Protein losing enteropathy and plastic bronchitis are severe complications in Fontan circulation, with 5-year survival ranging from 46% to 88%. We report risk factors and outcomes of protein losing enteropathy and plastic bronchitis in patients undergoing the Fontan.
We performed a retrospective analysis of 1561 patients from the Australia New Zealand Fontan Registry. Two end points were death and cardiac transplantation examined with Cox regression (if no competing risks) or cumulative incidence curves and cause-specific Cs regression.
A total of 55 patients with protein losing enteropathy/plastic bronchitis were included. Their median age at the Fontan was 5.7 years, and time to onset after the Fontan for protein losing enteropathy was 5.0 years and plastic bronchitis was 1.7 years. Independent predictors for developing protein losing enteropathy/plastic bronchitis were right-ventricular morphology with hypoplastic left-heart syndrome (hazard ratio, 2.30; confidence interval, 1.12-4.74), older age at Fontan (hazard ratio, 1.13; confidence interval, 1.03-1.23), and pleural effusions after Fontan (hazard ratio, 2.43; confidence interval, 1.09-5.41); left-ventricular morphology was protective (hazard ratio, 0.36; confidence interval, 0.18-0.70). In the protein losing enteropathy/plastic bronchitis population, freedom from death or transplantation after protein losing enteropathy/plastic bronchitis diagnosis at 5, 10, and 15 years was 70% (confidence interval, 58-85), 65% (confidence interval, 51-83), and 43% (confidence interval, 26-73), respectively; only older age (hazard ratio, 1.23; confidence interval, 1.01-1.52) was an independent predictor. Twenty-six surgical interventions were performed in 20 patients, comprising Fontan revisions (n = 5), fenestrations (n = 11), Fontan conversions (n = 5), atrioventricular valve repairs (n = 3), and hepatic vein diversion (n = 2).
Protein losing enteropathy and plastic bronchitis remain severe complications, preferably affecting patients with dominant right single ventricle, with older age at Fontan being a predictor of developing protein losing enteropathy/plastic bronchitis and poorer prognosis. Heart transplantation remains the ultimate treatment, with 30% dying or requiring transplantation within 5 years, and the remaining being stable for long periods.
Abbreviations and Acronyms:ANZFR (Australia and New Zealand Fontan Registry), AV (atrioventricular), CI (confidence interval), HLHS (hypoplastic left heart syndrome), HR (hazard ratio), IQR (interquartile range), PB (plastic bronchitis), PLE (protein losing enteropathy), RV (right ventricular)
To read this article in full you will need to make a payment
Purchase one-time access:Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
One-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:Subscribe to The Journal of Thoracic and Cardiovascular Surgery
Already a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
- Evaluation and management of the child and adult with Fontan circulation: a scientific statement from the American Heart Association.Circulation. 2019; 140: e234-e284
- Protein-losing enteropathy and the Fontan operation.Nutr Clin Pract. 2012; 27: 375-384
- Plastic bronchitis after extracardiac Fontan operation: further evidence.Ann Thorac Surg. 2013; 95: 2214-2215
- Protein-losing enteropathy after the Fontan operation: an international multicenter study. PLE study group.J Thorac Cardiovasc Surg. 1998; 115: 1063-1073
- Clinical outcomes and improved survival in patients with protein-losing enteropathy after the Fontan operation.J Am Coll Cardiol. 2014; 64: 54-62
- A multifaceted approach to the management of plastic bronchitis after cavopulmonary palliation.Ann Thorac Surg. 2014; 98: 634-640
- Low pulmonary vascular compliance predisposes post-Fontan patients to protein-losing enteropathy.Int J Cardiol. 2013; 165: 454-457
- The Australia and New Zealand Fontan registry: description and initial results from the first population-based Fontan registry.Intern Med J. 2014; 44: 148-155
- Longitudinal outcomes of patients with single ventricle after the Fontan procedure.J Am Coll Cardiol. 2017; 69: 2735-2744
- Protein-losing enteropathy after the Fontan operation.J Thorac Cardiovasc Surg. 1996; 112: 672-680
- Successful treatment of plastic bronchitis by selective lymphatic embolization in a Fontan patient.Pediatrics. 2014; 134: e590-e595
- Protein-losing enteropathy in patients with congenital heart disease.J Am Coll Cardiol. 2017; 69: 2929-2937
Published online: August 11, 2020
Accepted: July 31, 2020
Received in revised form: July 26, 2020
Received: January 21, 2020
Data collected are part of the Australia New Zealand Fontan Registry. HREC-Ethics Number 36260.
Crown Copyright © 2020 Published by Elsevier Inc. on behalf of The American Association for Thoracic Surgery
ScienceDirectAccess this article on ScienceDirect
- Commentary: Late adverse outcomes of the Fontan circulation: A PLEa for standardization of diagnostic criteriaThe Journal of Thoracic and Cardiovascular SurgeryVol. 161Issue 6
- PreviewProtein-losing enteropathy (PLE) and plastic bronchitis (PB) are 2 of the most feared complications in those with a Fontan circulation.1,2 Although treatment options for each exist, the success of medications and procedural interventions is often only transient, with only heart transplantation offering the possibility of long-term cure. However, late referral for transplant after months or years of hypoalbuminemia due to PLE may make patients ineligible for transplantation or at increased risk of waitlist and perioperative mortality.
- Commentary: Lymphatic fluid flows uphill in both hemispheres—and leaks in the same placesThe Journal of Thoracic and Cardiovascular SurgeryVol. 161Issue 6
- PreviewSharma and colleagues1 provide a comprehensive description of protein-losing enteropathy (PLE) and plastic bronchitis (PB) in patients with Fontan circulation—yet another testament to the utility of their Fontan registry. In a cohort of 1561 patients spanning 4 decades, the authors report 48 patients with PLE only, 4 with PB only, and 3 with both. Right ventricular morphology with hypoplastic left heart syndrome (hazard ratio [HR] of 2.3), prolonged effusion after the Fontan (HR of 2.4), and older age at Fontan (2-year increment, HR of 1.13) were associated with development of PLE/PB, whereas once PLE/PB was diagnosed, only older age at Fontan was associated with death or transplant (HR of 1.23).