Youth onset type 1 diabetes (T1D) and type 2 diabetes (T2D) is increasing and associated with earlier vascular complications and mortality. Dyslipidemia is an important modifiable cardiovascular (CVD) risk factor that is under-recognized and undertreated in youth with T1D and T2D. Given this, we evaluated the prevalence and associations between lipid concentrations and clinical CVD risk factors in youth with T1D compared to T2D at our large ethnically diverse diabetes center. A retrospective chart review was performed, evaluating patients with T1D or T2D seen at least once in clinic from 2015 to 2017, age 10–22 years of age, duration of diabetes at least 6 months on the date of most recent LDL-cholesterol (LDL-C) concentration, and not on statin therapy. We performed independent and multivariable linear regressions of LDL-C and HDL-cholesterol (HDL-C) concentrations. There were 32.7% with T1D (n = 1701) and 47.7% with T2D (n = 298) with LDL-C above recommend goal (> 100 mg/dL/2.6 mmol/L). Furthermore, there were 9% with T1D and 16.4% with T2D with LDL > 130 mg/dL (> 3.4 mmol/L), who likely met criteria for starting statin therapy. Higher LDL-C and/or lower HDL-C were associated with increased age, diabetes duration, higher HbA1C, female sex, Hispanic ethnicity, obesity, and T2D. After adjusting for these risk factors in a multivariable linear regression model, the association of higher LDL-C and lower HDL-C was higher with T2D than T1D. This highlights the need for more aggressive dyslipidemia screening and treatment in youth with diabetes, especially T2D. At our institution we have created and instituted quality improvement algorithms to try to address this need.

Children with homozygous familial hypercholesterolemia are at risk for early cardiovascular events secondary to coronary artery disease. Current medical therapy does not ameliorate this risk. Liver transplantation offers the most effective option to reduce circulating levels of low-density lipoprotein cholesterol and thereby reduce risk of cardiovascular events. Angiographic evidence of regression of coronary artery disease is presented.

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.

OBJECTIVE:Studies have shown improvement in lipid levels after institution of lifestyle changes in children enrolled in closely monitored programs. There programs are difficult to mimic in real world clinics. We aim to determine if diet and exercise result in improvement in lipid levels in patients seen in a designated lipid clinic in a real life setting.DESIGN:Retrospective review of patients followed for dyslipidemia at the Texas Children's Hospital Lipid Clinic from May 1, 2012 to May 1, 2015. Patients included were seen more than once, had repeat lipid testing, and abnormal baseline lipid levels. Multivariate analysis using mixed models were performed to compare outcomes in patients who did and did not participate in lifestyle change.RESULTS:Of the 268 patients seen within the study period, 174 (56% male, 44% female) met inclusion criteria. Median age was 11 years. Compared to patients who did not make lifestyle changes: patients who made only diet changes demonstrated significant improvement in weight only (slope = -1.55, P-value = .014), and those who made only exercise changes demonstrated significant improvements in serum cholesterol (slope = -22.8, P-value = .017) and non-HDL cholesterol (slope = -28.7, P-value = < .01) levels. Patients who participated in both diet and exercise demonstrated significant improvement in weight (slope = -1.13, P-value = .011), diastolic blood pressure (slope = -1.82, P-value = < .01), and serum lipid levels: LDL (slope = -10.8, P-value = 0.017), HDL (slope = 1.52, P-value = .24), Triglycerides (slope = -0.11, P-value = .033) compared to those who did not make lifestyle changes.CONCLUSIONS:Outpatient management of dyslipidemia is difficult. Only patients who participated in both diet and exercise showed significant improvement in outcomes when compared to those who did not make lifestyle changes.

Background: Hypertension (HTN) is becoming a more recognized problem in pediatrics in general, though the prevalence is not changing despite the increasing prevalence of obesity.(1) The prevalence of borderline high and high blood pressure in children and adolescents appears to have plateaued at 11-12%. (1) Annual blood pressure measurement in patients being seen in pediatric practices has long been recommended. Our goal is to evaluate whether or not patients with HTN seen in a pediatric cardiology practice were appropriately diagnosed with HTN. Methods: A retrospective analysis of patients seen in the Texas Children’s Hospital Pediatric Cardiology outpatient clinics between 2005 and 2015 was performed. All blood pressure measurements, heights, weights, and BMI obtained at the same clinic visit were obtained from the electronic medical record. Patients with missing data were excluded from the analysis The data was analyzed using SAS with calculations performed of means, standard deviations and interquartile ranges. Chi-squre and t-tests were used to compare means. Results: A total of 57,670 patients (53% male, mean age 8.7 +/- 8.3) were seen in clinic. The average BMI was 19 (range 0 to 95). There were a total of 125,750 visits, with a average number of visits 3.9 (range 1-64). 33,765 patients had blood pressure measurements in the pre-HTN or HTN range and 9,149 patients had Stage 2 HTN measured at the time of clinic visit. Most children (35,220, 61%) had only one visit and were therefore unable to truly be diagnosed with HTN. Of the remaining 22,450 patients, 3,751 (15%) patients had hypertensive blood pressure measurements on at least three occasions and 977 (26%) of those were diagnosed with HTN. The diagnosis of HTN was made in younger patients (p<0.001). Of those with the diagnosis of HTN, 691 (70%) were on antihypertensive medication. Younger patients were more likely to be treated for HTN than not, 10.3 +/- 11.7 years and 16.2 +/- 16.4 years respectively (p<0.001). Additionally proportionally females were more likely to be diagnosed with (p<0.001) and treated for HTN (p<0.001) than males. Conclusions: HTN is a common problem in pediatrics. In a pediatric cardiology practice, many patients have HTN that is undiagnosed. If HTN is diagnosed it is frequently either untreated or inadequately treated. While evaluation of blood pressure is being performed and documented in most patients adequate diagnosis and treatment is lacking.

Studies have shown improvement in lipid levels after institution of lifestyle changes in children enrolled in closely monitored outpatient settings, but it is difficult to mimic this in real world clinics. We aim to determine if diet and exercise result in improvement in lipid levels in patients

Advancements in paediatric heart failure management have resulted in improved survival and a focus on long-term outcomes including health-related quality of life. We compared health-related quality of life in children with heart failure with healthy patients, children with chronic conditions, and children with cardiovascular disease. Families (n=63) and children (n=73) aged 2-20 years with heart failure were enrolled and compared with data previously published for healthy patients (n=5480), those with chronic conditions (n=247), and those with cardiovascular disease (n=347). Patients and parents completed the PedsQL 4.0 and the Cardiac 3.0 Module health-related quality-of-life questionnaires. PedsQL scores including Total, Psychosocial Health Summary, and Physical were compared between groups. In general, patients with heart failure had lower scores than the healthy population (p=0.001), and comparable scores with those with chronic conditions. Parents perceived no difference in physical scores for children with heart failure when compared with healthy children, and perceived higher scores for children with heart failure when compared with those with chronic conditions (p⩽0.003). Furthermore, children with heart failure had decremental health-related quality-of-life scores as the American Heart Association stage of heart failure increased, such that patients with stage C heart failure had scores similar to children with severe cardiovascular disease. Children with heart failure reported significantly impaired health-related quality of life compared with healthy children and similar scores compared with children with chronic conditions. Parental perceptions appear to underestimate these impairments. Children with heart failure appear to have progressive impairment of health-related quality of life with advancing stage of heart failure.

Purpose: The purpose of this study was to engage staff nurses in research in an informative and fun way by determining nurses' preference for sugar-free chocolate chip cookies versus regular chocolate chip cookies.Design and Methods: A descriptive, crossover, double-blinded study was performed using a convenience sample of 300 staff nurses.Results: Nurses preferred the texture, flavor, and overall preference of the regular chocolate chip cookie as compared with the sugar-free chocolate chip cookie (p < 0.001).Implications: The Great American Cookie Experiment remains a creative teaching strategy to enhance knowledge and engage nurses in the research process. (C) 2015 Elsevier Inc. All rights reserved.

Congenital Heart DiseaseVolume 10, Issue 6 p. 520-528 STATE OF THE ART ARTICLE Liver Transplantation for HoFH in Children: Single Center Experience Constance E. Cephus PhD, CPNP, Corresponding Author Constance E. Cephus PhD, CPNP Baylor College of Medicine, Houston, Tex, USA Texas Children's Hospital, Houston, Tex, USACorresponding Author: Constance E. Cephus, PhD, CPNP Department of Pediatrics, Cardiology, Baylor College of Medicine, Texas Children's Hospital, 6621 Fannin Street, Houston, TX 77030, USA. Tel: 832-826-5710, Fax: 832-825-1107; E-mail: Cephus@bcm.eduSearch for more papers by this authorAthar M. Qureshi MD, Athar M. Qureshi MD Baylor College of Medicine, Houston, Tex, USA Texas Children's Hospital, Houston, Tex, USASearch for more papers by this authorS. Kristen Sexson-Tejtel MD, PhD, S. Kristen Sexson-Tejtel MD, PhD Baylor College of Medicine, Houston, Tex, USA Texas Children's Hospital, Houston, Tex, USASearch for more papers by this authorJohn A. Goss MD, John A. Goss MD Baylor College of Medicine, Houston, Tex, USA Texas Children's Hospital, Houston, Tex, USASearch for more papers by this authorDouglas S. Moodie MD, Douglas S. Moodie MD Baylor College of Medicine, Houston, Tex, USA Texas Children's Hospital, Houston, Tex, USASearch for more papers by this author Constance E. Cephus PhD, CPNP, Corresponding Author Constance E. Cephus PhD, CPNP Baylor College of Medicine, Houston, Tex, USA Texas Children's Hospital, Houston, Tex, USACorresponding Author: Constance E. Cephus, PhD, CPNP Department of Pediatrics, Cardiology, Baylor College of Medicine, Texas Children's Hospital, 6621 Fannin Street, Houston, TX 77030, USA. Tel: 832-826-5710, Fax: 832-825-1107; E-mail: Cephus@bcm.eduSearch for more papers by this authorAthar M. Qureshi MD, Athar M. Qureshi MD Baylor College of Medicine, Houston, Tex, USA Texas Children's Hospital, Houston, Tex, USASearch for more papers by this authorS. Kristen Sexson-Tejtel MD, PhD, S. Kristen Sexson-Tejtel MD, PhD Baylor College of Medicine, Houston, Tex, USA Texas Children's Hospital, Houston, Tex, USASearch for more papers by this authorJohn A. Goss MD, John A. Goss MD Baylor College of Medicine, Houston, Tex, USA Texas Children's Hospital, Houston, Tex, USASearch for more papers by this authorDouglas S. Moodie MD, Douglas S. Moodie MD Baylor College of Medicine, Houston, Tex, USA Texas Children's Hospital, Houston, Tex, USASearch for more papers by this author First published: 11 November 2015 https://doi.org/10.1111/chd.12301Citations: 1Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat Citing Literature Volume10, Issue6November/December 2015Pages 520-528 RelatedInformation

Objectives/Hypothesis: To study the incidence and implications of vocal fold paresis (VFP) following congenital neonatal cardiac surgery. Study Design: Retrospective chart review. Methods: All neonates who underwent median sternotomy for cardiac surgery from May 2007 to May 2008 were evaluated. Flexible laryngoscopy was performed to evaluate vocal fold function after extubation. Swallow evaluation and a modified barium swallow study were performed prior to initiating oral feeding if the initial screening was abnormal. Results: A total of 101 neonates underwent cardiac surgery during the study period. Ninety-four patients underwent a median sternotomy, and 76 of these were included in the study. Fifteen (19.7%) had vocal fold paresis (VFP) postoperatively. Almost 27% of the patients with aortic arch surgery had VFP while only 4.1% of the patients with nonaortic arch surgery developed VFP (P=0.02) Those patients who underwent aortic arch surgery weighed significantly less (P<0.01). All the patients with VFP had significant morbidity related to swallowing and nutrition (P=0.01) and required longer postsurgical hospitalization (P=0.02). Conclusions: The reported incidence of VFP following cardiac surgery via median sternotomy ranges between 1.7% and 67% depending on the type of surgery and the weight of the infant at the time of surgery. In our cohort, 19.7% had VFP. Surgery requiring aortic arch manipulation had a higher incidence of complications and required longer hospitalizations. These results may be used to improve informed consent and to manage postoperative expectations by identifying patients who are at higher risk for complications.

Objective: There has been considerable improvement in survival after the first stage of palliation for single-ventricle heart disease. Yet, interstage mortality continues to plague this population. Home monitoring has been proposed to reduce interstage mortality. We review our experience after creation of a Single Ventricle Program.Methods: All infants with a single ventricle heart defect who were admitted to Texas Children's Hospital from the inception of the Single Ventricle Program on September 1, 2007, to January 1, 2010, were included in the Single Ventricle Program cohort. Infants with a single ventricle presenting between January 1, 2002, and August 31, 2007, comprised the pre-Single Ventricle Program group. Anatomic, operative, and postoperative details were noted for all patients. End points included in-hospital death after the first stage of palliation, interstage death (defined as after discharge from the first stage of palliation and before the second stage of palliation), and death or heart transplantation by 1 year of age. Interstage weight gain was also compared.Results: A total of 137 infants with a single ventricle were included in the pre-Single Ventricle Program cohort, and 93 infants were included in the Single Ventricle Program cohort. Anatomic subtypes were similar between groups. There was significant improvement in rate of interstage weight gain, whereas age at the second stage of palliation was significantly reduced in the Single Ventricle Program group. In-house mortality decreased during the Single Ventricle Program era (P = .021). Interstage mortality did not significantly decrease in the Single Ventricle Program group. However, 1-year transplant-free survival improved during the Single Ventricle Program era (P = .002).Conclusions: The Single Ventricle Program improved interstage weight gain, thereby allowing for early second-stage palliation at an equivalent patient weight. Interstage mortality was not significantly reduced by our program. However, 1-year transplant-free survival was significantly improved in patients in the Single Ventricle Program. (J Thorac Cardiovasc Surg 2011;142:1358-66)

Objective; We present a case of trauma-induced complete atrioventricular block (transient) after placement of a central venous catheter.Design: Case report.Setting: Neonatal intensive care unit in a tertiary care children's hospital.Patient: Review of the medical record and clinical course of a single premature infant.Interventions: Removal of central venous catheter, monitoring of the cardiac output exam, and serial monitoring of the arrhythmia profile.Measurements and Main Results; Trauma-induced complete atrioventricular block from placement of the central venous catheter resolved in 9 days, obviating the need for pacemaker placement.Conclusions; Complete atrioventricular block is an infrequent complication of central venous catheter placement and may require several days to resolve.