Recovery of heart function in children with acute severe heart failure.

BACKGROUND: The prognosis of acute heart failure is such that many children are considered for transplantation. Recovery of severe heart failure in a proportion of patients diagnosed with either dilated cardiomyopathy or myocarditis is well recognized, and this complicates the assessment for transplantation. There is little data on the time scale of recovery of heart function in children. OBJECTIVES: To describe the time course over which echocardiographic improvement of systolic function occurred in a cohort of children who presented in acute heart failure, without structural or metabolic abnormality. METHODS: Children with a first presentation of acute severe heart failure between 1990 and 2005. Time from presentation to the echocardiogram before left ventricular fractional shortening (FS) improved to 20% and 30% (complete recovery) was recorded. RESULTS: Twenty-seven children (11 male) were identified, and all had an initial FS <15%. Twenty-one patients required intravenous inotropes and three patients required extracorporeal membrane oxygenation. Seven patients had been on the active transplant list for a mean duration of 155 days. Four patients had probable viral myocarditis. Mean age at presentation was 15.7 (range, 0.1-72) months. Mean time to an FS of 20% was 3.6 (0.2-18) months and to 30% was 8.9 (0.7-24) months. Complete recovery occurred within 6, 9, 12, 18, and 24 months of presentation in 44%, 55%, 66%, and 96%, respectively. There was no correlation between age of presentation and length of time to recovery. CONCLUSIONS: Complete recovery of left ventricular systolic function is often delayed to more than 1 year from presentation. This may have major implications for timing of transplantation in an era where prolonged mechanical cardiac support is feasible even in infants.

Computer calculated dose in paediatric prescribing.

BACKGROUND AND OBJECTIVE: Medication errors are an important cause of hospital-based morbidity and mortality. However, only a few medication error studies have been conducted in children. These have mainly quantified errors in the inpatient setting; there is very little data available on paediatric outpatient and emergency department medication errors and none on discharge medication. This deficiency is of concern because medication errors are more common in children and it has been suggested that the risk of an adverse drug event as a consequence of a medication error is higher in children than in adults. OBJECTIVE: The aims of this study were to assess the rate of medication errors in predominantly ambulatory paediatric patients and the effect of computer calculated doses on medication error rates of two commonly prescribed drugs. METHODS: This was a prospective cohort study performed in a paediatric unit in a university teaching hospital between March 2003 and August 2003. The hospital's existing computer clinical decision support system was modified so that doctors could choose the traditional prescription method or the enhanced method of computer calculated dose when prescribing paracetamol (acetaminophen) or promethazine. All prescriptions issued to children (<16 years of age) at the outpatient clinic, emergency department and at discharge from the inpatient service were analysed. A medication error was defined as to have occurred if there was an underdose (below the agreed value), an overdose (above the agreed value), no frequency of administration specified, no dose given or excessive total daily dose. The medication error rates and the factors influencing medication error rates were determined using SPSS version 12. RESULTS: From March to August 2003, 4281 prescriptions were issued. Seven prescriptions (0.16%) were excluded, hence 4274 prescriptions were analysed. Most prescriptions were issued by paediatricians (including neonatologists and paediatric surgeons) and/or junior doctors. The error rate in the children's emergency department was 15.7%, for outpatients was 21.5% and for discharge medication was 23.6%. Most errors were the result of an underdose (64%; 536/833). The computer calculated dose error rate was 12.6% compared with the traditional prescription error rate of 28.2%. Logistical regression analysis showed that computer calculated dose was an important and independent variable influencing the error rate (adjusted relative risk = 0.436, 95% CI 0.336, 0.520, p < 0.001). Other important independent variables were seniority and paediatric training of the person prescribing and the type of drug prescribed. CONCLUSIONS: Medication error, especially underdose, is common in outpatient, emergency department and discharge prescriptions. Computer calculated doses can significantly reduce errors, but other risk factors have to be concurrently addressed to achieve maximum benefit.

Successful bridge to transplant with the Berlin Heart after cavopulmonary shunt.

Mechanical cardiac assistance for infants and children may be accomplished using extracorporeal membrane oxygenation or ventricular assist device support, and are now well established as a bridge to cardiac transplantation or recovery in biventricular hearts, usually in the setting of low cardiac output states due to cardiomyopathy or acute myocarditis. Ventricular assist device support remains less well described in the setting of single ventricle physiology. We report the case of a 3-year-old girl who developed severe right ventricular failure 2 years after cavopulmonary shunt after an initial Stage I Norwood operation for hypoplastic left heart syndrome. She was successfully supported to cardiac transplantation using a single chamber Berlin Heart EXCOR ventricular assist device using right ventricular apex and aortic cannulation and is now well at home 10 months after transplant.

Chronic respiratory complications in pediatric heart transplant recipients.

BACKGROUND: The frequency and spectrum of chronic respiratory complications in pediatric heart transplant recipients have not been extensively studied. The aim of this study was to describe the chronic respiratory complications in 126 consecutive pediatric heart transplant recipients. METHODS: Retrospective review of medical records. RESULTS: Between 1987 and 2005, 126 (64 males and 62 females) heart transplantations were performed at Freeman Hospital, Newcastle upon Tyne, United Kingdom. The median age at transplantation was 7.4 years (range, 0.1-17) and the median length of follow-up was 6.8 years (range, 0-18.2). Twenty-four patients have died, and 36 have been transferred to adult follow-up, leaving 66 under pediatric follow-up. Chronic respiratory complications have been documented in 33 children (50%). Bronchiectasis has been identified in 10 children, and 12 further children have had recurrent lower respiratory tract infections (without bronchiectasis) requiring long-term antibiotic prophylaxis. Of those with infectious complications, 81% underwent transplantation before 4 years and had deficiency of pneumococcal-specific antibody response. Obstructive sleep apnea has occurred in 5 children, sub-glottic stenosis has occurred in 3, and significant compression of the left main stem bronchus related to a disproportionately large donor heart has occurred in 2. One child had marked mosaic attenuation on chest computed tomography scan indicative of small airways disease. CONCLUSION: Chronic respiratory complications are common in pediatric heart transplant recipients. The respiratory prognosis for this complex group of patients is usually good, but long-term follow-up by both a respiratory pediatrician and an immunologist is frequently required.

Improved outcome in childhood acute myeloid leukemia in Singapore with the MRC AML 10 protocol.

BACKGROUND: The introduction of the United Kingdom Medical Research Council's 10th AML trial (MRC AML 10) protocol incorporating high-dose anthracycline therapy has improved outcome of children with acute myeloid leukemia (AML). In this study, we review the results of childhood AML therapy in a Singapore university hospital over the last 17 years emphasizing toxicity and outcome. PROCEDURE: Retrospective analysis revealed 34 children with AML between 1988 and 2003. Prior to September 1996, therapy consisted of: POG-8498 (n = 10), others (n = 9). From September 1996, all but one of 15 children received MRC AML 10 treatment. RESULTS: At the time of analysis, 17 had died from disease, and 17 patients were alive among whom 2 had relapsed. MRC AML 10-treated patients (n = 14) had significantly better 3-year overall, event-free, and disease-free survival (74% vs. 35%, 77% vs. 20%, 83% vs. 31%; P = 0.019, P = 0.002, and P = 0.010, respectively) and were likelier to achieve complete remission (CR) than non-MRC AML 10 patients (P = 0.102). Among patients who achieved CR, MRC AML 10-treated patients were significantly more likely to achieve CR after only one cycle of chemotherapy (P = 0.016). Hematologic toxicity was similar among the different regimens (P = 0.9). CONCLUSIONS: These findings suggest that MRC AML 10 treatment results in significantly superior survival, without excess toxicity. Future studies should attempt to elucidate the relative importance of individual MRC AML 10 components and reduce the high cumulative anthracycline dose without compromising outcome.