Digoxin Use Is Associated With Reduced Interstage Mortality in Patients With No History of Arrhythmia After Stage I Palliation for Single Ventricle Heart Disease

Abstract

BackgroundInterstage mortality (IM) remains significant after stage 1 palliation (S1P) for single‐ventricle heart disease (SVD), with many deaths sudden and unexpected. We sought to determine whether digoxin use post‐S1P is associated with reduced IM, utilizing the multicenter database of the National Pediatric Cardiology Quality Improvement Collaborative (NPCQIC).Methods and ResultsFrom June 2008 to July 2013, 816 infants discharged after S1P from 50 surgical sites completed the interstage to stage II palliation, transplant, or IM. Arrhythmia during S1P hospitalization or discharge on antiarrhythmic medications were exclusions (n=270); 2 patients were lost to follow‐up. Two analyses were performed: (1) propensity‐score adjusted logistic regression with IM as outcome and (2) retrospective cohort analysis for patients discharged on digoxin versus not, matched for surgical site and other established IM risk factors. Of 544 study patients, 119 (21.9%) were discharged on digoxin. Logistic regression analysis with propensity score, site‐size group, and digoxin use as predictor variables showed an increased risk of IM in those not discharged on digoxin (odds ratio, 8.6; lower confidence limit, 1.9; upper confidence limit, 38.3; P<0.01). The retrospective cohort analysis for 60 patients on digoxin (matched for site of care, type of S1P, post‐S1P ECMO use, genetic syndrome, discharge feeding route, ventricular function, tricuspid regurgitation, and aortic arch gradient) showed 0% IM in the digoxin at discharge group and an estimated IM difference between the 2 groups of 9% (P=0.04).ConclusionsAmong SVD infants in the NPCQIC database discharged post‐S1P with no history of arrhythmia, use of digoxin at discharge was associated with reduced IM.

Interventional treatments and risk factors in patients born with hypoplastic left heart syndrome in England and Wales from 2000 to 2015

“…Previous focus on this interstage period has enabled the evaluation of therapeutic interventions to reduce mortality, including additional surveillance of patients in home monitoring programmes 6. Furthermore, non-randomised studies of digoxin use between stages 1 and 2 indicate this is linked to reduced mortality 28. Our study data identified further interstage deaths between stages 2 and 3, a less well-explored phase of the patient journey for HLHS.…”

Heart Failure in Pediatric Patients With Congenital Heart Disease

“…For example, the PCGC has not reported the presence of ventricular dysfunction or HF as a distinct or modifying phenotype, and the NPC-QIC has analyzed only cases that are at least moderately abnormal. 68, 110 A clear overarching need is to leverage existing cohorts by combining them to facilitate trials where both primary and secondary questions can be answered. Adopting a learning health system, such as the NPC-QIC, may improve the results of trials performed using existing cohorts since this approach has shown significant increases in patient participation, a common limitation in pediatric heart disease trials.…”

Impact of protein-losing enteropathy in children who underwent the Fontan operation

“…17 This protective impact of digoxin was also seen even among those with no history of arrhythmia. 18 Longer term follow-up questioned the net protective effect of digoxin. 19 A further large study is needed to assess the impact of digoxin on the well-being of children with post-Fontan protein-losing enteropathy to more clearly understand the age or stage at which it should be started.…”