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Recommendations for management of patients with Kawasaki disease (KD) and coronary artery aneurysms (CAA) include physical activity (PA) promotion. This study aimed to characterize self-reported practices of KD providers to evaluate practice variation in use of cardiopulmonary exercise testing (CPET) and PA recommendations. We developed a REDCap survey with different clinical scenarios of KD patients. It was completed by members of the International Kawasaki Disease Registry (IKDR) and community pediatric cardiologists. Twenty-eight physicians responded; 63% practiced in the US, 63% practiced in an academic setting, 48% were general pediatric cardiologists, and 55% were IKDR members. Most respondents (69%) followed 50 KD patients. The great majority (93%) agreed that patients with no CAA do not require CPET and could be cleared for all PA. For patients with small CAA, 43% of respondents recommended CPET and 75% cleared for all PA if CAAs regressed completely, but only 32% cleared if CAA persisted. For patients with medium CAA, 66% respondents cleared for PA if CAA regressed, and only 7% if CAA persisted; with 66% and 75% recommending CPET, respectively. For patients with large/giant CAA, 81% of respondents recommended CPET. No respondents felt comfortable clearing their patients with persistent large/giant CAA for PA and 19% would restrict from the entire physical education program. There is practice variation in use of CPET in KD patients with CAAs. Providers are hesitant to promote PA in KD patients with CAA despite known benefits and current guidelines.

Background American Academy of Pediatrics guidelines emphasize regionalized systems of care for pediatric chronic illness. There remains a paucity of information on the status of regionalized systems of care for pediatric congenital heart disease (CHD). Objectives This study evaluated variations in use of pediatric cardiology specialty care centers (PCSCC) for pediatric patients with CHD in California between 1983 and 2011. Methods We performed a retrospective, total population analysis of pediatric CHD patients using the California Office of Statewide Health Planning and Development unmasked database. PCSCCs were identified by California's Title V program. Results There were 164,310 discharges meeting inclusion criterion. Discharges from PCSCCs grew from 58% to 88% between 1983 and 2011. Regionalized care was highest for surgical (96%) versus nonsurgical (71%) admissions. Admissions with a public payer increased from 42% (1983) to 61% (2011). Total bed days nearly doubled, and median length of stay increased from 2 to 3 days (nonspecialty care) and from 4 to 5 days (specialty care). There was a decrease in the pediatric CHD in-hospital death rate from 5.1 to 2.3 per 100,000 between 1983 and 2011, and a shift toward a larger percent of deaths occurring in the newborn period. Conclusions California’s inpatient regionalized specialty care of pediatric CHD has increased substantially since 1983, especially for surgical CHD discharges. The death rate has decreased, the number of bed days has increased, and a large proportion of these discharges now have public payers. Health care reform efforts must consider these shifts while protecting advances in regionalization of pediatric CHD care.

The American College of Cardiology and American Heart Association guidelines recommend that management of adult congenital heart disease (ACHD) be coordinated by specialty ACHD centers and that ACHD surgery for patients with moderate or complex congenital heart disease (CHD) be performed by surgeons with expertise and training in CHD. Given this, the aim of this study was to determine the proportion of ACHD surgery performed at specialty ACHD centers and to identify factors associated with ACHD surgery being performed outside of specialty centers. This retrospective population analysis used California's Office of Statewide Health Planning and Development's discharge database to analyze ACHD cardiac surgery (in patients 21 to 65 years of age) in California from 2000 to 2011. Designation as a "specialty ACHD center" was defined on the basis of a national ACHD directory. A total of 4,611 ACHD procedures were identified. The proportion of procedures in patients with moderate and complex CHD delivered at specialty centers increased from 46% to 71% from 2000 to 2011. In multivariate analysis among those discharges for ACHD surgery in patients with moderate or complex CHD, performance of surgery outside a specialty center was more likely to be associated with patients who were older, Hispanic, insured by health maintenance organizations, and living farther from a specialty center. In conclusion, although the proportion of ACHD surgery for moderate or complex CHD being performed at specialty ACHD centers has been increasing, 1 in 4 patients undergo surgery at nonspecialty centers. Increased awareness of ACHD care guidelines and of the patient characteristics associated with differential access to ACHD centers may help improve the delivery of appropriate care for all adults with CHD.

The adult with congenital heart disease (CHD) is at risk of developing atherosclerotic cardiovascular disease (ASCVD). We performed a cross-sectional study to describe established ASCVD risk factors and estimate 10-year and lifetime risk of ASCVD in adults over age 18 with CHD of moderate or great complexity using three validated risk assessment tools—the Framingham Study Cardiovascular Disease Risk Assessment, the Reynolds Risk Score, and the Atherosclerotic Cardiovascular Disease (ASCVD) Risk Estimator. We obtained extensive clinical and survey data on 178 enrolled patients, with average age 37.1±12.6 years, 51% men. At least one modifiable ASCVD risk factor was present in 70%; the two most common were overweight/obesity (53%) and systemic hypertension (24%). Laboratory data was available in 103 of the 178 patients. Abnormal levels of glycated hemoglobin, high-sensitivity C-reactive protein, and high-density lipoprotein (HDL) were each found in around 30% of patients. The 10-year ASCVD predicted risk using all three tools was relatively low (i.e., at least 90% of patients

American Academy of Pediatrics guidelines emphasize regionalized systems of care for pediatric chronic illness. There remains a paucity of information on the status of regionalized systems of care for pediatric congenital heart disease (CHD).This study evaluated variations in use of pediatric cardiology specialty care centers (PCSCC) for pediatric patients with CHD in California between 1983 and 2011.We performed a retrospective, total population analysis of pediatric CHD patients using the California Office of Statewide Health Planning and Development unmasked database. PCSCCs were identified by California's Title V program.There were 164,310 discharges meeting inclusion criterion. Discharges from PCSCCs grew from 58% to 88% between 1983 and 2011. Regionalized care was highest for surgical (96%) versus nonsurgical (71%) admissions. Admissions with a public payer increased from 42% (1983) to 61% (2011). Total bed days nearly doubled, and median length of stay increased from 2 to 3 days (nonspecialty care) and from 4 to 5 days (specialty care). There was a decrease in the pediatric CHD in-hospital death rate from 5.1 to 2.3 per 100,000 between 1983 and 2011, and a shift toward a larger percent of deaths occurring in the newborn period.California's inpatient regionalized specialty care of pediatric CHD has increased substantially since 1983, especially for surgical CHD discharges. The death rate has decreased, the number of bed days has increased, and a large proportion of these discharges now have public payers. Health care reform efforts must consider these shifts while protecting advances in regionalization of pediatric CHD care.

Objective To evaluate the accuracy of pediatric cardiologists' interpretations of electrocardiograms (ECGs). Study design A series of 18 ECGs that represented conditions causing pediatric sudden cardiac death or normal hearts were interpreted by 53 members of the Western Society of Pediatric Cardiology. Gold-standard diagnoses and recommendations were determined by 2 electrophysiologists (100% concordance). Results The average number of correct ECG interpretations per respondent was 12.4 ± 2.2 (69%, range 34%-98%). Respondents achieved a sensitivity of 68% and a specificity of 70% for recognition of any abnormality. The false-positive and false-negative rates were 30% and 32%, respectively. Based on actual ECG diagnosis, sports participation was accurately permitted in 74% of cases and accurately restricted in 81% of cases. Respondents gave correct sports guidance most commonly in cases of long QT syndrome and myocarditis (98% and 90%, respectively) and least commonly in cases of hypertrophic cardiomyopathy, Wolff-Parkinson-White syndrome, and pulmonary hypertension (80%, 64%, and 38%, respectively). Respondents ordered more follow-up tests than did experts. Conclusions Preparticipation screening ECGs are difficult to interpret. Mistakes in ECG interpretation could lead to high rates of inappropriate sports guidance. A consequence of diagnostic error is overuse of ancillary diagnostic tests.

ACC/AHA guidelines recommend a structured preparation for and transfer to adult-oriented cardiac care for adult survivors of pediatric onset heart disease (POHD). Given this, we sought to describe the transition and transfer practices for a cohort of young adults with POHD and to determine factors associated with successful transfer to adult-oriented cardiac care. We performed a single-center, retrospective chart review on patients ≥18 years of age, with POHD likely to require lifelong cardiac care, who were seen in outpatient pediatric cardiology (PC) between 2008 and 2011. Successful transfer was defined as the subsequent attendance at adult cardiology (AC) within 2 years of PC visit. We identified 118 patients who met study criteria. Mean age 22.4 ± 2.0 years, 59 % male, 64 % white and 40 % Hispanic. Mean transition education topics noted was 3.3 ± 1.8 out of 20 and covered the underlying cardiac disease (89 %), follow-up and current medications (56 %) and exercise limitations (34 %). Recommendations for follow-up were AC (57 %) and PC (33 %). Of those told to transfer to AC, 79 % successfully transferred. Characteristics of successful transfer included: prior cardiac surgery (p = 0.008), cardiac medication use (p = 0.006) and frequency of follow-up ≤1 year (p = 0.037). One-quarter of all subjects did not follow-up within at least 2 years. Despite published guidelines, transition education appears lacking and the approach to transfer to adult cardiac care is not consistent. Given the increased risk of morbidity and mortality in this patient population, standardization of transition education and transfer processes appear warranted.

HLA-A,B and DR antigens were evaluated in 51 patients with mucocutaneous lymph node syndrome (Kawasaki disease) during both endemic and epidemic periods in the Boston area. Although 80% of patients were HLA-B5 (70% HLA-Bw51) during endemic periods, there were no HLA-B5 individuals during the epidemic period. There was, however, an increased incidence of HLA-Bw44 during the Boston epidemic. Such different associations in endemic versus epidemic periods suggest possible different, as yet unknown, inciting agents.

The immune status of 21 children in the acute phase of mucocutaneous lymph node syndrome (MCLS) was assessed and compared to that of control populations consisting of age-matched normal children and of children suffering from acute febrile nonbacterial illnesses. In contrast to the controls, 13 of 18 patients studied during the acute phase of MCLS had a significant reduction in circulating T8-positive (T8+) suppressor/cytotoxic T cells (P 0.05). Three of three patients were found to have a significantly increased number of Ia-bearing T4+ T cells, suggesting the presence of circulating activated helper cells. Furthermore, 17 of 18 patients with acute MCLS had a significantly elevated number of circulating cells spontaneously secreting IgG (P < 0.01) and IgM (P < 0.001) as determined by a reverse hemolytic plaque assay. Finally mononuclear cells from 18 of 20 patients with acute MCLS had increased cytotoxicity against 51Cr-labeled normal human skin fibroblasts (P < 0.01). Follow-up studies during the convalescence phase of MCLS indicated that most of the patients had gradual resolution of their immunologic abnormalities. These results suggested that immunoregulatory abnormalities may contribute to the pathogenesis of this syndrome.

The usual indices of left ventricular systolic performance have been incapable of accurately recognizing early myocardial impairment in many patients treated with doxorubicin. Recently, several new load-independent, highly sensitive indices of left ventricular contractility have been developed including the slope value of the endsystolic pressure (Pes)-dimension (Des) relation and the position of the left ventricular end-systolic wall stress (sigma es)-percent fractional shortening (% delta D) relation. We used these indices to study 46 patients receiving either low dose or high dose doxorubicin. Results were compared with data from 30 healthy subjects. Resting % delta D failed to accurately recognize left ventricular dysfunction in 9 of 17 patients with low normal values. These patients had reduced afterload, as measured by sigma es, permitting normal extent of left ventricular fiber shortening despite impaired contractility as quantified by diminished Pes-Des slope values. There was 98% concordance between the relative position of the sigma es-% delta D relation and the slope value of the Pes-Des, relation. These indices offer an improved means of recognizing and quantitating impaired contractility in patients treated with doxorubicin.

Associations of specific histocompatibility antigens with certain human diseases may elucidate basic mechanisms of disease. Since there is evidence for a disordered immune response in MLNS, HLA antigens were evaluated by NIGH standard technique in 27 patients (3 Oriental, 1 black) fulfilling CDC criteria for MLNS. In contrast to previous Japanese studies, we found no incidence of HLA-Bw22. We did, however, find a significant increase in HLA-Bw51: CHANCES OF DISTRIBUTION OF HLA PHENOTYPES IN CAUCASIANS To our knowledge, this is only the second disease associated with HLA-B5 specificity. In preliminary studies, 10/17 Caucasian patients with MLNS were DR-2 (frequency in control population 23%, p

Immunoregulatory status was assessed in 14 children during the acute phase of Kawasaki Disease (KD) and in 6 of these children following recovery. T cell subsets were enumerated using monoclonal antibodies which define antigens on peripheral T cells (T3), helper/inducer T cells (T4), and suppressor/cytotoxic T cells (T8). Patients with acute KD had a significant reduction of circulating T8+ cells (11 ± 4% vs. 22 ± 4% for normal controls; p