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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.

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.

Background: In recent years, patient-reported outcomes (PROs) have received increasing prominence in cardio-vascular 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., KU Leuven - University of Leuven; Research Foundation Flanders [1159522N, 12E9819N]; Swedish Heart and Lung Foundation [20190525]; Research Council of Aga Khan University in Karachi in Pakistan; Maltese Cardiac Society; Children's Heart Unit Fund; UnIC (Unidade de Investigacao Cardiovascular da FMUP); Italian Ministry of Health, This work is supported by KU Leuven - University of Leuven, Research Foundation Flanders through grants 1159522N and 12E9819N, Swedish Heart and Lung Foundation through grant 20190525, the University Research Council of the Aga Khan University in Karachi in Pakistan, Maltese Cardiac Society, UnIC (Unidade de Investigacao Cardiovascular da FMUP) supported the costs of the translations into Portuguese used in Portugal, Children's Heart Unit Fund ( www.chuf.org.uk), Newcastle upon Tyne through their funding of the CHUF Fontan nurse specialist. This study was partially supported by Ricerca Corrente funding from the Italian Ministry of Health to IRCCS Policlinico San Donato.

ObjectiveWe 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.MethodsGuardians of patients 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.ResultsOf 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]).ConclusionAlthough factors may alter the weight of influence on DM, most parents desire equal parental and medical team influence on DM.

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.

Purpose We aimed to construct a dynamic model for predicting severe QT interval prolongation in hospitalized patients using inpatient electronic health record (EHR) data. Methods A retrospective cohort consisting of all adults admitted to 2 large hospitals from January 2012 through October 2013 was established. Thirty-five risk factors for severe QT prolongation (defined as a Bazett’s formula—corrected QT interval [QTc] of ≥500 msec or a QTc increase of ≥60 msec from baseline) were operationalized for automated EHR retrieval; upon univariate analyses, 26 factors were retained in models for predicting the 24-hour risk of QT events on hospital day 1 (the Day 1 model) and on hospital days 2–5 (the Days 2–5 model). Results A total of 1,672 QT prolongation events occurred over 165,847 days of risk exposure during the study period. C statistics were 0.828 for the Day 1 model and 0.813 for the Days 2–5 model. Patients in the upper 50th percentile of calculated risk scores experienced 755 of 799 QT events (94%) allocated in the Day 1 model and 804 of 873 QT events (92%) allocated in the Days 2–5 model. Among patients in the 90th percentile, the Day 1 and Days 2–5 models captured 351 of 799 (44%) and 362 of 873 (41%) QT events, respectively. Conclusion The risk models derived from EHR data for all admitted patients had good predictive validity. All risk factors were operationalized from discrete EHR fields to allow full automation for real-time identification of high-risk patients. Further research to test the models in other health systems and evaluate their effectiveness on outcomes and patient care in clinical practice is recommended.

[Extract] The 2018 ACHD guideline is a full revision of the “2008 ACC/AHA Guidelines for the Management of Adults with Congenital Heart Disease” (S1.4-1), which was the first U.S. guideline to be published on the topic. This revision uses the 2008 ACHD guideline as a framework and incorporates new data and growing ACHD expertise to develop recommendations. Congenital heart disease (CHD) encompasses a range of structural cardiac abnormalities present before birth attributable to abnormal fetal cardiac development but does not include inherited disorders that may have cardiac manifestations such as Marfan syndrome or hypertrophic cardiomyopathy. Also not included are anatomic variants such as patent foramen ovale. Valvular heart disease (VHD) may be congenital, so management overlaps with the “2014 AHA/ACC Guidelines for the Management of Patients With Valvular Heart Disease” (S1.4-2), particularly for bicuspid aortic valve (BAV) disease. Where overlap exists, this document focuses on the diagnosis and treatment of congenital valve disease when it differs from acquired valve disease, whether because of anatomic differences, presence of concomitant lesions, or differences to consider given the relatively young age of patients with ACHD. This guideline is not intended to apply to children (

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.

Girls and women with Turner syndrome face a lifelong struggle with both congenital heart disease and acquired cardiovascular conditions. Bicuspid aortic valve is common, and many have left-sided heart obstructive disease of varying severity, from hypoplastic left-sided heart syndrome to minimal aortic stenosis or coarctation of the aorta. Significant enlargement of the thoracic aorta may progress to catastrophic aortic dissection and rupture. It is becoming increasingly apparent that a variety of other cardiovascular conditions, including early-onset hypertension, ischemic heart disease, and stroke, are the major factors reducing the life span of those with Turner syndrome. The presentations and management of cardiovascular conditions in Turner syndrome differ significantly from the general population. Therefore, an international working group reviewed the available evidence regarding the diagnosis and treatment of cardiovascular diseases in Turner syndrome. It is recognized that the suggestions for clinical practice stated here are only the beginning of a process that must also involve the establishment of quality indicators, structures and processes for implementation, and outcome studies.

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.

Many children with invisible chronic illnesses (ICIs) are living to adulthood, necessitating that they prepare for their future. Health care and education systems have different meanings and processes for transition, although both systems are designed to help young adults prepare for independence. As health care and educational services support and educate each child and the adult he or she will become, it makes sense for these systems to coordinate their services to improve student postschool outcomes. This article addresses the importance of coordination and collaboration between health care transition and education transition for youth with ICI. The authors present a model that may be used to guide the efforts to coordinate these two forms of transition along with recommending preliminary steps that can be taken to facilitate this merger.

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 ≥3months 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 60days 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. Copyright © 2011 John Wiley & Sons, Ltd.

Many children born with complex childhood illnesses that historically caused early death are now surviving into adulthood with the expectation of leading meaningful and productive lives. They will ultimately need to transition their care from pediatric to adult-centered care. Unfortunately, in the absence of structured programs to guide this transition, there is often delayed or inappropriate care, improper timing of the transfer of care, and undue emotional and financial stress on the patients, their families, and the healthcare system. At its worst, and as frequently happens now, patients are lost to appropriate follow-up. In fact, the number of adults with congenital heart disease (CHD) in the United States is rising exponentially and now exceeds 1 000 000.1,–,7 At least half of these patients may have complex CHD. Fewer than 30% of adults with CHD are seen by appropriate specialized providers. Fewer than 15% of these patients, who are seen in specialty adult CHD (ACHD) clinics, have CHD that is classified as severe.8 Thus, adolescents with CHD constitute a growing population of individuals for whom a well-planned and well-executed “transition process” is essential. The goals of a formal transition program are to prepare young adults for transfer of care. It should provide uninterrupted health care that is patient centered, age and developmentally appropriate, flexible, and comprehensive. It should include age-appropriate education about medical conditions and promote skills in communication, decision making, self-care, and self-advocacy.9,–,13 It should foster greater personal and medical independence and a greater sense of control over health, healthcare decisions, and psychosocial environment. The ultimate goal of a transition program is to optimize the quality of life (QOL), life expectancy, and future productivity of young patients.14 We acknowledge that the development of ideal transition programs is a …

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 ≥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 × 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. Copyright © 2010 John Wiley & Sons, Ltd.

Adult congenital heart disease (ACHD) patients face unique medical and social challenges that may contribute to psychological difficulties. The goals of this study were to identify predictors of symptoms of depression and anxiety and evaluate the prevalence of mood and anxiety disorders among North American ACHD patients.In this cross-sectional study, consecutive patients were recruited from two ACHD outpatient clinics. All patients completed self-report psychosocial measures and a subset was randomly selected to participate in structured clinical interviews. Linear regression models were used to predict symptoms of depression and anxiety.A total of 280 patients (mean age=32 years; 52% female) completed self-report measures. Sixty percent had defects of moderate complexity and 31% had defects of great complexity. Significant predictors of depressive symptoms were loneliness (p0.001), perceived health status (p0.001), and fear of negative evaluation (p=0.02). Predictors of anxiety symptoms were loneliness (p0.001) and fear of negative evaluation (p0.001). Disease severity and functional class did not predict mood or anxiety symptoms. Fifty percent of interviewed patients (29/58) met diagnostic criteria for at least one lifetime mood or anxiety disorder, of whom 39% had never received any mental health treatment.The results confirm an increased risk and under-treatment of mood and anxiety disorders in ACHD patients. Social adjustment and patient-perceived health status were more predictive of depression and anxiety than medical variables. These factors are modifiable and therefore a potential focus of intervention.

Many children with complex congenital heart disease are now surviving childhood with the potential to live meaningful and productive adult lives. The process of transitioning or preparing patients and families for transfer from pediatric to adult care is challenging and rarely implemented properly. An inadequate transition process results in delayed and inappropriate care, improper timing of transfer, and undue emotional and financial stress on the patients, their families, and the health care system. At worst, patients are lost to appropriate follow-up. This article discusses the general principles of transition and transfer for young adults with chronic illness, highlights the needs of young adults with congenital heart disease, discusses the barriers to transition, and proposes goals and key elements of a formal transition program.

OBJECTIVES: Safety concerns about central nervous system stimulants for the treatment of attention-deficit/hyperactivity disorder (ADHD) include adverse cardiac effects. This study aimed to compare the risk for cardiac events in users of methylphenidate and amphetamine salts. METHODS: A retrospective cohort design using claims data from the Florida Medicaid fee-for-service program representing a total of 2131953 children and adolescents was used. The analysis included all beneficiaries who were between 3 and 20 years of age, enrolled between July 1994 and June 2004, had at least 1 physician diagnosis of ADHD and were newly started on methylphenidate or amphetamine salts. Each month of follow-up was classified according to stimulant use into current use or former use. We defined cardiac events as first emergency department (ED) visit for cardiac disease or symptoms. Risk between current users of methylphenidate versus amphetamine salts and former users of drugs in these categories was compared by using a time-dependent Cox proportional hazard model that adjusted for differences in gender; race; age; year of the index date; disability; congenital anomalies; history of circulatory disease; history of hospital admission; and use of antidepressants, antipsychotics, and bronchodilators. RESULTS: A total of 456 youth visited the ED for cardiac reasons during 52783 years of follow-up. After adjustment for differences in covariates, the risk for cardiac ED visits was similar among current users of methylphenidate or amphetamines. Periods of former use had a similar risk between youth with an exposure history to methylphenidate or amphetamine. CONCLUSION: Exposure to methylphenidate and amphetamines salts showed similar risk for cardiac ED visits. Additional population-based studies that address manifestation of serious heart disease, especially after long-term use, dosage comparisons, and interactions with preexisting cardiac risk factors are needed to inform psychiatric treatment decisions.

Background: Little is known about longitudinal changes in drug utilization in attention-deficit/hyperactivity disorder (ADHD). Objective: To describe longitudinal trends in ADHD drug utilization and explore demographic differences among youths eligible for a large Southern state Medicaid program. Methods: A cross-sectional and longitudinal analysis of 10 years of claims data for all Medicaid beneficiaries younger than 20 years of age with 6 months or more of continuous insurance (N = 2,131,953) was conducted. Annual prevalence, incidence, and persistence in ADHD medication use (stimulants and atomoxetine) were estimated based on pharmacy claims and clinician-reported ADHD diagnosis. Results: ADHD prevalence increased 1.70-fold (95% CI 1.67 to 1.73) from 3.10% (21,904 of 705,573 beneficiaries) in fiscal year 1995–1996 to 5.27% (41,661 of 790,338) in 2003–2004, paralleled by a 1.84-fold (95% CI 1.81 to 1.87) increase in drug use to 4.63%. In 2003–2004, 0.69% of youths were diagnosed and newly started on drugs, reflecting a 1.38-fold (95% CI 1.33 to 1.43) increase over 1995–1996. One in five white males between the ages of 10 and 14 years (19.24%; 95% CI 18.81 to 19.67) received ADHD medication in 2003–2004. Males continued to be more likely diagnosed and treated than females (prevalence ratio [PR] in 2003–2004 = 2.96:95% CI 2.90 to 3.03 vs 3.82; 95% CI 3.69 to 3.96 in 1995–1996), as were whites when compared with Hispanics (PR in 2003–2004 = 2.65; 95% CI 2.57 to 2.73 vs 3.78; 95% CI 3.57 to 3.99 in 1995–1996) and blacks (PR in 2003–2004 = 1.81; 95% CI 1.76 to 1.85 vs 2.00; 95% CI 1.93 to 2.07 in 1995–1996). The most common starting age throughout the study period was 5–9 years, with 2.45% (95% CI 2.37 to 2.52) new ADHD drug users in 2003–2004, but largest increases in prevalence were observed in adolescents 15–19 years of age, with 2.47% (95% CI 2.38 to 2.55) in 2003–2004 compared with 0.45% (95% CI 0.41 to 0.49) in 1995–1996. Medication persistence varied, with only 49.9% (95% CI 49.4 to 50.5) of new users receiving drugs after 1 year, with yet another 17.2% (95% CI 16.4 to 18.0) continuing for 5 years or more. Conclusions: ADHD drug utilization continues to increase due to steady increases in diagnosis and chronic use of the drugs over several years. While racial, ethnic, and sex differences persist, the age distribution of drug users has shifted toward older children. These findings emphasize the need for studies that analyze determinants of treatment as well as outcomes, both benefits and risks, associated with long-term medication use.

The collective responsibility of transitioning youth with congenital heart disease (CHD) from pediatric to adult-focused care is shared by patients, their families, and members of their pediatric and adult health-care teams. In this chapter, we describe multidisciplinary collaboration (1) between pediatric and adult providers and (2) between various health-care professionals (nurses, physicians, and allied health professionals). For optimal outcomes, health-care providers should work together to send a uniform message to adolescents and young adults and their families, namely one that encourages increased health-care knowledge and self-management skills.

OBJECTIVES. Case reports have raised concerns about the risk of cardiac events associated with central nervous system stimulants for the treatment of attention-deficit/hyperactivity disorder. PATIENTS AND METHODS. This was a retrospective cohort study that used 10 years (July 1994 to June 2004) of Florida Medicaid claims data cross-linked to Vital Statistics Death Registry data. The cohort was composed of all youth 3 to 20 years old who were newly diagnosed with attention-deficit/hyperactivity disorder. Each month of follow-up was classified according to stimulant claims (methylphenidate, amphetamines, and pemoline) as current use (active stimulant claim), former use (time after periods of current use), or nonuse (time preceding the first stimulant claim, including follow-up of youth who were never exposed to stimulants). The study's end points were (1) cardiac death, (2) first hospital admission for cardiac causes or (3) first emergency department visit for cardiac causes. Risks were compared with time-dependent Cox regression analysis adjusting for various cardiac risk factors. RESULTS. During 124932 person-years of observation (n = 55383), 73 youth died, 5 because of cardiac causes. No cardiac death occurred during 42612 person-years of stimulant use. Hospital admissions for cardiac cause occurred for 27 children (8 during stimulant use, 11 during 35671 person-years of former use, and 8 during 46649 person-years of nonuse); and 1091 children visited the emergency department for cardiac causes (8.7 per 1000 person-years). Current stimulant use was associated with a 20% increase in the hazard for emergency department visits when compared with nonuse. No increased risk was found for periods of former use when compared with nonuse. CONCLUSIONS. Incidence rates of cardiac events requiring hospitalization were small and similar to national background rates. Stimulants were associated with an increase in cardiac emergency department visits. More evidence is needed that addresses the long-term risk/benefit of the various treatment options and the effect of other cardiac risk factors and comedications.

As a result of significant advances in diagnostic, surgical, interventional, and pharmacological approaches, up to 95% of infants born with a congenital heart defect now survive into adulthood and there are at least 800,000 adult congenital heart disease patients living in the United States. Unfortunately, many of these individuals consider themselves "cured" or "fixed" and might have the misperception of a cure for a variety of reasons. The "cured" label is problematic and congenital heart disease is most accurately considered a chronic condition. This article outlines the concerns associated with the cured label. This is followed by the presentation of 4 illustrating case studies. Members of an adult congenital cardiology healthcare team must be prepared to address the full spectrum of concerns faced by patients who experience unexpected health deterioration. This spectrum includes both biomedical and psychosocial factors.

1.1 Document Development Process The Society of Pediatric Cardiology Training Program Directors (SPCTPD) board assembled a Steering Committee that nominated 2 chairs, 1 SPCTPD Steering Committee member, and 4 additional experts from a wide range of program sizes, geographic regions, and

After completing this article, readers should be able to: 1. Describe the developments that have allowed advances in neonatal cardiac surgery. 2. Describe the diagnostic approach to hypoplastic left heart syndrome (HLHS). 3. Delineate the preoperative management of the neonate who has HLHS. 4. Describe the original Norwood procedure and subsequent modifications. 5. Delineate the optimal therapy in the postoperative period for neonates who have a single ventricle. Advances in the surgical and medical management of children who have congenital heart disease (CHD) have led to a dramatic improvement in overall survival and reduced the long-term sequelae of open-heart surgery. With improved survival and decreased morbidity over the past 2 decades, the early complete repair of complex congenital heart problems in preterm and low-birthweight neonates has gained wider acceptance. Cardiologists and cardiothoracic surgeons now consider and perform complete neonatal repair more frequently than palliative surgery in this patient population. The treatment of hypoplastic left heart syndrome (HLHS) best illustrates the evolution of management strategies to improve survival and long-term outcome. A recent modification of the Norwood operation that involves a right ventricle-to-pulmonary artery conduit has led to much improved early postoperative stability and, in many centers, improved survival to a stage II superior cavopulmonary connection. A multidisciplinary team approach is required to provide the necessary care for this complex patient population. In 1938, Robert Gross at Children’s Hospital in Boston successfully ligated a patent ductus arteriosus, and this procedure—performed against the wishes of surgeon-in-chief William Ladd—opened the era of surgery for CHD. (1) Gross and Hufnagel performed detailed animal experiments to develop a technique for treatment of coarctation of the aorta that involves excision and end-to-end anastomosis. (2) Clarence Craaford of Stockholm, Sweden, had visited Gross to observe his experimental work, and in October 1944, Craaford and Nylin were the first to repair aortic …

The ultra-fast, thin-cut computerised tomographic angiogram is an efficient method to diagnose extracardiac lesions associated with congenital cardiac disease. For the purposes of this review, we evaluated various facets of the technique as used in 30 patients who were referred for diagnosis of congenital cardiac disease. The technique had high diagnostic accuracy, with a sensitivity of 93 percent in 15 of these patients referred for either interventional catheterisation or surgery. There were no immediate side-effects associated with the scanning procedure. The scan was also found to be more cost-effective as compared to an alternative noninvasive modality for imaging modality, namely magnetic resonance imaging. The angiographic technique, however, does expose the child to between 2 and 2.5 rems of radiation, despite the short period of scanning, of 10 plus or minus 2 seconds.

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

This report describes a female infant with a rare chromosome defect, del. 12 (q22-24.1), who has severe pulmonary valve stenosis, an atrial septal defect, and a small muscular ventricular septal defect. At 4 months of age a balloon pulmonary valvuloplasty was performed in the cardiac catheterization laboratory. During the procedure, a large aberrant artery from the aorta to a sequestration of the right lower lobe of lung was found. The flow-off from the sequestration was into a dilated left atrium. The single artery supplying the sequestration was successfully occluded using an Amplatzer Duct Occluder device. There were no complications and the infant remains well at 1-year follow-up.

OBJECTIVE: To assess parental knowledge regarding lifelong congenital cardiac care (LLCCC). BACKGROUND: National guidelines recommend that nearly 50% of adult survivors with congenital heart disease (CHD) receive LLCCC; the number of adults who receive such care seems far less. Inadequate parental knowledge of LLCCC might contribute to care interruption. METHODS: In this multicenter study, we administered a questionnaire to parents of children with moderate and complex CHD to assess knowledge of LLCCC. RESULTS: A total of 500 parents participated; the median age of their children was 10 years (range: 2–18 years). Most parents (81%) understood that their child would need LLCCC, but only 44% recognized that their child's cardiology care should be guided by an adult congenital heart specialist in adulthood. More than half (59%) of the parents stated that their current cardiology team had never spoken to them about LLCCC, but 96% wished to learn more. Variables associated with parental LLCCC knowledge included previous discussions regarding LLCCC, underlying cardiac surgical diagnosis, and level of parental education. CONCLUSIONS: A substantial number of parents of children with moderate and complex CHD lack knowledge about LLCCC, but almost all of them have a desire to learn more about the care their child will need as an adult.

To develop and validate an algorithm to identify preterm infants in the absence of birth certificates within Medicaid data.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.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%.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.

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.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.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.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.

The world of congenital heart disease (CHD) is evolving at a rapid pace. Patients are not only surviving longer but many are surviving healthier and are anticipated to live long and productive lives into adulthood. With the tremendous advances in cardiology and cardiac surgery, the field is no longer just about ‘blue babies’ and struggling to survive past an operation, but rather about how to move successfully into and through adulthood. Current estimates suggest that adult survivors with CHD now outnumber children with CHD in the USA. The faces of patients with CHD are changing and guidance and practices must change with it. Transition is defined as “movement, passage, or change from one position, state, stage, subject, concept, etc., to another” (http://dictionary.reference.com/browse/transition). For congenital heart patients and providers, the process of transitioning patients to adulthood and adult-oriented care involves many facets of both medical and psychosocial care. Transition in CHD does not necessarily mean a change in physician, but rather a change in the approach to medical care by the patient and the provider and the knowledge and skills to adapt to these changes. The process often requires a shift from paediatric family-centred care to adult patient-centred care and patients learning new strategies for managing health, coping with medical decision making and facing morbidity and possibly early mortality. The process also involves educational and vocational transition to appropriately prepare for a productive adulthood. Most children will accomplish a transition to adulthood and achieve independence at some point in their lives. For children with chronic medical conditions, this is equally, if not more, important because poor transitions and gaps in care have been associated with late medical concerns.1–3 Consequences from poor transitions or gaps in care that can affect patients and the medical system include increased morbidity, emotional stress and …

OBJECTIVE: Survey of current attitudes of pediatric hospitalists related to transition of care. METHODS: We developed and piloted a survey that was validated by an expert on transition. It was introduced it to the AAP/Pediatric Hospital Medicine Listserv using Survey MonkeyTM. Any participant who agreed to the informed consent was included in the survey. RESULTS: Patients aged 16–17 with chronic medical conditions were taken care of by pediatric hospitalists 70% of the time. Patients aged 18–20 were cared for by pediatric hospitalists 36.8% of the time. Advantages of hospitalist participation in healthcare transition include improved continuity of care and quality of care. The biggest impediments might be lack of time and resources. Most surveyed would be interested in a web based educational module to develop their understanding of healthcare transition. CONCLUSION: The survey provides a snapshot of current attitudes of pediatric hospitalist involvement in transition of care. Pediatric hospitalists are interested in participating in healthcare transition. Although more research is needed to compare current models of transition services and a hospitalist model, the perception for inpatients is that better quality of care can be expected. Targeted educational modules might provide a foundation for pediatric hospitalists to build their scope of practice to include transition services. Journal of Hospital Medicine 2012. © 2011 Society of Hospital Medicine

The objective of this study was to explore the current practice and attitudes of pediatric cardiologists in the United States and Europe on the transfer and transition of children with congenital heart disease (CHD). A survey among pediatric cardiology programs in the United States and Europe was undertaken. Sixty-nine centers completed and returned the 61-item questionnaire that was specifically devised for this survey. Of 69 participating centers, 74% reported that they transfer their patients to adult-focused care. When a center transfers its patients, 80% transfer them to a formalized Adult Congenital Heart Disease Program. The median age of transfer is 18 years. Comorbidities, pregnancy, and patient/family request to leave pediatric cardiology were identified as initiators for transfer. Complexity of the heart defect was relatively less important when deciding whether to transfer patients. Only one-third of the centers that transfer their patients provide a structured preparation for patients and family. Development of a formal transition program is planned at 59% of the centers that transfer patients. In conclusion, timely transfer and a structured transition process of children with CHD are not implemented in all pediatric cardiology programs. Health-care providers working in pediatric cardiology should make their transfer policies explicit and transition programs ought to be developed.

Objective: This report of a pediatric patient with acute upper airway obstruction causing asphyxiation emphasizes the need to maintain clinical suspicion for acquired myocardial dysfunction, despite the presumed role of noncardiogenic causes for pulmonary edema after an acute upper airway obstruction. Design: Case report. Setting: A tertiary pediatric intensive care unit. Patient: A 10-year-old girl with no significant medical history who developed flash pulmonary edema and acute myocardial dysfunction after an acute upper airway obstruction. Interventions: Serial echocardiograms, exercise stress test, and coronary angiography were performed. Serial pro-brain natriuretic peptide, troponins, and CK-MB levels were also followed. Results: Troponin level normalized approximately 7 days after the acute event. CK-MB and pro-brain natriuretic peptide levels decreased but had not completely normalized by time of discharge. The patient was discharged home 10 days after the event on an anticipated 6-month course of metoprolol without any signs or symptoms of cardiac dysfunction. Conclusions: Myocardial dysfunction is rarely documented in children after an acute upper airway obstruction or an asphyxiation event. Pediatric intensivists and hospitalists should maintain a high degree of clinical suspicion and screen for possible myocardial dysfunction in the pediatric patient with an acute severe hypoxic event especially when accompanied by pulmonary edema. Prompt evaluation ensures appropriate support. Additionally, some role may exist for early adrenergic receptor blockade.

Carcinoid tumors are rare, with an incidence of 1 in 100,000 of the general population. Carcinoid heart disease can be the initial presentation of carcinoid tumors in up to 20-50% of the cases. A 25-year-old woman presenting to our outpatient cardiology clinic with a 4-year history of progressive