Chest computed tomography in children undergoing extra-corporeal membrane oxygenation: a 9-year single-centre experience.

We retrospectively reviewed the imaging findings, indications, technique and clinical impact in children who had undergone chest CT while undergoing extra-corporeal membrane oxygenation (ECMO). Radiology and ECMO databases were searched to identify all 19 children who had undergone chest CT (20 scans in total) while on ECMO at our institution between May 2003 and May 2012. We reviewed all CT scans for imaging findings. Chest CT is performed in a minority of children on ECMO (4.5% in our series). Timing of chest CT following commencement of ECMO varied among patient groups but generally it was performed earlier in the neonatal group. Clinically significant imaging findings were found in the majority of chest CT scans. Many scans contained several findings, with most cases demonstrating parenchymal or pleural abnormalities. Case examples illustrate the spectrum of imaging findings, including underlying pathology such as necrotising pneumonia and severe barotrauma, and ECMO-related complications such as tension haemothoraces and cannula migration. The results of chest CT led to a change in patient management in 16 of 19 children (84%). There were no adverse events related to patient transfer. An understanding of scan technique and awareness of potential findings is important for the radiologist to provide prompt and optimal image acquisition and interpretation in appropriate patients.

Long-term survival outcomes and causes of late death in neonates, infants, and children treated with extracorporeal life support.

OBJECTIVES: Extracorporeal life support is a resource-intense treatment offered to the sickest patients. We aimed to investigate long-term survival rates and late deaths. DESIGN: Retrospective cohort study. SETTING: Tertiary referral center for extracorporeal life support. PATIENTS: All patients who required extracorporeal life support from 1992 to 2010 at our center. The U.K. National Health Service number was used to trace survival status of all patients who received extracorporeal life support at our center, grouped by diagnosis. Death more than 90 days after extracorporeal life support was defined as late, and these medical records were reviewed. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: A total of 741 children with 272 early deaths (36.7%) and 46 late deaths (6.2%) were included. Median follow-up time in survivors was 7.1 (interquartile range, 3.0-11.9) years. Five-year survival estimates were highest for meconium aspiration syndrome 88.0% (95% CI, 80.6-92.7%) and lowest for congenital heart disease 32.3% (95% CI, 25.1-39.8%). Five-year survival estimates conditional on being alive at 90 days were highest for meconium aspiration syndrome 97.9% (95% CI, 92.0-99.5%) and lowest for congenital diaphragmatic hernia 73.6% (52.3-86.5%). There was increased risk of late death in congenital diaphragmatic hernia, congenital heart disease, and acquired heart disease (p < 0.001, p < 0.01, p = 0.01) in comparison with the risk in meconium aspiration syndrome. For 46 late deaths, 17 had a cardiac cause, 16 had a respiratory cause, 10 had a comorbid cause, one died of sepsis, and in two, causation was unknown. CONCLUSIONS: Although the majority of deaths were early, late mortality was observed following extracorporeal life support. Late deaths were more prevalent in children with underlying complex long-term conditions, particularly heart disease and congenital diaphragmatic hernia. Evaluation of longer term survival is an important component of audit for extracorporeal life support outcomes.

Outcomes following general anaesthesia in children with hypertrophic cardiomyopathy.

BACKGROUND: Children with hypertrophic cardiomyopathy (HCM) have historically been considered to be high-risk candidates for general anaesthesia (GA), but there is currently a paucity of evidence regarding the safety of anaesthesia and perioperative outcomes in this population. METHODS: Clinical features and outcomes of all paediatric patients (<18 years) with HCM undergoing GA between 2000 and 2016 were reviewed. RESULTS: 86 patients (median 12.4 years (IQR 6.5, 14.9)) underwent 164 separate GA procedures. Aetiology included non-syndromic disease (n=44, 56%), malformation syndromes (n=22, 26%), inborn error of metabolism (n=10, 12%) and neuromuscular disease (n=4, 5%). At the time of GA, mean maximal wall thickness (MWT) on echocardiography was 19 mm (SD±8 mm), 23 (14%) patients had severe left ventricular hypertrophy (MWT>30 mm) and 35 (21%) patients had a haemodynamically significant left ventricular outflow tract (LVOT) gradient (>50 mm Hg). The majority (n=143, 87%) had no perioperative complications. 20 (12%) patients had minor perioperative complications: bradycardia (n=4), hypotension (n=15) or transient ST segment changes (n=1). One (0.6% of GA procedures) patient experienced a cardiac arrest during anaesthetic induction with death occurring 3 days later. Clinical parameters (including age, MWT, LVOT gradient, systolic and diastolic dysfunction) were not associated with an increased risk of complications CONCLUSIONS: This is the largest published series to date of paediatric patients with HCM undergoing GA, which demonstrates that, in an expert centre, patients can be anaesthetised with a relatively low perianaesthetic mortality (0.6%) and prevalence of minor complications (12%). Future studies are required to systematically identify clinical features that may predict anaesthetic risk.

Perceived quality of life of children after successful bridging to heart transplantation.

BACKGROUND: Mechanical circulatory support is increasingly used to bridge children with end-stage heart failure to transplant. Quality of life (QoL) has not been systematically evaluated in children bridged to heart transplant. METHODS: All children transplanted for cardiomyopathy during 2001 to 2008 and currently being followed at our center (n = 84) had QoL assessed during 2006 to 2009, at a median of 3 years post-transplant, using a validated generic measure (PedsQL4.0). RESULTS: Twenty-six children, aged 2.7 to 18 (median 7.4) years who were bridged to transplant, were compared with 58 children, aged 2.0 to 18.0 (median 13.0) years, who were transplanted in the same era without bridging. There were no significant differences between the 2 groups on any domains of QoL assessed by children or parents, although the small number of bridged patients increases the likelihood of a Type II error. Bridged children who were younger (r = 0.48, p = 0.02) or more recently transplanted (r = 0.42, p = 0.04) were scored by their parents as having poorer emotional QoL. Regression analysis indicated that age at transplant was the only medical or demographic variable associated with parent-reported total QoL scores (ß = 0.27, p = 0.01). With few links between QoL scores and medical or demographic factors, other subjective psychologic factors may be of greater salience in determining QoL. CONCLUSIONS: Despite greater severity of illness, children who required mechanical bridging to transplantation report a QoL comparable to that of other children undergoing heart transplantation. Younger children may require greater psychologic support to reach their full potential in terms of QoL.