Current practices for refractory chylothorax following congenital heart surgery.

INTRODUCTION: Chylothorax following paediatric cardiac surgery is associated with significant morbidity, particularly those that are refractory to conservative therapy. It is our impression that there is important variability in the medical, surgical, and interventional therapies used to manage refractory chylothorax between congenital heart programmes. We therefore conducted a survey study of current practices for managing refractory chylothorax. METHODS: The Chylothorax Work Group, formed with the support of the Pediatric Cardiac Critical Care Consortium, designed this multi-centre survey study with a focus on the timing and indication for utilising known therapies for refractory chylothorax. The survey was sent to one chylothorax expert from each Work Group centre, and results were summarised and reported as the frequency of given responses. RESULTS: Of the 20 centres invited to participate, 17 (85%) submitted complete responses. Octreotide (13/17, 76%) and sildenafil (8/17, 47%) were the most utilised medications. Presently, 9 (53%) centres perform pleurodesis, 15 (88%) perform surgical thoracic duct ligation, 8 (47%) perform percutaneous lymphatic interventions, 6 (35%) utilise thoracic duct decompression procedures, and 3 (18%) perform pleuroperitoneal shunts. Diagnostic lymphatic imaging is performed prior to surgical thoracic duct ligation in only 7 of the 15 (47%) centres that perform the procedure. Respondents identified barriers to referring and transporting patients to centres with expertise in lymphatic interventions. CONCLUSIONS: There is variability in the treatment of refractory post-operative chylothorax across a large group of academic heart centres. Few surveyed heart centres have replaced surgical thoracic duct ligation or pleurodesis with image-guided selective lymphatic interventions.

Junctional Ectopic Tachycardia: Recognition and Modern Management Strategies.

Junctional ectopic tachycardia is a common dysrhythmia after congenital heart surgery that is associated with increased perioperative morbidity and mortality. Risk factors for development of junctional ectopic tachycardia include young age (neonatal and infant age groups); hypomagnesemia; higher-complexity surgical procedure, especially near the atrioventricular node or His bundle; and use of exogenous catecholamines such as dopamine and epinephrine. Critical care nurses play a vital role in early recognition of dysrhythmias after congenital heart surgery, assessment of hemodynamics affecting cardiac output, and monitoring the effects of antiarrhythmic therapy. This article reviews the underlying mechanisms of junctional ectopic tachycardia, incidence and risk factors, and treatment options. Currently, amiodarone is the pharmacological treatment of choice, with dexmedetomidine increasingly used because of its anti-arrhythmic properties and sedative effect.

Anomalous left subclavian artery from the pulmonary artery in a neonate.

The presence of an isolated left subclavian artery arising from the pulmonary artery in a patient with d-transposition of the great arteries is exceedingly rare. Prior to undertaking repair of this congenital cardiac anomaly, identification of an isolated left subclavian artery originating from the pulmonary artery is imperative in order to plan the appropriate intervention to prevent the development of subclavian artery steal or vertebrobasilar insufficiency. We describe the case of a 5 day-old girl in whom an isolated left subclavian artery arising from the pulmonary artery was detected after surgical entry for repair of transposition of the great arteries.

Healthcare Disparities in Outcomes of a Metropolitan Congenital Heart Surgery Center: The Effect of Clinical and Socioeconomic Factors.

OBJECTIVE: The purpose of this study is to identify the impact of demographic, socioeconomic, and clinical factors on congenital heart surgery outcomes. STUDY DESIGN: This retrospective cohort study included 234 congenital heart surgery patients from 2011 through 2015, in a racially/ethnically diverse metropolitan children's hospital. Outcomes included length of stay (LOS), age at first echocardiogram, length of mechanical ventilation, and incidence of complications. RESULTS: Compared to others, black children underwent their first echocardiogram at a later age (median 23 versus 2 days, p = 0.014) and were more likely to be diagnosed with congenital heart disease in the emergency room (p = 0.026). Hispanic children were more likely to have major non-cardiac congenital anomalies (p = 0.045). Increased LOS during elective admissions was associated with higher surgical complexity (STAT category 4 and 5 Estimate 3.905 days, p = 0.001), compared to STAT category 1, and number of complications (Estimate = 2.306 days per complication, p < 0.001). Increased LOS in non-elective admissions was associated with the number of complex chronic conditions (Estimate = 15.446 days, p = 0.045) and the number of complications (Estimate = 11.591 days per complication, p < 0.001). However, in multivariate analysis, race and ethnicity was not associated with increased LOS or age at first echocardiogram. CONCLUSION: In this diverse setting, race/ethnicity was not associated with increased LOS, age at first echocardiogram, length of ventilation, or complications. Surgical complexity, chronic conditions, and complications were associated with increased LOS. We discuss some interventions to reduce disparities in congenital heart surgery outcomes.

Long-Term Outcomes of Children With Trisomy 13 and 18 After Congenital Heart Disease Interventions.

BACKGROUND: The purpose of this study is to report short- and long-term outcomes after congenital heart defect (CHD) interventions in patients with trisomy 13 or 18. METHODS: A retrospective review of the Pediatric Cardiac Care Consortium (PCCC) identified children with trisomy 13 or 18 with interventions for CHD between 1982 and 2008. Long-term survival and cause of death were obtained through linkage with the National Death Index. RESULTS: A total of 50 patients with trisomy 13 and 121 patients with trisomy 18 were enrolled in PCCC between 1982 and 2008; among them 29 patients with trisomy 13 and 69 patients with trisomy 18 underwent intervention for CHD. In-hospital mortality rates for patients with trisomy 13 or trisomy 18 were 27.6% and 13%, respectively. Causes of in-hospital death were primarily cardiac (64.7%) or multiple organ system failure (17.6%). National Death Index linkage confirmed 23 deaths after discharge. Median survival (conditioned to hospital discharge) was 14.8 years (95% confidence interval [CI]: 12.3 to 25.6 years) for patients with trisomy 13 and 16.2 years (95% CI: 12 to 20.4 years) for patients with trisomy 18. Causes of late death included cardiac (43.5%), respiratory (26.1%), and pulmonary hypertension (13%). CONCLUSIONS: In-hospital mortality rate for all surgical risk categories was higher in patients with trisomy 13 or 18 than that reported for the general population. However, patients with trisomy 13 or 18, who were selected as acceptable candidates for cardiac intervention and who survived CHD intervention, demonstrated longer survival than previously reported. These findings can be used to counsel families and make program-level decisions on offering intervention to carefully selected patients.