Abstract
Objectives
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.
Methods
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.
Results
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).
Conclusions
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.
Graphical abstract
Graphical Abstract
Key Words
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
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References
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Article info
Publication history
Published online: August 11, 2020
Accepted:
July 31,
2020
Received in revised form:
July 26,
2020
Received:
January 21,
2020
Footnotes
Data collected are part of the Australia New Zealand Fontan Registry. HREC-Ethics Number 36260.
Identification
Copyright
Crown Copyright © 2020 Published by Elsevier Inc. on behalf of The American Association for Thoracic Surgery
ScienceDirect
Access this article on ScienceDirectLinked Article
- 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.
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- 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).
- Full-Text
- Preview
