Protein-losing enteropathy and plastic bronchitis after the Fontan procedure

ObjectivesProtein losing enteropathy and plastic bronchitis are severe complications in Fontan circulation, with 5-year survival ranging from 46% to 88%. We report risk factors outcomes of protein patients undergoing the Fontan.MethodsWe performed a retrospective analysis 1561 Australia New Zealand Registry. Two end points were death cardiac transplantation examined Cox regression (if no competing risks) or cumulative incidence curves cause-specific Cs regression.ResultsA total 55 enteropathy/plastic included. Their median age at was 5.7 years, time onset after for 5.0 years 1.7 years. Independent predictors developing right-ventricular morphology hypoplastic left-heart syndrome (hazard ratio, 2.30; confidence interval, 1.12-4.74), older 1.13; 1.03-1.23), pleural effusions 2.43; 1.09-5.41); left-ventricular protective 0.36; 0.18-0.70). In population, freedom diagnosis 5, 10, 15 70% (confidence 58-85), 65% 51-83), 43% 26-73), respectively; only 1.23; 1.01-1.52) an independent predictor. Twenty-six surgical interventions 20 patients, comprising revisions (n = 5), fenestrations 11), conversions atrioventricular valve repairs 3), hepatic vein diversion 2).ConclusionsProtein remain complications, preferably affecting dominant right single ventricle, being predictor poorer prognosis. Heart remains ultimate treatment, 30% dying requiring within 5 remaining stable long periods.