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Familial hypercholesterolemia (FH) is a prevalent and underrecognized disorder. A young girl with previously undiagnosed homozygous FH presented with acute coronary syndrome. Severe coronary ostial stenosis and severe supravalvular aortic stenosis from atheromatous plaque was discovered. This case highlights the importance of screening and timely diagnosis of FH.

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BACKGROUND: This is the first study to report on the impact of race on differences in the prevalence of echocardiographic left ventricular hypertrophy and left ventricular adaptation at the time of diagnosis of essential hypertension in children. METHODS: This cross-sectional, single-centre study included patients aged 3-18 years who had newly diagnosed essential hypertension. Echocardiography was used to assess left ventricular mass index and left ventricular relative wall thickness. An left ventricular mass index > the 95th percentile for age and gender, and an left ventricular relative wall thickness > 0.42, were used to diagnose left ventricular hypertrophy and concentric adaptation. Various echocardiographic parameters were compared between African Americans and Caucasians. RESULTS: The study included 422 patients (289 African Americans and 133 Caucasians) diagnosed with essential hypertension at a median age of 14.6 (interquartile range; 12.1-16.3) years. Eighty-eight patients (20.9%) had left ventricular hypertrophy. There was no statistically significant difference in the prevalence of left ventricular hypertrophy between African Americans and Caucasians (22.5% versus 17.3%, p=0.22). The median left ventricular relative wall thickness was 0.35 (0.29-0.43), and 114 patients (27.0%) had an left ventricular relative wall thickness > 0.42. The presence of an left ventricular relative wall thickness > 0.42 was significantly higher among African Americans compared to Caucasians (30.1% versus 20.3%, p = 0.04). The African American race was a strong predictor for an left ventricular relative wall thickness > 0.42 (odds ratio 1.7, p = 0.04), but not for left ventricular mass index > the 95th percentile (p = 0.22). Overweight/obesity was a strong predictor for an left ventricular mass index > the 95th percentile. CONCLUSIONS: There was no difference in the prevalence of left ventricular hypertrophy in children with essential hypertension of different races. Obesity, rather than being African American, is associated with left ventricular hypertrophy.

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Obesity is a modifiable, independent risk factor for adverse long-term outcomes in children and adults. Our objective was to determine the prevalence of overweight and obese status in a cohort of adults with congenital heart disease (CHD) as well as to assess longitudinal trends over a 20-year period. The study group consisted of patients 18 years of age and older followed at our adult CHD clinic. Body mass index (BMI) data were collected from our index period, consisting of patient encounters from 2009 to 2012 (Period 2), as well as during 2000-2003 (Period 1) and 2017-2020 (Period 3) when available. The study cohort was subdivided into three groups per published guidelines: simple, moderate, and greater CHD complexity. The prevalence of obesity and overweight status was compared among the different groups as well as with published data (NHANES). Our cohort in Period 2 consisted of 261 subjects. The median age (25-75% interquartile range) for Period 2 was 27.6 (21.1-35.9) years and BMI was 25.2 (21.7-30.0) kg/m2 with 8.0% underweight, 40.0% with normal weight, 27.0% overweight, and 25% obese. 95 patients had follow-up data from each time period, with 96% of patients having moderate or greater complexity of CHD. The combined percentage of overweight and obese patients for the moderate and greater complex CHD groups increased from 42 and 37% in period 1 to 60% and 65% in period 3, respectively. The percentage of obese patients with moderate and greater CHD complexity increased by 250% and 55%, respectively, from Period 1 to 3. Our study cohort had a high prevalence of overweight and obese weight status. Given adults with CHD have high baseline cardiovascular morbidity, the presence of obesity can increase their risk for poor outcomes, highlighting the need for prevention of this modifiable risk factor.

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Reduced exercise capacity and restrictive lung physiology are common in patients after Fontan palliation (FP). However, there is paucity of data regarding the association between specific spirometry patterns and key exercise parameters in this population. This is a single-center, cross-sectional, study correlating pulmonary function and exercise parameters in children with FP. Patients who were ≤ 18 years of age and underwent a comprehensive cardiopulmonary treadmill exercise stress test (CPT) and spirometry at the same time, were included. Patients were categorized as (i) normal or (ii) abnormal based on the results of spirometry. The abnormal group was subdivided into (a) restrictive, (b) obstructive, and (c) mixed patterns. Demographic and key exercise parameters were compared between groups. Our study included 82 patients who underwent CPT at 13.6 (IQR, 11.3-15.4) years of age. A reduced exercise capacity (%VO2 ≤ 85%) was noted in the majority (n = 50, 61%). Spirometry was abnormal in 47 (57%) patients [restrictive (n = 25, 30%), obstructive (n = 12, 15%), and mixed (n = 10, 12%)]. The abnormal spirometry group had significant lower %VO2 (77% vs. 92%, p = 0.01) and METS (8.4 vs. 9.6, p = 0.02). Subgroup analysis revealed that obstructive (p = 0.04) and mixed (p = 0.02) patterns were associated with a significant decrease in % VO2. Majority of the children demonstrated an abnormal spirometry pattern post-FP. Abnormal pulmonary function was associated with the reduced exercise capacity. Identification and treatment of the abnormal lung function may improve the exercise capacity in these patients and improve the morbidity.

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Left ventricular outflow tract obstruction in patients with pulmonary atresia with intact ventricular septum has been rarely reported. Data are lacking on the impact and management of systemic ventricular outflow tract obstruction that developed following the Fontan procedure. We report a case of an 8-year-old male who developed left ventricular outflow tract obstruction 6 months after the Fontan procedure.

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Conduit stenosis is a major, albeit rare, complication following the Fontan palliation. A single-baffle conduit with polytetrafluoroethylene is widely used for an extracardiac type Fontan palliation. A polyethylene terephthalate conduit (Dacron) is sometimes used for the conduit when more flexibility is required. A Y-shaped conduit is rarely used, but it may reduce the energy loss and achieve better hepatic flow distribution. Data on the long-term patency and complications when using a Y-shaped Dacron conduit is lacking. We report a case of a severely stenotic Y-shaped Dacron conduit in a patient who underwent extracardiac Fontan palliation.

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BACKGROUND: Obesity is a modifiable, independent risk factor for mortality and morbidity after cardiovascular surgery in adults. Our objective was to evaluate the impact of obesity on short-term outcomes in adolescents undergoing surgery for congenital heart disease (CHD). METHODS: This retrospective chart review included patients 10-18 years of age who underwent CHD surgery. Our exclusion criteria were patients with a known genetic syndrome, heart transplantation, and patients with incomplete medical records. The clinical data collected included baseline demographics and multiple perioperative variables. Charting the body mass index in the Centers for Disease Control and Prevention growth curves, the entire cohort was divided into three categories: obese (>95th percentile), overweight (85th-95th percentile), and normal weight (<85th percentile). The composite outcome included survival, arrhythmias, surgical wound infection, acute neurologic injury, and acute kidney injury. RESULTS: The study cohort (n = 149) had a mean standard deviation (SD), body mass index (BMI) of 22.6 ± 6.5 g/m2, and 65% were male. There were 27 obese (18.1%), 24 overweight (16.1%), and 98 normal weight (65.8%) patients. Twenty-seven (18%) patients had composite adverse outcomes. Overweight and obese patients had significantly higher adverse outcomes compared with normal weight patients (odds ratio (OR): 2.9; confidence interval (CI): 1-8.5, p = 0.04 and OR: 3; CI: 1-8.5, p = 0.03, respectively). In multivariate analysis, obesity was an independent predictor of adverse outcome in our cohort (p = 0.04). CONCLUSIONS: Obesity is associated with short-term adverse outcome and increased health resource utilisation in adolescents following surgery for CHD. Further studies should evaluate if intervention in the preoperative period can improve outcomes in this population.

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We report the case of a premature newborn diagnosed with coarctation of the aorta after spontaneous closure of ductus arteriosus who was successfully managed with prostaglandin E1 infusion until surgical repair could be performed. This case, together with a review of the literature, suggests an important role for prostaglandin in the management of coarctation even in the absence of a patent ductus arteriosus. The putative mechanism for the utility of prostaglandin infusion is that it may relieve the obstruction in neonates with severe coarctation by not only opening of the ductus but, in select cases, relaxing the ductal tissue encircling the aortic isthmus region. We also found a possible dose dependence of the efficacy of the prostaglandin infusion when the ductus is closed.

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The selection criteria, safety, and efficacy of bariatric surgery are well established in adults but are less well defined for severely obese adolescents. The number of severely obese adolescents who could benefit from weight loss surgery is increasing, although referral rates have plateaued. Surgical options for these adolescents are controversial and raise several questions. Recent studies, including the prospective Teen-Longitudinal Assessment of Bariatric Surgery Study and the Adolescent Morbid Obesity Surgery Study, help answer these questions. Early bariatric surgical intervention improves body mass index but, more importantly, improves cardiovascular and metabolic co-morbidities of severe obesity. A review of the medical, psychosocial, and economic risks and benefits of bariatric surgery in severely obese adolescents is a step toward improving the management of a challenging and increasing population. We describe the current knowledge of eligibility criteria, preoperative evaluation, surgical options, outcomes, and referral barriers of adolescents for bariatric surgery.

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Obesity in the child and adolescent population is a growing problem. This article discusses the identification of obesity and overweight youth using body mass index and anthropometry and reviews literature to show the relationship between obesity and premature cardiovascular disease.

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Anomalous origin of the left main coronary artery from the right sinus (ALMCA) is a rare entity associated with an increased risk for sudden cardiac death. Although transthoracic echocardiography often is the primary screening tool for the diagnosis, its accuracy remains undefined, resulting in the use of more expensive methods such as cardiac catheterization and magnetic resonance imaging when clinical suspicion exists. This report aims to describe a novel echocardiographic screening method for detecting ALMCA and to apply it retrospectively for patients with confirmed findings intraoperatively and/or on autopsy. All patients with a confirmed diagnosis of ALMCA who presented to our institution from 2004 to 2012 were included in the study. Eight patients had adequate images, including two patients with an initial diagnosis of normal coronary origins. A control group consisted of 35 patients with normal coronaries shown by echocardiogram. Five of these patients had normal coronary artery origins confirmed by magnetic resonance imaging (MRI). Echocardiographic images were reviewed, and the left main coronary was identified in the parasternal long-axis view between the aorta and pulmonary trunk. The angle of the left main coronary course was measured. In patients with ALMCA, the proximal course of the vessel was steeper as it coursed posteriorly. In contrast, the course was almost horizontal in patients with normal coronary origins. Based on these findings, a cutoff angle of 28° is proposed. In the parasternal long-axis view, ALMCA can be identified by its anomalous proximal course. This screening method is reliable and increases the accuracy of transthoracic echocardiograms.

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Congenital arteriovenous malformations are a well-described cause of neonatal heart failure. Fistulous connections are typically intrahepatic or intracranial. We present a case of a neonate with an intrathoracic arteriovenous malformation between the subclavian artery and superior vena cava resulting in florid neonatal heart failure. This unusual fistulous connection has only rarely been reported in the literature, and in those reports, it has not resulted in neonatal heart failure.

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