Management of Complex Pulmonary Vein Stenosis at Altitude Combining Comprehensive Percutaneous Interventional Treatment with Sirolimus, Pulmonary Hypertension Medications and Intraluminal Imaging with Optical Coherence Tomography.

BACKGROUND: Pulmonary vein stenosis (PVS) is a growing problem for the pediatric congenital heart population. Sirolimus has previously been shown to improve survival and slow down the progression of in-stent stenosis in patients with PVS. We evaluated patients before and after initiation of sirolimus to evaluate its effects on re-intervention and vessel patency utilizing Optical Coherence Tomography (OCT). METHODS: We performed a retrospective study, reviewing the charts of patients with PVS, who had been prescribed sirolimus between October 2020 and December 2021. OCT was performed in the pulmonary vein of interest as per our published protocol. Angiographic and OCT imaging was retrospectively reviewed. Statistical analysis was performed using Chi square and Wilcoxon signed-rank test to compare pre-and post-sirolimus data. RESULTS: Ten patients had been started and followed on sirolimus. Median age at sirolimus initiation was 25 months with median weight of 10.6 kg and average follow-up of 1 year. Median total catheterizations were 7 for patients prior to starting sirolimus and 2 after starting treatment (p = 0.014). Comparing pre- and post-sirolimus, patients were catheterized every 3 months vs every 11 months (p = 0.011), median procedure time was 203 min vs 145 min (p = 0.036) and fluoroscopy time, 80 min vs 57.2 min (p = 0.036). 23 veins had severe in-stent tissue ingrowth prior to SST (luminal diameter < 30% of stent diameter). Post-sirolimus, 23 pulmonary veins had moderate to severe in-stent tissue ingrowth that responded to non-compliant balloon inflation only with stent luminal improvement of > 75%. CONCLUSION: Our study suggests that the addition of sirolimus in patients with moderate-severe PVS helps to decrease disease progression with decrease frequency of interventions. Reaching therapeutic levels for sirolimus is critical and medication interactions and side-effects need careful consideration. OCT continues to be important for evaluation and treatment guidance in this patient population.

A preoperative standardized feeding protocol improves human milk use in infants with complex congenital heart disease.

OBJECTIVE: To evaluate the hypothesis that implementation of a preoperative standardized feeding protocol increases human milk use in infants with complex congenital heart disease (CHD). STUDY DESIGN: Single-center, quasi-experimental study of infants with complex CHD. A cohort of 546 infants pre protocol was compared to 55 patients post protocol. Feeding regimen and peri-operative outcomes information were collected. RESULT: Human milk use increased significantly (58.4% versus 100%, p < 0.01) and there was no formula use post protocol (18.7% versus 0%, p < 0.01). Preoperative necrotizing enterocolitis occurred in 18/546 (3.3%) infants pre protocol versus 1/55 (1.8%) post protocol, p = 1.00. Days to full feeds and length of hospital stay in both cohorts were not significantly different. CONCLUSION: Successful implementation of a preoperative standardized feeding protocol can increase human milk and decrease formula use in infants with complex CHD without significant adverse outcomes. A larger study is needed to evaluate the association of human milk use with peri-operative outcomes.

The Relationship Between Preoperative Feeding Exposures and Postoperative Outcomes in Infants With Congenital Heart Disease.

OBJECTIVES: To evaluate the association of preoperative risk factors and postoperative outcomes in infants with complex congenital heart disease. DESIGN: Single-center retrospective cohort study. SETTING: Neonatal ICU and cardiovascular ICU. PATIENTS: Infants of all gestational ages, born at Texas Children's Hospital between 2010 and 2016, with complex congenital heart disease requiring intervention prior to discharge. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: A total of 399 patients were enrolled in the study. Preoperative risk factors included feeding, type of feeding, feeding route, and cardiac lesion. Postoperative outcomes included necrotizing enterocolitis, hospital length of stay, and days to full feeds. The occurrence rate of postoperative necrotizing enterocolitis (all stages) was 8%. Preoperative feeding, type of feeding, feeding route, and cardiac lesion were not associated with higher odds of postoperative necrotizing enterocolitis. Cardiac lesions with ductal-dependent systemic blood flow were associated with a hospital length of stay of 19.6 days longer than those with ductal-dependent pulmonary blood flow (p < 0.001) and 2.9 days longer to reach full feeds than those with ductal-dependent pulmonary blood flow (p < 0.001), after controlling for prematurity. Nasogastric feeding route preoperatively was associated with a length of stay of 29.8 days longer than those fed by mouth (p < 0.001) and 2.4 days longer to achieve full feeds (p < 0.001), after controlling for prematurity and cardiac lesion. Preoperative diet itself was not associated with significant change in length of stay or days to reach full feeds. CONCLUSIONS: Although cardiac lesions with ductal-dependent systemic blood flow are considered high risk and may increase length of stay and days to achieve full feeds, they are not associated with a higher risk of postoperative necrotizing enterocolitis. Nasogastric route is not associated with a significantly higher risk of necrotizing enterocolitis, but longer length of stay and days to reach full feeds. These findings challenge our perioperative management strategies in caring for these infants, as they may incur more hospital costs and resources without significant medical benefit.