Impact of Pharmacotherapy on Interstage Mortality and Weight Gain in Children with Single Ventricle

OBJECTIVE.: Infants with single ventricle physiology have a high mortality and poor somatic growth during the interstage period. We retrospectively assessed the impact of pharmacotherapy in this population using a multicenter database. DESIGN AND RESULTS.: Records for 395 patients (63.5% boys) with single ventricle were obtained from the National Pediatric Cardiology Quality Improvement Collaborative registry. Median of five medications were prescribed per patient at discharge after stage 1 palliation (interquartile range 3 to 6); the most common medications being aspirin (95.7%), diuretics (90.4%), angiotensin convertase enzyme inhibitors (37.7%), proton pump inhibitors (33.4%), H2 receptor blockers (30.6%), and digoxin (27.6%). Interstage mortality was 9.4%. Digoxin use was associated with lower risk of death (P =.03) on univariable analysis, however no single medication was an independent predictor on regression analysis. Change in weight-for-age z-score was studied as outcome of somatic growth with 36.3% patients showing a decrease during the interstage period. Total number of medications prescribed to a patient showed a negative correlation with the interstage change in z-score (r = -0.19, P =.002). On univariable comparisons, use of metoclopramide and lansoprazole were associated with decreased z-score (P =.004 and.041, respectively) although linear regression failed to identify any agent as independent predictor. CONCLUSIONS.: Children with single ventricle have high mortality and a profound medication burden. No individual medication is independently associated with better survival or weight gain during interstage period. Despite widespread use, proton pump inhibitors and prokinetic agents are not associated with better outcomes and may be associated with poor growth.