Coronary artery disease in a child with homozygous familial hypercholesterolemia: Regression after liver transplantation.

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.

Health-related quality of life in children with heart failure as perceived by children and parents.

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.

The great American cookie experiment: engaging staff nurses in research.

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.

The impact of a dedicated single-ventricle home-monitoring program on interstage somatic growth, interstage attrition, and 1-year survival.

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.

Transient complete atrioventricular block after placement of a central venous catheter in a neonate.

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.

Moderate acute rejection detected during annual catheterization in pediatric heart transplant recipients.

BACKGROUND: Acute rejection commonly occurs within the first year after heart transplantation, and then decreases in frequency with time. Recently, the long-term utility of endomyocardial biopsy during routine annual catheterization has been questioned. The purpose of this study was to retrospectively review the prevalence of biopsy-proven rejection during routine annual catheterization in our patient population, determine whether biopsies late after transplant are useful, and identify factors that correlate with late unsuspected rejection. METHODS: Biopsy results from the annual catheterization were evaluated from 1986 to August 2000. The prevalence of moderate rejection was evaluated and compared with the patient's immunosuppressive regimen; the prevalence of late rejection; and how late rejection correlated with recipient age, number of first-year rejections and presence of sub-therapeutic cyclosporine. RESULTS: A total of 1108 biopsies were performed in 269 children with a mean follow-up of 5 +/- 3 years (median 5 years, range 1 to 11 years). Three-drug immunosuppressive therapy, including steroids, was used in 93 patients. There was a persistent 8% to 10% prevalence of moderate rejection at up to 10 years post-transplantation. Moderate rejection was more likely in patients: (1). on 3-drug immunosuppressive therapy; (2). with a recipient age >1 year; and (3). with a relatively lower cyclosporine level. CONCLUSIONS: These data suggest that continued surveillance of pediatric transplant patients for acute rejection is indicated for long-term follow-up.