Protein-losing enteropathy and plastic bronchitis after the Fontan procedure

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Varun J Sharma ¹, Ajay J Iyengar ², Diana Zannino ³, Thomas Gentles ⁴, Robert Justo ⁵, David S Celermajer ⁶, Andrew Bullock ⁶, David Winlaw ⁷, Gavin Wheaton ⁸, Luke Burchill ⁹, Rachael Cordina ¹⁰, Yves d’Udekem ¹¹
J Thorac Cardiovasc Surg. 2020 Aug 12;S0022-5223(20)32359-X.
doi: 10.1016/j.jtcvs.2020.07.107. Online ahead of print.
PMID: 32928546;  DOI: 10.1016/j.jtcvs.2020.07.107

Take Home Points:

• Median time of onset of protein-losing enteropathy after Fontan is 5 years.
• Prevalence of protein-losing enteropathy and plastic bronchitis 30 years following after Fontan is 5%.
• Independent predictors for the risk of developing protein-losing enteropathy and plastic bronchitis were right ventricular morphology with HLHS, older age at Fontan, and prolonged pleural effusions after Fontan. Left ventricular morphology was protective.
• Freedom from death/transplantation after diagnosis of protein-losing enteropathy or plastic bronchitis at 5 and 10 years were 91% and 77% respectively.

Comment from Dr. Shaji Menon (Salt Lake City, Utah), section editor of Pediatric Cardiology Journal Watch:   This is a retrospective analysis of 1561 patient from the Australia and New Zealand Fontan registry. A total of 55 patients with protein losing enteropathy/plastic bronchitis were studied. 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.  Protein-losing enteropathy and plastic bronchitis remain severe complications of Fontan circulation specially in patients with a dominant right ventricle.  No significant improvement in transplant-free survival was noted in the current era. A quarter of patients require heart transplantation within a few years of diagnosis.