Abstract
Normal appearances of Fontan circulation, as established with older and with more recent techniques, are described, as well as complications and signs of failure.
The Fontan procedure refers to any operation that results in the flow of systemic venous blood to the lungs without passing through a ventricle. It is performed to treat several complex congenital heart abnormalities including tricuspid atresia, pulmonary atresia with intact ventricular septum, hypoplastic left heart syndrome, and double-inlet ventricle. The original Fontan procedure included direct anastomosis of the right atrium to the main pulmonary artery; however, multiple modifications have been employed. Creation of Fontan circulation is palliative in nature, with good results in patients with ideal hemodynamics and substantial morbidity and mortality in those with poor hemodynamics. Complications of Fontan circulation include exercise intolerance, ventricular failure, right atrium dilatation and arrhythmia, systemic and hepatic venous hypertension, portal hypertension, coagulopathy, pulmonary arteriovenous malformation, venovenous shunts, and lymphatic dysfunction (eg, ascites, edema, effusion, protein-losing enteropathy, and plastic bronchitis). Magnetic resonance imaging is best for postoperative evaluation of patients who underwent the Fontan procedure, and cardiac transplantation remains the only definitive treatment for those with failing Fontan circulation.
© RSNA, 2011
References
- 1 . Surgical repair of tricuspid atresia. Thorax 1971;26(3):240–248. Crossref, Medline, Google Scholar
- 2 . The Fontan circulation: a challenge to William Harvey? Nat Clin Pract Cardiovasc Med 2005;2(4):202–208. Crossref, Medline, Google Scholar
- 3 . The Fontan circulation. Heart 2005;91(6):839–846. Crossref, Medline, Google Scholar
- 4 . Total cavopulmonary connection: a logical alternative to atriopulmonary connection for complex Fontan operations: experimental studies and early clinical experience. J Thorac Cardiovasc Surg 1988;96(5):682–695. Crossref, Medline, Google Scholar
- 5 . Hemi-Fontan procedure for hypoplastic left heart syndrome: outcome and suitability for Fontan. Ann Thorac Surg 1999;68(4):1361–1367; discussion 1368. Crossref, Medline, Google Scholar
- 6 . Fenestration improves clinical outcome of the Fontan procedure: a prospective, randomized study. Circulation 2002;105(2):207–212. Crossref, Medline, Google Scholar
- 7 . The Fontan pathway: what's down the road? Ann Pediatr Cardiol 2008;1(2):83–92. Crossref, Medline, Google Scholar
- 8 . Magnetic resonance flow analysis of classic and extracardiac Fontan procedures: the seesaw sign. Int J Cardiovasc Imaging 2004;20(5):397–405; discussion 407–408. Crossref, Medline, Google Scholar
- 9 . Hepatic changes in the failing Fontan circulation. Heart 2007;93(5):579–584. Crossref, Medline, Google Scholar
- 10 . Progression of liver pathology in patients undergoing the Fontan procedure: chronic passive congestion, cardiac cirrhosis, hepatic adenoma, and hepatocellular carcinoma. J Thorac Cardiovasc Surg 2005;129(6):1348–1352. Crossref, Medline, Google Scholar
- 11 . Extent of aortopulmonary collateral blood flow as a risk factor for Fontan operations. Ann Thorac Surg 1995;59(2):433–437. Crossref, Medline, Google Scholar
- 12 . Thromboembolic complications after Fontan procedures: the role of prophylactic anticoagulation. J Thorac Cardiovasc Surg 1998;115(3):493–498. Crossref, Medline, Google Scholar
- 13 . Chylous bronchial casts after Fontan operation. Circulation 2001;103(7):1031–1033. Crossref, Medline, Google Scholar
- 14 . Plastic bronchitis: is thoracic duct ligation a real surgical option? Ann Thorac Surg 2006;81(6):2281–2283. Crossref, Medline, Google Scholar
Article History
Received: Feb 12 2010Revision requested: June 21 2010
Revision received: Oct 7 2010
Accepted: Oct 25 2010
Published online: Mar 7 2011
Published in print: Mar 2011
