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BACKGROUND: Within the spectrum of congenital heart disease referred to as hypoplastic left heart syndrome (HLHS), there is variation in the morphology and function of the left ventricle which could influence outcomes after stage I Norwood palliation. OBJECTIVE: To determine if left ventricular (LV) morphology is associated with outcome after stage I Norwood palliation for HLHS. METHODS: Echocardiograms were reviewed from 108 patients who had undergone Norwood palliation at our institution over the past 11 years. Total cardiac diameter, thickness of the interventricular septum (IVS), LV area and LV myocardial area were calculated. Competing risk analysis was performed for survival to a stage II operation and to determine potential predictors. RESULTS: From the Norwood operation up to stage II operation, mortality was predicted by IVS thickness, while the absence of right ventricular (RV) dysfunction was predictive of survival to stage II operation. For the complete pathway, from Norwood to the Fontan operation, mortality was predicted by IVS, a lower RV fractional area change and the presence of significant tricuspid regurgitation. Cardiac transplantation during this period was predicted by a lower RV fractional area change (p = 0.02) and a larger LV area in diastole. CONCLUSIONS: These results indicate that LV hypertrophy and decreased RV function adversely effect survival after the Norwood operation. They suggest that LV morphology, especially septal hypertrophy, can influence outcomes in HLHS and should be considered when evaluating treatment options. [ABSTRACT FROM AUTHOR]

To define outcome and time dependence of predictors of outcome in pediatric acute hypoxemic respiratory failure, 131 patients (age range, 1 month to 18 years) were prospectively followed. Parametric models were used to describe time-related events, and competing risks analysis was performed for mortality estimates. Multiple logistic analysis was applied to describe time-related predictors of ventilation time and mortality. Overall mortality was 27%. Peak oxygenation index (OI) measured at any time point (p < 0.001, 91% reliability in bootstrapping, after inverse transformation) and Pediatric Risk of Mortality, or PRISM, score within the first 12 hours of mechanical ventilation (p < 0.001, 63% reliability in bootstrapping, after square transformation) were identified as independent predictors of mortality. Peak OI, younger age, and need for renal replacement therapy were significantly associated with a longer time to extubation. Although OI was less reliable as outcome predictor within the first 12 hours of intubation, it still predicted duration of mechanical ventilation. No clear-cut threshold of OI was identified that could accurately predict mortality. Survival was characterized by a peak rate of extubations at approximately 1 week, with a more gradual decline thereafter, whereas death appeared as a constant risk over time, which exceeded chances of survival at approximately 4 weeks. Severity of oxygenation failure at any point in time during acute hypoxemic respiratory failure correlates with duration of mechanical ventilation and mortality. This is best reflected by the OI, which shows a direct correlation to outcome in a time-independent manner. [ABSTRACT FROM AUTHOR]

Objectives: The study evaluated the additional benefit of transesophageal echocardiography (TEE) versus transthoracic echocardiography (TTE) in pediatric cases with suspected bacterial endocarditis.Background: In adult patients, TTE has a lower sensitivity and specificity than TEE for the detection of vegetations or aortic root abscess formation. Few data are available about the relative benefits of TEE over TTE in the pediatric age group.Methods: Patients were included if they had positive blood cultures for typical microorganisms and had a TTE and TEE within 14 days of each other. The patients had to meet the Duke criteria for a positive diagnosis of bacterial endocarditis. The TTE and TEE data were analyzed using the McNemar test for the significance of change.Results: Twenty-one patients fulfilled the criteria, at a median age of 9.5 years. Congenital heart disease was present in 13 patients; 4 patients were previously healthy and 4 patients had other medical problems. Nine patients had surgical confirmation of bacterial endocarditis. Fifteen patients had a positive cardiac finding, with 12 vegetations, 2 vegetations plus aortic root abscess, and 1 isolated abscess. There was excellent agreement between TTE and TEE in those cases with positive cardiac findings, with a p = 0.32, kappa 0.89. Using positive TEE cardiac findings as the gold standard, the sensitivity for TTE was 86% for all 15 events and 93% for the detection of a vegetation.Conclusions: In pediatric cases, TTE has a high degree of sensitivity for the detection of supportive evidence of endocarditis, and TEE should be reserved for patients with a poor transthoracic window. [ABSTRACT FROM AUTHOR]

Objective: To describe clinical outcomes of a paediatric population with histologically confirmed lymphocytic myocarditis.Design: A retrospective review between November 1984 and February 1998.Setting: A major paediatric tertiary care hospital.Patients: 36 patients with histologically confirmed lymphocytic myocarditis.Main Outcome Measures: Survival, cardiac transplantation, recovery of ventricular function, and persistence of dysrhythmias.Results: Freedom from death or cardiac transplantation was 86% at one month and 79% after two years. Five deaths occurred within 72 hours of admission, and one late death at 1.9 years. Extracorporeal membrane oxygenation support was used in four patients, and three patients underwent heart replacement. 34 patients were treated with intravenous corticosteroids. In the survivor/non-cardiac transplantation group (n = 29), the median follow up was 19 months (range 1.2-131.6 months), and the median period for recovery of a left ventricular ejection fraction to > 55% was 2.8 months (range 0-28 months). The mean (SD) final left ventricular ejection and shortening fractions were 66 (9)% and 34 (8)%, respectively. Two patients had residual ventricular dysfunction. No patient required antiarrhythmic treatment. All survivors reported no cardiac symptoms or restrictions in physical activity.Conclusions: Our experience documents good outcomes in paediatric patients presenting with acute heart failure secondary to acute lymphocytic myocarditis treated with immunosuppression. Excellent survival and recovery of ventricular function, with the absence of significant arrhythmias, continued cardiac medications, or restrictions in physical activity were the normal outcomes. [ABSTRACT FROM AUTHOR]

Aim: To determine the diagnostic accuracy of physical examination by office based (general practice) paediatricians in the evaluation of heart murmurs.Design: Each of 30 office based paediatricians blindly examined a random sample of children with murmurs (43% of which were pathological). Sensitivity and specificity were calculated and were related to paediatricians' characteristics.Results: Mean (SD) sensitivity was 82 (24)% with a mean specificity of 72 (24)% in differentiating pathological from innocent murmurs, with further investigations requested for 54% of assessments. The addition of a referral strategy would have increased mean sensitivity to 87 (20)% and specificity to 98 (8)%. Diagnostic accuracy was not significantly related to the paediatricians' age, education or practice characteristics, but was related to referral practices and confidence in assessment.Conclusions: Diagnostic accuracy of clinical assessment of heart murmurs by office based paediatricians is suboptimal, and educational strategies are needed to improve accuracy and reduce unnecessary referrals and misdiagnosis. [ABSTRACT FROM AUTHOR]