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
Icahn School of Medicine at Mount Sinai. University of Pittsburgh Medical Center, Children’s Hospital of Pittsburgh and University of Pittsburgh School of Medicine. University of Cincinnati College of Medicine.
United States
Journal of the American College of Cardiology
J Am Coll Cardiol 2023; 81: 2434-2444
DOI: 10.1016/j.jacc.2023.04.036
Abstract
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.
Category
Pulmonary Arteriovenous Malformations
Review Articles Concerning Pulmonary Vascular Disease
Age Focus: Pediatric Pulmonary Vascular Disease or Adult Pulmonary Vascular Disease
Fresh or Filed Publication: Filed (PHiled). Greater than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: Yes