19, 95%CI 0.94-1.50). CONCLUSIONS: Pre-existing cardiovascular conditions reduce the likelihood of stimulant therapy but not atomoxetine treatment in adult ADHD patients. However, many adult ADHD patients with pre-existing cardiovascular conditions initiate stimulant therapy.

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.