Background Chylothorax following congenital heart surgery is a common complication with associated morbidities, but consensus treatment guidelines are lacking. Variability exists in the duration of medical treatment and timing for surgical intervention. Methods Following institution of a clinical practice guideline for management of post-operative chylothorax at a single center, pediatric cardiothoracic intensive care unit (ICU) in June 2010, we retrospectively analyzed two cohorts of patients: those with chylothorax from 1/2008-5/2010 (early cohort; n = 118) and from 6/2010-8/2011 (late cohort; n = 45). Data collected included demographics, cardiac surgical procedure, treatments for chylothorax, bloodstream infections, hospital mortality, length of hospitalization, duration of mechanical ventilation, and device utilization. Results There were no demographic differences between the cohorts. No differences were found in octreotide use or surgical treatments for chylothorax. Significant differences were found in median times to chylothorax diagnosis (9 in early cohort versus 6 days in late cohort, p = 0.004), ICU length of stay (18 vs. 9 days, p = 0.01), hospital length of stay (30 vs. 23 days, p = 0.005), and total durations of mechanical ventilation (11 vs. 5 days, p = 0.02), chest tube use (20 vs. 14 days, p = 0.01), central venous line use (27 vs. 15 days, p = 0.001), and NPO status (9.5 vs. 6 days, p = 0.04). Conclusions Institution of a clinical practice guideline for treatment of chylothorax following congenital heart surgery was associated with earlier diagnosis, reduced hospital length of stay, mechanical ventilation, and device utilization for these patients.

Chest pain in children and adolescents frequently involves referral to a pediatric cardiologist. The etiology of chest pain in pediatrics is broad, and the vast majority of cases are not due to underlying cardiac pathology. However, evaluations are often pursued due to fear about missing a potentially serious cardiac diagnosis, which may lead to sudden cardiac death. The management of these patients can lead to extensive investigations, medical visits, and hospitalizations, which is costly and unnecessary in many cases. This article reviews noncardiac and cardiac etiologies of chest pain, highlights pertinent details of the patient history and physical examination, discusses the evaluation of patients with chest pain, and identifies when referral to a pediatric cardiologist is recommended.

IntroductionDiagnosis of infantile thiamine deficiency disorders (TDD) is challenging due to the non-specific, highly variable clinical presentation, often leading to misdiagnosis. Our primary objective is to develop a case definition for thiamine responsive disorders (TRD) to determine among hospitalised infants and young children, which clinical features and risk factors identify those who respond positively to thiamine administration.Methods and analysisThis prospective study will enrol 662 children (aged 21 days to <18 months) seeking treatment for TDD symptoms. Children will be treated with intravenous or intramuscular thiamine (100 mg daily for a minimum of 3 days) alongside other interventions deemed appropriate. Baseline assessments, prior to thiamine administration, include a physical examination, echocardiogram and venous blood draw for the determination of thiamine biomarkers. Follow-up assessments include physical examinations (after 4, 8, 12, 24, 36, 48 and 72 hours), echocardiogram (after 24 and 48 hours) and one cranial ultrasound. During the hospital stay, maternal blood and breast-milk samples and diet, health, anthropometric and socio-demographic information will be collected for mother–child pairs. Using these data, a panel of expert paediatricians will determine TRD status for use as the dependent variable in logistic regression models. Models identifying predictors of TRD will be developed and validated for various scenarios. Clinical prediction model performance will be quantified by empirical area under the receiver operating characteristic curve, using resampling cross validation. A frequency-matched community-based cohort of mother–child pairs (n=265) will serve as comparison group for evaluation of potential risk factors for TRD.Ethics and disseminationEthical approval has been obtained from The National Ethics Committee for Health Research, Ministry of Health, Lao PDR and the Institutional Review Board of the University of California Davis. The results will be disseminated via scientific articles, presentations and workshops with representatives of the Ministry of Health.Trial registration numberNCT03626337.

Background Tetralogy of Fallot with absent pulmonary valve is associated with high mortality, but it remains difficult to predict outcomes prenatally. We aimed to identify risk factors for mortality in a large multicenter cohort. Methods and Results Fetal echocardiograms and clinical data from 19 centers over a 10‐year period were collected. Primary outcome measures included fetal demise and overall mortality. Of 100 fetuses, pregnancy termination/postnatal nonintervention was elected in 22. Of 78 with intention to treat, 7 (9%) died in utero and 21 (27%) died postnatally. With median follow‐up of 32.9 months, no deaths occurred after 13 months. Of 80 fetuses with genetic testing, 46% had chromosomal abnormalities, with 22q11.2 deletion in 35%. On last fetal echocardiogram, at a median of 34.6 weeks, left ventricular dysfunction independently predicted fetal demise (odds ratio [OR], 7.4; 95% CI 1.3, 43.0; P =0.026). Right ventricular dysfunction independently predicted overall mortality in multivariate analysis (OR, 7.9; 95% CI 2.1–30.0; P =0.002). Earlier gestational age at delivery, mediastinal shift, left ventricular/right ventricular dilation, left ventricular dysfunction, tricuspid regurgitation, and Doppler abnormalities were associated with fetal and postnatal mortality, although few tended to progress throughout gestation on serial evaluation. Pulmonary artery diameters did not correlate with outcomes. Conclusions Perinatal mortality in tetralogy of Fallot with absent pulmonary valve remains high, with overall survival of 64% in fetuses with intention to treat. Right ventricular dysfunction independently predicts overall mortality. Left ventricular dysfunction predicts fetal mortality and may influence prenatal management and delivery planning. Mediastinal shift may reflect secondary effects of airway obstruction and abnormal lung development and is associated with increased mortality.

Peak LA systolic strain and LA distensibility may be more useful surrogates of left ventricular filling pressure than E/E' in the pediatric heart transplant population, with greater reproducibility of LA distensibility. Longitudinal studies are needed to evaluate which parameters track changes in PCWP and clinical outcome.