Light Shed on Deadly GI Disease in Infants Born with Complex Congenital Heart Disease

Recent findings from a study published in Pediatric Critical Care Medicine by researchers from the Nationwide Children’s Hospital show that not only are infants who are born with complex congenital heart disease at risk for serious heart-related complications, they are also at risk—regardless of surgical intervention type for their heart disease—for developing the deadly bowl disease necrotizing enterocolitis (NEC)."NEC and congenital heart disease are two distinct disease processes, but they appear to be inter-related, particularly in patients with the congenital heart condition known as hypoplastic left heart syndrome," said Wendy Luce, MD, lead author and principal investigator, Center for Perinatal Research, Nationwide Children's Hospital.

With research showing “that neonates undergoing the Norwood surgery for hypoplastic left heart syndrome (HLHS) have the highest risk for NEC of all congenital heart disease patients,” a hybrid approach was developed at Nationwide Children’s as an alternative to the standard Norwood procedure for managing HLHS and other complex congenital heart diseases. The risk of major open heart surgery and cardiopulmonary bypass is shifted to later in infancy using the hybrid approach.

“Since both the Norwood and hybrid procedures have been shown to be effective in treating the immediate dangers associated with complex congenital heart diseases, it's important that we begin to compare the secondary outcomes and quality of life measures related to both surgical approaches," said Luce.

After reviewing the charts of 73 patients who underwent hybrid stage I procedure for treating complex congenital heart disease and Nationwide Children’s over 6 years, Luce’s team found that 11% developed moderate-to-severe NEC at an average of 8 days after the operation. Although the percentage is similar to that seen in neonates who undergo the Norwood procedure, just two of those who underwent the hybrid procedure required abdominal surgery for NEC, compared to nearly 60% of those document in other reports.

"Our early and aggressive treatment of neonates with symptoms of NEC in this high risk population appears to be warranted and may contribute to the relatively low need for abdominal surgery in this patient population," said Dr. Luce.

In aiming to determine risk factors that are associated with NEC and the studied patient population, Luce’s team looked at “pregnancy factors such as mother's age and history of prenatal care; pre-surgical factors such as ventilation at the time of the procedure and maximum dose of prostaglandin infusion -- a medicine required to maintain blood flow to the body in patients with HLHS and other forms of complex congenital heart disease that result in decreased or no blood flow to the body -- ; and factors related to the surgery including patient's age on the day of surgery and the mode in which the vascular stent was placed during the procedure.” Just three were associated significantly with NEC: birth at fewer than 37 weeks gestational age, being the recipient of a lower than maximum prostaglandin dose; and unexpected readmission to the ICU.

Dr. Luce said that “although the study's findings can't be immediately generalized to other patients with congenital heart disease, the data reinforces the belief that clinicians should continue to be watchful for NEC in neonates undergoing surgery for congenital heart disease. Also, multidisciplinary approaches to feeding regimens in these high-risk patients are needed to improve outcomes and quality of life.”