This study aims to examine the surgical outcome of Kabuki syndrome patients after neonatal congenital heart surgery.

This was a single-centre retrospective study of Kabuki syndrome patients undergoing neonatal congenital heart surgery from 2018 to 2023. Primary outcome was survival to discharge after index surgery. Secondary outcomes were morbidities and complications. Survival and hospital length of stay were compared to neonates with non-Kabuki genetic anomalies undergoing congenital heart surgery in the same time period.

A total of seven patients were reviewed. All Kabuki syndrome patients had left-sided lesions including three with hypoplastic left heart syndrome, three with aortic stenosis and/or aortic arch hypoplasia, and one with an isolated coarctation of aorta. Hospital survival was 5/7 (71% compared to 88% for neonates with non-Kabuki genetic anomalies). To date, four remain alive, including one with hypoplastic left heart syndrome. A higher percentage of Kabuki syndrome patients had unplanned interventions (43% vs 15% in non-Kabuki), abnormal brain imaging (29% vs 5%), and bacteremia (29% vs 9%). Median total ventilator days for Kabuki patients were also longer (16 days vs 6 days in non-Kabuki) as was hospital length of stay (66 days vs 41 days).

Despite survival to discharge after index operation, Kabuki syndrome patients with single ventricle physiology remain at high risk of mortality and morbidity after cardiac surgery. However, they may be discharged without ventilator dependency and survive to toddler years.

Poor oral feeding is a known contributor to growth challenges in neonates with complex CHD who require early surgery. Almost 60% of these infants do not achieve full oral feeding by hospital discharge. This study’s objective was to identify predictors of the inability to achieve full oral feeding by discharge in neonates with complex CHD following surgical intervention with cardiopulmonary bypass.

A retrospective analysis of a prospective study of 192 full-term neonates with complex CHD was performed. A stepwise selection logistic regression model was developed to predict oral feeding status at hospital discharge. Univariate subgroup analysis was performed with groups determined based on a CHD classification system.

58% of neonates (112/192) failed to achieve full oral feeding by hospital discharge. A logistic regression model identified duration of deep hypothermic circulatory arrest and reintubation as predictors of the inability to achieve full oral feeding. Among neonates who achieved full oral feeding by discharge (42%), only 7.5% did so after postoperative day 10. Brain maturation, brain injury, and preoperative oral feeding were not predictors of full postoperative oral feeding.

Many infants with CHD fail to achieve full oral feeding by time of hospital discharge. Longer duration of deep hypothermic circulatory arrest and increased number of intubations were predictive of poor feeding after surgery. Prolonging hospitalisation solely to achieve full oral feeding after postoperative day ten is of limited utility; earlier discharge should be promoted to avoid negative impacts on neonatal neurodevelopment as unintended consequences of lengthy hospitalisations.

The use of peritoneal catheters for prophylactic dialysis or drainage to prevent fluid overload after neonatal cardiac surgery is common in some centres; however, the multi-centre variability and details of peritoneal catheter use are not well described.

Twenty-two-centre NEonatal and Pediatric Heart Renal Outcomes Network (NEPHRON) study to describe multi-centre peritoneal catheter use after STAT category 3–5 neonatal cardiac surgery using cardiopulmonary bypass. Patient characteristics and acute kidney injury/fluid outcomes for six post-operative days are described among three cohorts: peritoneal catheter with dialysis, peritoneal catheter with passive drainage, and no peritoneal catheter.

Of 1490 neonates, 471 (32%) had an intraoperative peritoneal catheter placed; 177 (12%) received prophylactic dialysis and 294 (20%) received passive drainage. Sixteen (73%) centres used peritoneal catheter at some frequency, including six centres in >50% of neonates. Four centres utilised prophylactic peritoneal dialysis. Time to post-operative dialysis initiation was 3 hours [1, 5] with the duration of 56 hours [37, 90]; passive drainage cohort drained for 92 hours [64, 163]. Peritoneal catheter were more common among patients receiving pre-operative mechanical ventilation, single ventricle physiology, and higher complexity surgery. There was no association with adverse events. Serum creatinine and daily fluid balance were not clinically different on any post-operative day. Mortality was similar.

In neonates undergoing complex cardiac surgery, peritoneal catheter use is not rare, with substantial variability among centres. Peritoneal catheters are used more commonly with higher surgical complexity. Adverse event rates, including mortality, are not different with peritoneal catheter use. Fluid overload and creatinine-based acute kidney injury rates are not different in peritoneal catheter cohorts.

We thought of assessing the prevalence and predictors of feeding disorders in patients with congenital heart defects after neonatal cardiac surgery.

Retrospective study of 82 consecutive neonates (48 males, 34 females) who underwent surgery for congenital heart defects from 1999 to 2002. Information was taken from patient charts and nursing notes. The presence of a feeding disorder was assessed by a questionnaire sent to the paediatricians when the child was 2 years of age. A feeding disorder was defined as a need for tube feeding, inadequate food intake for age, or failure to thrive. Data were analysed with descriptive statistics and logistic regression.

Feeding disorders occurred in 22% of the study population. Reoperation and early feeding disorders were identified as independent risk factors for later feeding disorders (odds ratio 5.8, p 0.01; odds ratio 20.7, p 0.02). There was a trend towards more feeding disorders in patients with neurological abnormalities during the first hospital stay.

Feeding disorder is a frequent, long-term sequela after neonatal cardiac surgery. Patients with congenital heart defects who undergo multiple cardiac surgeries and those with early feeding disorders are at risk of developing later feeding disorders. Patients with these risk factors need to be selected for preventive strategies.

The purpose of this study was to assess the pattern of weight change from surgical intervention to home discharge and to determine predictors of poor growth in this population of infants with congenital cardiac disease.

Neonates with functionally univentricular physiology enrolled in a prospective cohort study examining growth between March, 2003 and May, 2007 were included. Weights were collected at birth, before surgical intervention, and at hospital discharge. In addition, retrospective echocardiographic data and data about post-operative complications were reviewed. Primary outcome variables were weight-for-age z-score at discharge and change in weight-for-age z-score between surgery and discharge.

A total of 61 infants met the inclusion criteria. The mean change in weight-for-age z-score between surgery and hospital discharge was minus 1.5 plus or minus 0.8. Bivariate analysis revealed a significant difference in weight-for-age z-score between infants who were discharged on oral feeds, minus 1.1 plus or minus 0.8 compared to infants with feeding device support minus 1.7 plus or minus 0.7, p-value equal to 0.01. Lower weight-for-age z-score at birth, presence of moderate or greater atrioventricular valve regurgitation, post-operative ventilation time, and placement of an additional central venous line were associated with 60% of the variance in weight-for-age z-score change.

Neonates undergoing staged surgical repair for univentricular physiology are at significant risk for growth failure between surgery and hospital discharge. Haemodynamically significant atrioventricular valve regurgitation and a complex post-operative course were risk factors for poor post-operative weight gain. Feeding device support appears to be insufficient to ensure adequate weight gain during post-operative hospitalisation.