Pulmonary Complications in Patients With Fontan Circulation: JACC Review Topic of the Week

Ali Abdulkarim, Shawn Shaji, Mahmud Elfituri, Megan Gunsaulus, Muhammad A. Zafar, Ali N. Zaidi, Robert H. Pass, Brian Feingold, Geoffrey Kurland, Jacqueline Kreutzer, Rod Ghassemzadeh, Bryan Goldstein, Shawn West, Tarek Alsaied

Research output: Contribution to journalReview articlepeer-review

1 Citation (Scopus)


The Fontan operation has resulted in significant improvement in survival of patients with single ventricle physiology. As a result, there is a growing population of individuals with Fontan physiology reaching adolescence and adulthood. Despite the improved survival, there are long-term morbidities associated with the Fontan operation. Pulmonary complications are common and may contribute to both circulatory and pulmonary insufficiency, leading ultimately to Fontan failure. These complications include restrictive lung disease, sleep abnormalities, plastic bronchitis, and cyanosis. Cyanosis post-Fontan procedure can be attributed to multiple causes including systemic to pulmonary venous collateral channels and pulmonary arteriovenous malformations. This review presents the unique cardiopulmonary interactions in the Fontan circulation. Understanding the cardiopulmonary interactions along with improved recognition and treatment of pulmonary abnormalities may improve the long-term outcomes in this growing patient population. Interventions focused on improving pulmonary function including inspiratory muscle training and endurance training have shown a promising effect post-Fontan procedure.

Original languageEnglish
Pages (from-to)2434-2444
Number of pages11
JournalJournal of the American College of Cardiology
Issue number25
Publication statusPublished - 27 Jun 2023
Externally publishedYes


  • Fontan operation
  • congenital heart disease
  • heart failure
  • pulmonary abnormalities
  • single ventricle


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