Prognostic Evaluation of Mortality after Pediatric Resuscitation Assisted by Extracorporeal Life Support.

To improve survival rates during cardiopulmonary resuscitation (CPR), some patients are put on extracorporeal life support (ECLS) during active resuscitation (ECPR). Our objective was to assess the clinical outcomes after pediatric ECPR in Switzerland and to determine pre-ECPR prognostic factors for mortality. The present study is a retrospective analysis. The study setting included three pediatric intensive care units in Switzerland that use ECPR. All patients (<16 years old) undergoing ECPR from 2008 to 2016 were included in the study. There were no interventions. Data before ECLS initiation and clinical outcomes were collected. An ECPR score was designed to predict mortality, based on variables significantly different between survivors and non-survivors. Fifty-five patients were included, with a median age of 13.5 months. Eighty percent were cardiac patients. The mortality rate was 75%. Mortality was significantly associated with CPR duration ( p = 0.02), last lactate ( p = 0.05), and last pH ( p = 0.01) before ECLS initiation. Based on these three variables, an ECPR score was designed as follows: CPR duration (in minutes): 1 point if < 40; 2 points if ≥ 40; 3 points if ≥ 60; 6 points if ≥ 105. Lactate (in mmol/L): 1 point if < 8; 2 points if ≥ 8; 3 points if ≥ 14; 6 points if ≥ 18. pH: 1 point if > 7.00; 2 points if ≤ 7.00; 3 points if ≤ 6.85; 6 points if ≤ 6.60. The area under the receiver-operating characteristic curve was 0.74. The positive predictive value of a score ≥ 9 was 94%. In our population, a score based on three variables easily available prior to ECLS initiation had good discrimination and could appropriately predict mortality. This score now needs validation in a larger population.

The Morbidity and Mortality Conference in Pediatric Intensive Care as a Means for Improving Patient Safety.

OBJECTIVES: To present our experience in an interdisciplinary and interprofessional morbidity and mortality conference, with special emphasis on its usefulness in improving patient safety. DESIGN: Retrospective analysis. SETTING: Tertiary interdisciplinary neonatal PICU. PATIENTS: Morbidity and mortality conference minutes on 48 patients (newborns to 17 yr), January 2009 to June 2014. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: The authors' PICU implemented a morbidity and mortality conference guideline in 2009 using a system-based approach to identify medical errors, their contributing factors, and possible solutions. In the subsequent 5.5 years, there were 44 mortality conferences (of 181 deaths [27%] over the same period) and four morbidity conferences. The median death/morbidity event-morbidity and mortality conference interval was 90 days (range, 7 d to 1.5 yr). The median age of patients was 4 months (range, newborn to 17 years). In six cases, the primary reason for PICU admission was a treatment complication. Unsafe processes/medical errors were identified and discussed in 37 morbidity and mortality conferences (77%). In seven cases, new autopsy findings prompted the discussion of a possible error. The 48 morbidity and mortality conferences identified 50 errors, including 30 in which an interface problem was a contributing factor. Fifty-four improvements were identified in 34 morbidity and mortality conferences. Four morbidity and mortality conferences discussed specific ethical issues. CONCLUSIONS: From our experience, we have found that the interdisciplinary and interprofessional morbidity and mortality conference has the potential to reveal unsafe processes/medical errors, in particular, diagnostic and communication errors and interface problems. When formatted as a nonhierarchical tool inviting contributions from all staff levels, the morbidity and mortality conference plays a key role in the system approach to medical errors.

Propionic acidemia in a previously healthy adolescent with acute onset of dilated cardiomyopathy.

UNLABELLED: Propionic acidemia (PA) is a rare autosomal recessive organic aciduria resulting from defects in propionyl-CoA-carboxylase (PCC), a key enzyme of intermediate energy metabolism. PA mostly manifests during the neonatal period, when affected newborns develop severe metabolic acidosis and hyperammonemia. We present a previously healthy teenager, who suffered from acute fatigue and breathlessness. The patient was tachycardic, displayed a precordial heave and a systolic murmur. Cardiac investigations revealed severe dilated cardiomyopathy (DCM). Biochemical work up led to the diagnosis of PA. Remarkably, this patient of consanguineous Hispanic origin was in a good general health condition before the acute onset of DCM. Diagnosis of PA was confirmed by enzymatic and molecular genetic analysis, the latter revealing a novel homozygous mutation in the PCCB gene (c.1229G > A; p.R410Q). Residual PCC enzyme activity of approximately 14 % of normal was detected in patient's lymphocytes and fibroblasts, thereby providing a possible explanation for the hitherto asymptomatic phenotype. CONCLUSION: Isolated DCM, although rare, can be the leading and/or sole symptom of late-onset PA. Therefore, patients with DCM should receive a comprehensive diagnostic evaluation including selective screening for inborn errors of metabolism.

Children with genetic disorders undergoing open-heart surgery: are they at increased risk for postoperative complications?

OBJECTIVES: Children with congenital heart disease and genetic disorders may be at increased risk for postoperative mortality and morbidity compared with children with congenital heart disease alone. The aim of the present study was to determine differences in postcardiopulmonary bypass outcome between these two groups. DESIGN: Prospective cohort study. SETTING: Tertiary university children's hospital. PATIENTS: We enrolled 211 infants (<1 yr) who underwent bypass surgery for congenital heart disease. Data on perioperative course were compared between infants with and without genetic disorders. Univariate analysis was followed by regression analysis to control for confounders. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: We enrolled 148 infants without and 63 infants with a genetic disorder. The majority of infants with genetic disorders had trisomy 21 (n = 32), six had microdeletion 22q11, and 25 had other genetic disorders. There was no significant difference in mortality between infants with and without genetic disorders. An underlying genetic disorder was an independent risk factor for renal insufficiency (p = .003) and reintubation (p = .02). Trisomy 21 was an independent risk factor for chylothorax (p = .01) and sepsis (p = .05). The length of hospital stay was longer in infants with genetic disorders other than trisomy 21 compared with infants with trisomy 21 (p = .009). CONCLUSIONS: Infants with congenital heart disease and genetic disorders are not at increased risk for postoperative mortality. However, a genetic disorder is a risk factor for reintubation and renal insufficiency, whereas infants with trisomy 21 have a higher risk of chylothorax and sepsis. Intensive care providers need to be aware of these differences in morbidity to improve management decisions and parental counseling.

Effect of sedation and analgesia on postoperative amplitude-integrated EEG in newborn cardiac patients.

The aim of this study is to describe the effect of sedation and analgesia on postoperative amplitude-integrated EEG (aEEG) in newborns with congenital heart disease (CHD) undergoing heart surgery. This is a consecutive series of 26 newborns with CHD of which 16 patients underwent cardiopulmonary bypass (CPB) surgery and 10 patients did not. aEEG was monitored for at least 12 h preoperatively and started within the first 6 h postoperatively for 48 h. Outcome was assessed at 1 year of age. All 26 patients showed a normal preoperative continuous cerebral activity with sleep-wake cycles (SWC). The postoperative duration to return to normal background activity with SWC was similar for both groups. Independent of group assignment, patients requiring midazolam had a significantly later onset of a normal SWC than those without midazolam (p=0.03). Three patients in the CPB group and two in the non-CPB group showed continuous low voltage or flat trace after administration of fentanyl. These changes did not correlate with neurodevelopmental outcome. Sedation with midazolam has a transient effect on the background activity, whereas fentanyl can induce a severe pathologic background pattern. The significance of these changes on outcome is not yet clear. Thus, more attention should be paid to these effects when interpreting aEEG in this population.

Late morbidity during childhood and adolescence in previously premature neonates after patent ductus arteriosus closure.

The health status of previously premature neonates after closure of a patent ductus arteriosus (PDA) was analyzed in childhood and adolescence. Physician questionnaires were used to study 180 hospital survivors among 210 consecutive premature neonates who underwent PDA closure between 1985 and 2005. Complete follow-up data were obtained for 129 patients (72%). During a median follow-up period of 7 years (range, 2-22 years), three late deaths (2.3%) had occurred. Only 45% of the patients were considered healthy. Morbidity included developmental delay (41.1%), pulmonary illness (12.4%), neurologic impairment (14.7%), hearing impairment (3.9%), gastrointestinal disease (3.1%), and thoracic deformity (1.2%). None of the adverse variables during the neonatal period (intraventricular hemorrhage, bradycardia apnea syndrome, bronchopulmonary dysplasia, pulmonary bleeding, hyaline membrane disease, artificial respiration time [continuous positive airway pressure + intubation], or necrotizing enterocolitis) statistically predicted respective system morbidity at the follow-up evaluation. Hyaline membrane disease (odds ratio, 2.5; p = 0.026) and longer hospitalization time (odds ratio, 1.2 days per 10 hospitalization days; p = 0.032) in the newborn period were significant predictors of an unhealthy outcome at the last follow-up evaluation. Survival until childhood after closure of a hemodynamically significant PDA in premature neonates is satisfactory. However, physical and neurodevelopmental co-morbidity persist for half of the patients, perhaps as a sequela of prematurity unrelated to ductus closure.

Risk factors for reoperation after relief of congenital subaortic stenosis.

BACKGROUND: Congenital subaortic stenosis entails a lesion spectrum, ranging from an isolated obstructive membrane, to complex tunnel narrowing of the left outflow associated with other cardiac defects. We review our experience with this anomaly, and analyze risk factors leading to restenosis requiring reoperation. METHODS: From 1994 to 2006, 58 children (median age 4.3 years, range 7 days-13.7 years) underwent primary relief of subaortic stenosis. Patients were divided into simple lesions (n=43) or complex stenosis (n=15) associated with other major cardiac defects. Age, pre- and postoperative gradient over the left outflow, associated aortic or mitral valve insufficiency, chromosomal anomalies, arteria lusoria, and operative technique (membrane resection (22) vs associated myectomy (34) vs Konno (2)) were analyzed as risk factors for reoperation (Kaplan-Meier, Cox regression). RESULTS: There was no operative mortality. Median follow-up spanned 2.7 years (range 0.1-10), with one late death at 4 months. Reoperation was required for recurrent stenosis in 11 patients (19%) at 2.6 years (range 0.3-7.5) after initial surgery. Risk factors for reoperation included complex subaortic stenosis (p=0.003), younger age (p=0.012), postoperative residual gradient (p=0.019), and the presence of an arteria lusoria (p=0.014). For simple lesions, no variable achieved significance for stenosis recurrence. CONCLUSIONS: Surgical relief of congenital subaortic stenosis, even with complex defects, yields excellent results. Reoperation is not infrequent, and should be anticipated with younger age at operation, complex defects, residual postoperative gradient, and an arteria lusoria. Myectomy concomitant to membrane resection, even in simple lesions, does not provide enhanced freedom from reoperation, and should be tailored to anatomic findings.

The effect of ductal diameter on surgical and medical closure of patent ductus arteriosus in preterm neonates: size matters.

OBJECTIVE: We sought to analyze the effect of patent ductus arteriosus diameter on treatment success in premature neonates. METHODS: Among 537 consecutive neonates born between 1985 and 2005 with a diagnosed patent ductus arteriosus, 201 premature patients (<35 weeks' gestation) treated for a hemodynamically significant patent ductus arteriosus were retrospectively reviewed. Two groups were compared: group MED (n = 154; successful treatment with indomethacin) and group FAIL (n = 47; failure of medication to reduce the patent ductus arteriosus diameter to hemodynamic insignificance). RESULTS: After unsuccessful medical treatment, 33 patients required surgical patent ductus arteriosus closure, 12 died before further possible treatment, and 2 were discharged home without clinical symptoms but with an open patent ductus arteriosus. Mean patent ductus arteriosus diameter in the FAIL group (2.8 +/- 0.9 mm) was significantly larger than that in the MED group (2.4 +/- 0.6 mm, P < .01). Assisted respiration time (ventilation plus continuous positive airway pressure) before patent ductus arteriosus closure was longer in the FAIL group (20 days) than in the MED group (9 days, P < .001) but was similar after patent ductus arteriosus closure. By using an index of patent ductus arteriosus diameter squared/birth weight (in square millimeters per kilogram), a cutoff value of less than 9 mm2/kg correctly predicts medical patent ductus arteriosus closure in 87.5% of patients. Values of greater than 9 mm2/kg correctly predict medication failure in 41.5% of patients. CONCLUSIONS: In preterm babies requiring surgical patent ductus arteriosus closure, longer respiration times reflect a delay while attempting medical treatment, but respiration time is equally short between groups after shunt elimination. Medical treatment, although a valid first option, is likely to fail with larger patent ductus arteriosus diameters and lower birth weights. Unwarranted assisted respiration and corresponding hospital stay might be shortened by earlier surgical referral for patent ductus arteriosus closure in preterm babies with a patent ductus arteriosus index of greater than 9 mm2/kg.