A cardiopulmonary bypass strategy to support a patient with vein of Galen malformation.

We present a dissection of the patent ductus arteriosus and pulmonary artery for surgical repair utilising cardiopulmonary bypass in the setting of vein of Galen malformation. Several strategies were employed to attenuate the cerebral shunt including pH-stat, high cardiac index, restrictive venous drainage, continuous ventilation and deep hypothermic circulatory arrest. The patient recovered from surgery with no apparent neurological sequelae.

Clinical course and outcome for critically ill children with Down syndrome: a retrospective cohort study.

PURPOSE: Children with Down syndrome (DS) have several genetic anomalies within chromosome 21 which may influence their response to critical illness. We compared the intensive care course and outcome of children with DS versus those without. METHODS: Retrospective cohort study in four English paediatric intensive care units (ICUs) (2003-2009, n = 33,485). We examined, via a competing risks model, whether risk (subhazard) for ICU mortality differed for children with DS, after adjusting for important confounders. RESULTS: DS patients exhibited lower disease severity at ICU admission but subsequently required a higher proportion of cardiovascular support, and similar renal support to non-DS patients. Children with DS (n = 1,278) had lower crude mortality than those without (4.2 versus 6.2 %, p = 0.003). This was not significant when expressed as standardized mortality ratio: 0.83 [95 % confidence interval (CI) 0.63-1.09] versus 0.90 (95 % CI 0.86-0.94). However, the competing risks model showed that mortality risk was influenced by length of ICU stay. At admission, DS patients exhibited a subhazard for mortality of 0.63 (95 % CI 0.46-0.85), which increased to 1.00 by day 10 of admission, and continued rising above that of non-DS children thereafter. CONCLUSIONS: Children with DS require a higher proportion of organ support than expected by disease severity at ICU admission. In addition, the mortality risk for children with DS is dependent upon length of ICU stay. These findings could reflect differences in case mix, but are also compatible with different response to critical illness in this group.

A prospective audit of safety issues associated with general anesthesia for pediatric cardiac magnetic resonance imaging.

BACKGROUND/OBJECTIVES: Cardiac MRI (CMR) is increasingly used for surgical planning and serial monitoring of children with congenital heart disease (CHD). For small children, general anesthesia (GA) is required. We describe our experience of the safety of GA for pediatric CMR, using data collected prospectively over 3 years. METHODS: All consecutive infants undergoing GA for CMR at our institution, between November 2005 and May 2008, were included. Informed and written consent to participate in research investigation was acquired from the guardians of every patient prior to CMR. The cardiac anesthetist completed a standardized data collection form during each procedure. Information collected included demographics, diagnosis, surgical history, anesthetic management, significant incidents, and discharge circumstances. RESULTS: A total of 120 patients with varying cardiac physiology and a range of hemodynamics underwent GA for CMR during the study period. Gas induction was predominantly used, even in those with impaired ventricular function. The majority (71%) of procedures were undertaken without significant incident. Minor adverse incidents were recorded in 32 patients, mild hypotension being most frequent. One major adverse event occurred. A patient with hypoplastic left heart syndrome (HLHS) suffered hypotension then cardiac arrest in the scanner. This patient was successfully resuscitated. CONCLUSION: Although the majority of cases were safe and without incident, the complication rate in children with CHD receiving a GA for CMR is higher than in the general pediatric population. This reinforces the need for a senior, multidisciplinary team to be involved in the care of these children during imaging.

Do tracheas grow after slide tracheoplasty?

BACKGROUND: Slide tracheoplasty has become the surgical technique of choice for repair of congenital tracheal stenosis. Despite the initial reluctance regarding the ability of this "reconstructed" trachea to grow, the reduced morbidity and mortality have allowed slide tracheoplasty to be widely adopted. The aim of this study was to evaluate tracheal growth after slide tracheoplasty. METHODS: This was a retrospective study. In follow-up bronchography performed 1, 6, 12, 18, and 24 months after slide tracheoplasty, we measured the cross-sectional areas of the midtrachea and distal trachea at each investigation and correlated the measurements with the anthropomorphic factors (body weight, height, and body surface). RESULTS: Fourteen patients were enrolled in this study. The midtracheal and distal tracheal cross-sectional areas significantly increased with time (p ≤ 0.0001). The average rates of midtracheal growth were 21.0 mm(2)year in the first 6 months and 8.0 mm(2)/year in the first 2 years, and the distal trachea grew 18.5 mm(2)/year and 8.4 mm(2)/year, respectively. Regression analysis showed that both the midtrachea and the distal trachea increase significantly with weight (r(2) = 0.257, p ≤ 0.0001), height (r(2) = 0.376, p ≤ 0.0001), and body surface area (r(2) = 0.315, p ≤ 0.0001). Balloon dilation did not significantly alter the tracheal growth in the first 2 years after slide tracheoplasty. CONCLUSIONS: Slide tracheoplasty does not inhibit tracheal growth. The reconstructed trachea grows faster in the first 6 months and slows in the following 18 months. There is a positive correlation between tracheal cross-sectional area and weight, height, and body surface area.