Despite improvements in outcomes after completion of the Fontan circulation, long-term functional state varies. We sought to identify pre- and postoperative characteristics associated with overall function.

We analyzed data from 476 survivors with the Fontan circulation enrolled in the Pediatric Heart Network Fontan Cross-sectional Study. Mean age at creation of the Fontan circulation was 3.4 plus or minus 2.1 years, with a range from 0.7 to 17.5 years, and time since completion was 8.7 plus or minus 3.4 years, the range being from 1.1 to 17.3 years. We calculated a functional score for the survivors by averaging the percentile ranks of ventricular ejection fraction, maximal consumption of oxygen, the physical summary score for the Child Health Questionnaire, and a function of brain natriuretic peptide. The mean calculated score was 49.5 plus or minus 17.3, with a range from 3 to 87. After adjustment for time since completion of the circulation, we found that a lower score, and hence worse functional state, was associated with: right ventricular morphology (p less than 0.001), higher ventricular end-diastolic pressure (p equals 0.003) and lower saturations of oxygen (p equals 0.047) prior to completion of the Fontan circulation, lower income for the caregiver (p equals 0.003), and, in subjects without a prior superior cavopulmonary anastomosis, arrhythmias after completion of the circulation (p equals 0.003). The model explained almost one-fifth (18%) of the variation in the calculated scores. The score was not associated with surgical centre, sex, age, weight, fenestration, or the period of stay in hospital after completion of the Fontan circuit. A validation model, using 71 subjects randomly excluded from initial analysis, weakly correlated (R equals 0.17, p equals 0.16) with the score calculated from the dataset.

Right ventricular morphology, higher ventricular end-diastolic pressure and lower saturations of oxygen prior to completion of the Fontan circuit, lower income for the provider of care, and arrhythmias after creation of the circuit, are all associated with a worse functional state. Unmeasured factors also influence outcomes.

To investigate the effect of bosentan in patients with a failing Fontan circulation.

A multicentric open label, non-controlled study.

5 tertiary care centres for congenital cardiology.

We included 10 patients with a failing Fontan circulation. Their median age at inclusion was 12.12 years, with a range from 4.41 to 33,41 years. The median interval between the Fontan operation and inclusion was 7.84 years, with a range from 1.96 to 12,18 years. Participants received half the usual dose of bosentan for 4 weeks, and then the full dose for a further 12 weeks.

We assessed saturations of oxygen at rest and during exercise, using a 6 minutes walk test, at baseline, and during and after 16 weeks of treatment. At each visit, we assessed blood chemistry and hepatic function, and asked the patients to complete a questionnaire concerning quality of life. All medical events and possible side effects were recorded.

Of the cohort, 1 patient withdrew. The changes in saturations of oxygen, exercise performance, and scores for the questionnaire did not reach statistical significance for the whole group. We noted, nonetheless, that saturations of oxygen and/or exercise capacity improved in 5 of the patients. This was further confirmed when those patients deteriorated again when the drug was discontinued.

Our study failed to show significant improvement after 3 months of treatment with bosentan in a small group of patients with failing Fontan circulations. Some individuals, nonetheless, did improve. When planning larger trials, it would be better to identify those patients who might potentially benefit from the treatment prior to commencing the trial.

In this study we sought to determine, first, whether maximal exercise capacity reflects ventricular function, and second, whether the age of the patient, and the age of completion of the Fontan circulation, influence ventricular function and exercise performance.

Cardiac magnetic resonance imaging and cardiopulmonary exercise testing were performed in 29 patients at a median time of 6.9 years after completion of the Fontan circulation. We divided the patients into 2 groups, the first 19 having their operation below the age of 18 years, and the second group, of 10 patients, having completion of the Fontan circulation when they were older than 18 years. Parameters for ventricular function and exercise were compared for both groups with controls.

Compared to controls, the younger patients had normal end-diastolic ventricular volumes, but significantly impaired ventricular function, lower maximal work load and consumption of oxygen. The older patients had greater end-diastolic ventricular volumes, and significantly poorer ventricular function than both the younger patients and the controls. Maximal work load and consumption of oxygen were significantly lower in the older patients than in the younger ones and the controls.

Patients with the Fontan circulation have an impaired systolic ventricular function, which correlates with maximal exercise capacity and uptake of oxygen. Those having completion of the Fontan circulation when younger than 18 years had significantly better ventricular function and exercise performance than those who had completion of the Fontan circulation at an older age. An early creation of the Fontan circulation may preserve cardiac function and exercise capacity.

To describe the pattern of prescribing for palivizumab in the Glasgow area over the period 1999 through 2007, and to compare recent prescribing to the current recommendations by the Joint Committee on Vaccination and Immunisation of the United Kingdom Department of Health. Our secondary objective was to describe admissions to paediatric intensive care in patients with respiratory syncytial virus receiving palivizumab.

Tertiary children’s hospital out-patient immunisation clinic and paediatric intensive care unit.

Prospective analysis of prescribing and admissions data for the period 1999 through 2007.

Number of prescriptions and admissions to the paediatric intensive care unit.

The number of children receiving palivizumab annually initially rose more than 5-fold, from 17 in the season of 1999 and 2000 versus 115 in 2004 and 2005, although it has declined in the past 2 years, to 63 in 2006 and 2007, following publication of the recommendations of the Joint Committee on Vaccination and Immunisation established by the United Kingdom Department of Health. There has been no significant change in demographics of patients during this period. Prior to publication of these recommendations, 35 of 44 (80%) patients with congenitally malformed hearts who received palivizumab in the season of 2005 and 2006 deviated from the current recommendations, compared to 5 of 51 (10%) who received palivizumab for non-cardiac indications. No patients who received palivizumab required admission to the paediatric intensive care unit with proven respiratory syncytial virus infection over the 8 year period.

The number of children receiving palivizumab initially increased significantly, although it has now fallen following implementation of national recommendations. Much prescribing, particularly for children with congenitally malformed hearts, did not fulfil current recommendations. The absence of admissions to paediatric intensive care reflects the success of targeted immunisation in our population.

Florida is the fourth largest state in the United States of America. In 2004, 218,045 live babies were born in Florida, accounting for approximately 1744 new cases of congenital heart disease. We review the initial experience of The Society of Thoracic Surgeons Congenital Heart Surgery Database with a regional outcomes report, namely the Society of Thoracic Surgeons Florida Regional Report.

Eight centres in Florida provide services for congenital cardiac surgery. The Children’s Medical Services of Florida provide a framework for quality improvement collaboration between centres. All congenital cardiac surgical centres in Florida have voluntarily agreed to submit data to the Society of Thoracic Surgeons Database. The Society of Thoracic Surgeons and Duke Clinical Research Institute prepared a Florida Regional Report to allow detailed regional analysis of outcomes for congenital cardiac surgery.

The report of 2007 from the Society of Thoracic Surgeons Congenital Heart Surgery Database includes details of 61,014 operations performed during the 4 year data harvest window, which extended from 2003 through 2006. Of these operations, 6,385 (10.5%) were performed in Florida. Discharge mortality in the data from Florida overall, and from each Florida site, with 95% confidence intervals, is not different from cumulative data from the entire Society of Thoracic Surgeons Database, both for all patients and for patients stratified by complexity.

A regional consortium of congenital heart surgery centres in Florida under the framework of the Children’s Medical Services has allowed for inter-institutional collaboration with the goal of quality improvement. This experience demonstrates, first, that the database maintained by the Society of Thoracic Surgeons can provide the framework for regional analysis of outcomes, and second, that voluntary regional collaborative efforts permit the pooling of data for such analysis.

We report the experience in a single institution with balloon aortic valvoplasty for congenital aortic stenosis. Unlike most other reported series, we included patients with associated lesions involving the left side of the heart.

Between November, 1986, and November, 2006, we performed 161 interventions on 143 patients, of whom 33 were neonates, 33 infants, and 77 children, just over one-quarter (28.6%) having associated lesions.

The overall reduction in peak-to-peak gradient of 60 ± 24% (p < 0.01), was more effective in primary versus secondary intervention (63 ± 24% versus 47 ± 23%; p < 0.03), and in those with fused bifoliate as opposed to truly bifoliate valves (66 ± 17% versus 53 ± 30%; p = 0.01). Patients with associated lesions were younger (40.89 ± 60.92 months versus 81.9 ± 72.9 months; p = 0.001), and were less likely to achieve a final pressure gradient of less than 20 mmHg (35.0% versus 61.2%; p < 0.01). Overall mortality was higher in cases with associated lesions (27.5% versus 1.9%; p < 0.0001) but not catheter-related death (2.5% versus 1.9%; p = 1.0). Reintervention was more frequently required in infants (p = 0.02) but not in cases with associated lesions (p = 0.35).

Balloon valvoplasty is a safe and effective method for the treatment of congenital aortic stenosis. Prior surgery to the aortic valve, reintervention, associated cardiovascular lesions, and the anatomy of the valve predict a less effective reduction in the gradient. Major complications and catheterization-related death are mainly secondary to very young age, but not to associated cardiac lesions.