Prevalence and cause of early fontan complications: Does the lymphatic circulation play a role?

Reena M. Ghosh, Heather M. Griffis, Andrew C. Glatz, Jonathan J. Rome, Christopher L. Smith, Matthew J. Gillespie, Kevin K. Whitehead, Michael L. O’byrne, David M. Biko, Chitra Ravishankar, Aaron G. Dewitt, Yoav Dori

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36 Scopus citations


BACKGROUND: Recent studies suggest that lymphatic congestion plays a role in development of late Fontan complications, such as protein-losing enteropathy. However, the role of the lymphatic circulation in early post-Fontan outcomes is not well defined. METHODS AND RESULTS: This was a retrospective, single-center study of patients undergoing first-time Fontan completion from 2012 to 2017. The primary outcome was early Fontan complication ≤6 months after surgery, a composite of death, Fontan takedown, extracorporeal membrane oxygenation, chest tube drainage >14 days, cardiac catheterization, readmission, or transplant. Complication causes were assigned to 1 of 4 groups: (1) Fontan circuit obstruction, (2) ventricular dysfunction or atrioventricular valve regurgitation, (3) persistent pleural effusions in the absence of Fontan obstruction or ventricular dysfunc-tion, and (4) chylothorax or plastic bronchitis. T2-weighted magnetic resonance imaging sequences were used to assess for lymphatic perfusion abnormality. The cohort consisted of 238 patients. Fifty-eight (24%) developed early complications: 20 of 58 (34.5%) in group 1, 8 of 58 (14%) in group 2, 18 of 58 (31%) in group 3, and 12 of 58 (20%) in group 4. Preoperative T2 imaging was available for 126 (53%) patients. Patients with high-grade lymphatic abnormalities had 6 times greater odds of developing early complications (P=0.001). CONCLUSIONS: There is substantial morbidity in the early post-Fontan period. Half of those who developed early complications had lymphatic failure or persistent effusions unrelated to structural or functional abnormalities. Preoperative T2 imaging demonstrated that patients with higher-grade lymphatic perfusion abnormalities were significantly more likely to develop early complications. This has implications for risk stratification and optimization of patients before Fontan palliation.

Original languageEnglish
Article numbere015318
JournalJournal of the American Heart Association
Issue number7
StatePublished - 2020


  • Cardiovascular magnetic resonance imaging
  • Congenital heart disease
  • Fontan procedure
  • Lymph
  • Morbidity/mortality


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