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Introduction:Chylothorax after congenital cardiac surgery is associated with increased risk of malnutrition. Nutritional management following chylothorax diagnosis varies across sites and patient populations, and a standardised approach has not been disseminated. The aim of this review article is to provide contemporary recommendations related to nutritional management of chylothorax to minimise risk of malnutrition.Methods:The management guidelines were developed by consensus across four dietitians, one nurse practitioner, and two physicians with a cumulative 52 years of experience caring for children with CHD. A PubMed database search for relevant literature included the terms chylothorax, paediatric, postoperative, CHD, chylothorax management, growth failure, and malnutrition.Results:Fat-modified diets and nil per os therapies for all paediatric patients (Conclusions:Our deliverable is a clinically useful guide for the nutritional management of chylothorax following paediatric cardiac surgery.

Congenital heart disease (CHD) is the most commonly reported birth defect in newborns. Neonates with CHD are more likely to be born prematurely, and a higher proportion of preterm neonates have CHD than their term counterparts. The implications of preterm birth on the cardiac and noncardiac organ systems are vast and require special management considerations. The feasibility of surgical interventions in preterm neonates is frequently limited by patient size and delicacy of immature cardiac tissues. Thus, special care must be taken when considering the appropriate timing and type of cardiac intervention. Despite improvements in neonatal cardiac surgical outcomes, preterm and early term gestational ages and low birthweight remain important risk factors for in-hospital mortality. Understanding the risks of early delivery of neonates with prenatally diagnosed CHD may help guide perioperative management in neonates who are born preterm. In this review, we will describe the risks and benefits of early delivery, postnatal cardiac and noncardiac evaluation and management, surgical considerations, overall outcomes, and future directions regarding optimization of perinatal evaluation and management of fetuses and preterm and early term neonates with CHD.

Approximately 50% of newborns with Down syndrome have congenital heart disease. Non-cardiac comorbidities may also be present. Many of the principles and strategies of perioperative evaluation and management for patients with congenital heart disease apply to those with Down syndrome. Nevertheless, careful planning for cardiac surgery is required, evaluating for both cardiac and noncardiac disease, with careful consideration of the risk for pulmonary hypertension. In this manuscript, for children with Down syndrome and hemodynamically significant congenital heart disease, we will summarize the epidemiology of heart defects that warrant intervention. We will review perioperative planning for this unique population, including anesthetic considerations, common postoperative issues, nutritional strategies, and discharge planning. Special considerations for single ventricle palliation and heart transplantation evaluation will also be discussed. Overall, the risk of mortality with cardiac surgery in pediatric patients with Down syndrome is no more than the general population, except for those with functional single ventricle heart defects. Underlying comorbidities may contribute to postoperative complications and increased length of stay. A strong understanding of cardiac and non-cardiac considerations in children with Down syndrome will help clinicians optimize perioperative care and long-term outcomes.

Growth failure is a widespread health problem in infants with congenital heart disease, specifically infants with single ventricle heart lesions, including hypoplastic left heart syndrome. Optimizing nutrition delivery for neonates undergoing cardiac surgery is challenging given environmental and physiologic barriers. Concomitant with nutrition delivery challenges, wide practice variations exist among clinicians. Malnutrition and poor growth are associated with poor wound healing, increased infection risk, prolonged hospitalizations, and long-term neurodevelopmental disability, including worse school performance. The cause of early postnatal growth failure is multifactorial and related to the delay in onset of enteral feedings, increased metabolic stress of cardiac surgery, cyanosis, low cardiac output, increased energy expenditure, altered splanchnic perfusion, and gastrointestinal morbidities. In addition, cardiac infants are susceptible to unique complications, including chylothorax, vocal cord dysmotility, and swallowing dysfunction.

Chylothorax is a well-described complication after cardiothoracic surgery in children. Medical nutritional therapy for chylothorax includes medium-chain triglyceride (MCT) formulas and reduction in enteral long-chain triglyceride intake to reduce chyle production. Human milk is usually eliminated from the diet of infants with chylothorax because of its high long-chain triglyceride content. However, given the immunologic properties of human milk, young infants with chylothorax may benefit from using human milk over human milk substitutes. We performed a retrospective cohort study to describe the feasibility and efficacy of defatted human milk (DHM) for the treatment for chylothorax in infants after cardiac surgery and to compare growth outcomes between infants treated with DHM (n = 14) versus MCT formula (n = 21). There were no differences in mortality or length of hospital stay between the DHM and MCT formula treatment groups. The DHM treatment group had a significantly higher weight-for-age z-score at hospital discharge compared to the MCT formula group with median z-scores of -1 (-2 to 0.5) and -1.5 (-2 to 0), respectively (p = 0.02). In infants with chylothorax after cardiac surgery, DHM is a safe and feasible medical nutritional treatment and may have potential benefits for improved nutrition and growth.