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Although intravenous immunoglobulin (IVIG) is effective therapy for Kawasaki disease, 10–20% of patients have recrudescent fever as a sign of persistent inflammation and require additional treatment. We aimed to compare infliximab with a second infusion of IVIG for treatment of resistant Kawasaki disease.

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AbstractBackgroundAnomalous aortic origin of a coronary artery is the second leading cause of sudden cardiac arrest/death in young athletes in the United States of America. Limited data are available regarding family history in this patient population.MethodsPatients were evaluated prospectively from 12/2012 to 02/2017 in the Coronary Anomalies Program at Texas Children’s Hospital. Relevant family history included the presence of CHD, sudden cardiac arrest/death, arrhythmia/pacemaker use, cardiomyopathy, and atherosclerotic coronary artery disease before the age of 50 years. The presence of one or more of these in 1st- or 2nd-degree relatives was considered significant.ResultsOf 168 unrelated probands (171 patients total) included, 36 (21%) had significant family history involving 19 (53%) 1st-degree and 17 (47%) 2nd-degree relatives. Positive family history led to cardiology referral in nine (5%) patients and the presence of abnormal tests/symptoms in the remaining patients. Coronary anomalies in probands with positive family history were anomalous right (27), anomalous left (five), single right coronary artery (two), myocardial bridge (one), and anomalous circumflex coronary artery (one). Conditions present in their family members included sudden cardiac arrest/death (15, 42%), atherosclerotic coronary artery disease (14, 39%), cardiomyopathy (12, 33%), CHD (11, 31%), coronary anomalies (3, 8%), myocardial bridge (1, 3%), long-QT syndrome (2, 6%), and Wolff–Parkinson–White (1, 3%).ConclusionIn patients with anomalous aortic origin of a coronary artery and/or myocardial bridges, there appears to be familial clustering of cardiac diseases in approximately 20% of patients, half of these with early occurrence of sudden cardiac arrest/death in the family.

Long‐term outcome data on patients with anomalous aortic origin of coronary arteries (AAOCA) is sparse and they are often managed in a nonuniform manner. There is subjective perception of anxiety and unmet needs in these patients and families. An online survey of 13 questions was sent to 74 families of patients with AAOCA between May and October 2015. Descriptive statistics were performed. A total of 31 (47%) families responded. Of these, 27 expressed the need to interact with other patients/families with AAOCA. The majority were interested in either face‐to‐face meetings (77%) or online support groups (71%). Regarding content of the meeting, 74% were interested in brief talks by medical personnel/families, 58% suggested informal interactions with families, 55% proposed a structured discussion with a moderator and 39% mentioned fun activities/games. Regarding participants in these meetings, 90% would like to include healthcare providers, 61% suggested including family friends, 58% wished to include psychologists and 16% mentioned including social workers. The families currently use various social media including Facebook (87%), YouTube (39%), Google+ (36%), and LinkedIn (32%). For future online resources, 77% of families would like a Facebook site, an informative website (58%), a blog (52%), or an open forum (29%). The majority of the families (77%) were interested in attending a dedicated AAOCA meeting. There appears to be an unmet need for family support in those affected by AAOCA, a substantial life changing diagnosis for patients and families. Further research is needed to assess quality of life in this population.

Anomalous aortic origin of a coronary artery (AAOCA) is the second leading cause of sudden cardiac death in young athletes in the USA. Long‐term outcome data for these patients are lacking to date. There is insufficient knowledge on the best approach to these patients and they are managed in a nonuniform manner. An online survey of 15 questions regarding management of AAOCA was sent out to 198 cardiac healthcare providers. The goal was to define gaps in knowledge to justify a dedicated scientific forum for discussion of AAOCA. Descriptive statistics were performed. A total of 91 providers (46%) completed the survey including pediatric cardiology subspecialists (40%), general pediatric cardiologists (24%), cardiovascular (CV) surgeons (22%), adult cardiologists (10%), nurse practitioners (8%), cardiology fellows (3%) and CV anesthesiologist (1%). Forty‐eight percent had been practicing for over 15 years and 28% were in their first 5 years of practice. Fifty‐two percent of the providers cared for adults and 93% cared for children/adolescents. Eighty‐eight percent were affiliated with an academic institution. All but one provider practiced in the USA, 62% practiced in Texas. Half of participants (50%) were very comfortable managing AAOCA patients and 36% were somewhat comfortable. Providers utilized various imaging tests to confirm the anatomy including computed tomography angiography 88%, cardiac magnetic resonance imaging 70%, cardiac catheterization 60%, echocardiogram 12%, IVUS 2% and myocardial perfusion scan 1%. The majority felt comfortable in counseling the families and felt that depending on the type of lesion these patients should get surgical referral (85%) vs clinical follow up (67%) with exercise restriction (65%). There is heterogeneity in the way AAOCA patients are currently evaluated and managed. A knowledge gap exists even with participants from academic institutions. Long‐term data with a defined approach to management of these patients may help to improve outcomes and prevent unnecessary exercise restriction or surgery.

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Background:Elevated lipids are becoming an increasing problem in children particularly with increasing rate of obesity. We undertook an evaluation to determine if the risk factors are similar for children in a pediatric cardiology practice who are evaluated for lipids is similar to those for children without congenital heart disease.Methods:We performed a medical record review from January 2006- January 2018 of all children in a pediatric cardiology practice who had lipids obtained. We evaluated their weight, height, BMI, blood pressure and the lipid results. A descriptive analysis was performed to determine the rate of risk factors for hyperlipidemia in this population.Results:In total, 12,950 children had 38,158 lipid panels obtained. The average age at the time the lipid panel was obtained was 11.4 years and 57% (7,365) were male. Of these children, 4,441 were overweight with a weight over the 90 percentile for age and 3,389 were obese with a weight over 95 percentile. Of these children, 82% (or 30331 labs) had elevated total cholesterol over 130 and 51% (or 19299 labs) were over 160. However, of those with elevated cholesterol, 45% were overweight or obese. In addition to elevated total cholesterols, the HDL level was less than 40 in 32% (or 10,654 labs) and less than 35 in 20% (or 6,508 labs). Sub fractionation of the HDL was rarely performed.Conclusions:We are identifying a large population of patients with elevated cholesterol and low HDL cholesterol. The risk factors however may be different as there is a lower proportion of these patients who are overweight or obese than in a typical population. Further evaluation for other risk factors in the population of children with congenital heart disease is necessary.