Non-surgical treatment of congenital left ventricle to coronary sinus fistula and Wolf-Parkinson-White.

Congenital left ventricle to coronary sinus fistula is a rare entity. We report a case of an infant with prenatal finding of left ventricle to right atrial shunt. The anatomy was defined by multi-modality imaging. Baseline electrocardiogram was notable for a Wolff-Parkinson-White pattern. He underwent successful catheter device closure of the left ventricle to coronary sinus fistula. The patient developed supraventricular tachycardia and underwent successful ablation of the accessory pathway.

Impaired myocardial deformation and ventricular vascular coupling in obese adolescents with dysglycemia.

BACKGROUND: It is unknown that dysglycemia in obese adolescents has effects on myocardial deformation that are more pronounced when compared to obesity alone. We hypothesized that obesity associated abnormal glucose tolerance (dysglycemia) would have adverse effects on two-dimensional speckle tracking echocardiography derived longitudinal, radial and circumferential strain (LS, RS, CS) compared to age and gender lean controls. We also examined if changes in deformation would be reflected in abnormal ventricular vascular coupling indices (VVI). METHODS: In a prospective cross-sectional design 39 obese adolescents (15.9 ± 1.7 years; 101.5 ± 39 kg; female - 58%) were compared to age and gender matched lean controls (15.7 ± 1.8 yrs, 60 ± 12.8 kg). Based on results from an oral glucose tolerance test (OGTT), obese adolescents were categorized as obese normoglycemic (ONG, n = 25) or obese dysglycemic (ODG, n = 14). Left ventricular (LV) global and average LS, CS, RS and strain rate were measured. LV ejection fraction and mass index were measured and VVI approximated as ratio of arterial elasticity (Ea) and end-systolic elastance (Ees). RESULTS: Adolescents with ODG had significantly (P = 0.005) impaired global LS (- 20.98% ± 2.8%) compared to controls (- 23.01% ± 2.3%). A similar (P = 0.0027) reduction was observed in average LS for adolescents with ODG (18.87% ± 2.5%) compared to controls (20.49% ± 2%). Global CS was also decreased (P = 0.03) in ODG (- 23.95%) compared to ONG (- 25.80). A similar trend was observed in average CS after multivariate regression for BMI and blood pressure. CS correlated with HbA1c in both groups (P = 0.05). VVI had a negative correlation with both LS (r = - 0.4, P = 0.025) and CS rate (r = - 0.36, P = 0.04). CONCLUSIONS: Myocardial strain and strain rate were significantly altered in obese adolescents. Unfavorable subclinical reductions in global and average CS were more pronounced in adolescents with dysglycemia compared to obese adolescents with normoglycemia and controls. These data indicate progressive worsening of subendocardial function across the spectrum of glucose tolerance. Strain rate was predictive of VVI in obese adolescents, suggesting strain rate may be a sensitive marker for cardiac remodeling in abnormal glucose homeostasis states.

A unique ALCAPA variant in a neonate.

Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly present in approximately one in 300,000 live births. Here, we present a unique ALCAPA variant identified in a neonate. The left anterior descending artery originated posterolaterally on the main pulmonary artery, and the circumflex originated separately from the distal right pulmonary artery.

Prevalence of obesity among patients with congenital and acquired heart disease.

Overweight and obesity rates have risen dramatically in the United States, with subsequent detrimental comorbidity risks. The rates for obesity among children with congenital and acquired heart disease have rarely been reported. A retrospective cross-sectional study was conducted to determine the prevalence of overweight and obesity in children with heart disease and to identify subgroups at increased risk. A total of 795 cases were identified from a chart review of patients presenting to an urban center's Pediatric Cardiology Program between 1 January and 31 December 2006. A body mass index (BMI) at the 85th percentile or higher was defined as overweight, and a BMI at the 95th percentile or higher was defined as obese. Subjects with comorbidities affecting body habitus were excluded from the study. Overall, overweight and obesity rates were similar to national data. No significant differences in overweight or obesity rates were detected between heart disease and non-heart disease groups (P = 0.50). According to multivariate analysis, Hispanic ethnicity and male gender were the only predictors of obesity. This study shows that children with heart disease are not immune to the common predictors of obesity such as gender and ethnicity and that the future care of children with heart disease should include general discussions about the risks for obesity.

A primary cardiac sarcoma with features of a desmoplastic small round cell tumor in an adolescent male.

Malignant primary cardiac tumors are very rare.Desmoplastic small round cell tumors are also rare tumors and,when present, are generally found in the abdomen. We report a case of an adolescent male who presented with chest pain,abdominal pain, and difficulty in breathing, who was found to have a primary cardiac sarcoma with several characteristic features of a desmoplastic small round cell tumor.

Deferoxamine-related ocular toxicity: incidence and outcome in a pediatric population.

PURPOSE: Deferoxamine (DFO) is a chelating agent used widely for the treatment of transfusional hemochromatosis. DFO-related ocular toxicity has been previously reported several times, and many institutions have adopted an ophthalmic screening protocol for patients treated with DFO despite little information regarding the rate of ocular toxicity. Our study aimed to determine the incidence of DFO toxicity at a major pediatric hospital that uses regular ophthalmic screening for all DFO-treated patients. METHODS: A retrospective case series of all patients treated with DFO for transfusional hemochromatosis at The Hospital for Sick Children (Toronto, Ontario, Canada) between 1995 and 2005 inclusive. RESULTS: A total of 84 patients received regular DFO treatment for transfusional hemochromatosis related to long-term hypertransfusion. A total of 421 ophthalmic screening examinations were performed (average, 5.0 examinations per patient). DFO-related ocular toxicity was found only in one patient (1.2%). This patient had central blurriness and retinal pigmentary changes shown by examination and decreased central responses shown by electroretinography, but these changes were all found to be completely reversible after a change from intravenous to subcutaneous therapy at a reduced dose. CONCLUSIONS: In this large pediatric center, DFO-related ocular toxicity has been a rare and mild finding. Regular ophthalmic screening should be carried out for patients receiving high-dose subcutaneous or intravenous therapy, because early detection of retinal toxicity may lead to optimization of the DFO dose and thus prevention of long-term visual sequelae.