Objective: To assess long term outcome of patients who underwent Mustard or Senning repair for transposition of the great arteries up to 30 years earlier.Design: Retrospective review of medical records.Setting: The six university hospitals in Belgium with paediatric cardiology departments.Patients: 339 patients were reviewed, of whom 124 underwent the Mustard procedure and 215 the Senning procedure. This represents almost the entire population of patients in Belgium with either simple or complex transposition.Main outcome measures: Mortality, morbidity, functional abilities, social integration.Results: Overall mortality was 24.2%. Early mortality (⩽ 30 days after surgery) accounted for 16.5%, late mortality for 7.7%. Actuarial survival of early survivors at 10, 20, and 30 years after surgery was 91.7%, 88.6%, and 79.3%, respectively. Patients in the Senning cohort had a slightly better survival rate than those in the Mustard cohort (NS). Baffle obstruction occurred more often after Mustard repair (15.3%) than after the Senning procedure (1.4%). Arrhythmia-free survival did not differ between the two cohorts, but was determined by the complexity of the transposition. Survivors of the Senning cohort had better functional status, and tended to engage in more sports activities.Conclusions: The long term outcome for patients surviving the Mustard or Senning operation was favourable in terms of late mortality, morbidity, functional, and social status. Overall mortality in the Senning cohort did not differ from the Mustard group, but Senning patients had better functional status, greater participation in sports activities, and fewer baffle related problems.

Aims: To determine the prevalence of sleep-disordered breathing (SDB) in a clinical sample of overweight and obese children and adolescents, and to examine the contribution of fat distribution. Methods: Consecutive subjects without chronic lung disease, neuromuscular disease, laryngomalacia, or any genetic or craniofacial syndrome were recruited. All underwent measurements of neck and waist circumference, waist-to-hip ratio, % fat mass and polysomnography. Obstructive apnoea index >1 or obstructive apnoea-hypopnoea index (OAHI) >2, further classified as mild (2(OAHI,5) or moderate-tosevere (OAHI>5), were used as diagnostic criteria for obstructive sleep apnoea (OSA). Central sleep apnoea was diagnosed when central apnoeas/hypopnoeas >10 s were present accompanied by .1 age-specific bradytachycardia and/or .1 desaturation ,89%. Subjects with desaturation (85% after central events of any duration were also diagnosed with central sleep apnoea. Primary snoring was diagnosed when: snoring was detected by microphone and normal obstructive indices and saturation. Results: 27 overweight and 64 obese subjects were included (40 boys; mean (standard deviation (SD)) age 11.2 (2.6) years). Among the obese children, 53% were normal, 11% had primary snoring, 11% had mild OSA, 8% had moderate-to-severe OSA and 17% had central sleep apnoea. Half of the patients with central sleep apnoea had desaturation ,85%. Only enlarged tonsils were predictive of moderate-to-severe OSA. On the other hand, higher levels of abdominal obesity and fat mass were associated with central sleep apnoea. Conclusion: SDB is very common in this clinical sample of overweight children. OSA is not associated with abdominal obesity. On the contrary, higher levels of abdominal obesity and fat mass are associated with central sleep apnoea.

Survival of congenital heart disease is excellent and continued to improve in the early 21st century. New therapeutic options are increasingly used. This study provides baseline data for the longitudinal follow-up of this cohort.

OBJECTIVE -This study was designed to assess whether children and adolescents with type 1 diabetes have early echocardiographic signs of subclinical cardiac dysfunction and whether sex, state of metabolic control, and diabetes duration are of influence.RESEARCH DESIGN AND METHODS -Systolic and diastolic blood pressure in supine and upright positions and echocardiographic parameters, including tissue Doppler measurements of the septal mitral annulus, were evaluated in 80 children and adolescents with stable type 1 diabetes and 52 age-and sex-matched control subjects. A possible correlation was examined for age, sex, HbA 1c , and diabetes duration with univariate and multivariate regression analysis. 58 Ϯ 8 ms) compared with female control subjects, suggesting delayed myocardial relaxation. Male diabetic patients only differed significantly from their control subjects for IVRT (66 Ϯ 9 vs. 59 Ϯ 8 ms). The measured parameters showed an expected correlation with age and BMI standard deviation scores in the control group. This correlation was significantly weaker in the diabetic population; only a weak influence was found for diabetes duration and glycosylated hemoglobin levels. RESULTSCONCLUSIONS -Young diabetic patients already have significant changes in left ventricular dimensions and myocardial relaxation, with the girls clearly being more affected. Tissue Doppler proved to have additional value in the evaluation of ventricular filling in this population. Almost no correlation was found for diabetes duration and HbA 1c with the cardiovascular changes. Diabetes Care 27:1947-1953, 2004S everal studies have established diabetes as a strong risk factor for cardiovascular morbidity and mortality, especially in women (1-4). This increased risk cannot be explained only by the high prevalence of comorbidity, such as coronary heart disease or arterial hypertension in diabetes (5). Therefore, the existence of a "diabetic" cardiomyopathy distinct from ischemic heart disease has been suggested to cause systolic or diastolic dysfunction (1,6,7). Considerable debate exists regarding the exact nature and cause of this cardiac dysfunction (8 -11). The autonomic nervous system dysfunction explains the frequently reported higher heart rate in diabetic patients compared with normal subjects and may result in changed cardiac dynamics (12,13). We previously reported that corrected QT prolongation and an increased QT dispersion are already present in children with diabetes (14), and these abnormalities have been linked to an increased mortality rate in adults (15). Adult diabetic patients without clinical heart failure are reported to have hypertrophic and noncompliant left ventricles, causing essentially diastolic dysfunction (16 -20). The association between these findings and metabolic control or diabetes duration is controversial (21,22). The aim of this study was to determine whether echocardiographic signs of diastolic or systolic dysfunction are already present in diabetic children and adolescents, a population in whom comorbid...