This Article Full Text Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Request Permissions Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Rychik, J. Articles by Jacobs, M. L. Search for Related Content PubMed PubMed Citation Articles by Rychik, J. Articles by Jacobs, M. L. Pubmed/NCBI databases Medline Plus Health Information Pleural Disorders

(Circulation. 1997;96:33-36.)
© 1997 American Heart Association, Inc.

Articles

Late Surgical Fenestration for Complications After the Fontan Operation

Jack Rychik, MD; Jonathan J. Rome, MD; ; Marshall L. Jacobs, MD

From the Divisions of Cardiology and Cardiothoracic Surgery, Children's Hospital of Philadelphia, and the Departments of Pediatrics and Surgery, University of Pennsylvania School of Medicine, Philadelphia. Dr Jacobs is now at the Department of Surgery, Deborah Heart and Lung Center, Browns Mills, NJ.

Correspondence to Jack Rychik, MD, Division of Cardiology, The Children's Hospital of Philadelphia, 34th St and Civic Center Blvd, Philadelphia, PA 19104. E-mail jrychik{at}mail.med.upenn.edu

Background Significant morbidity after Fontan operation results in either takedown, heart transplantation, or death. Initial creation of a fenestration results in less morbidity and mortality; however, the role of late creation of a fenestration in aiding patients manifesting morbidity after an initial nonfenestrated Fontan operation is unclear.

Methods and Results We reviewed our experience with late creation of a surgical fenestration in 9 patients (5.2±3.1 years old) exhibiting chronic effusions (n=4) or protein-losing enteropathy (PLE) (n=5) after lateral tunnel-type Fontan operation. Patients with effusions had creation via coronary punch of two or three 3-mm defects; patients with PLE had creation of a large, 5-mm defect. One child with effusions and multisystem organ failure before fenestration died 7 weeks after surgery secondary to low cardiac output; the other 3 had resolution of effusions within 4 to 6 weeks. Of the 5 with PLE, 3 had normalization of serum proteins and resolution of symptoms at 2 to 6 weeks. The 2 failures had arterial saturations >89% after surgery. Follow-up was from 25 to 30 months. Spontaneous closure of defects occurred in all 3 with effusions. No return of symptoms was noted in 2; however, the third reaccumulated effusions and has undergone refenestration with a large defect. All 3 patients with PLE have remained asymptomatic with patency of the fenestration (4 to 5 mm on echocardiography) and arterial saturation 85% for >2 years.

Conclusions Late surgical creation of fenestration results in resolution of morbidity after Fontan operation. Improvement is related to the degree of right-to-left shunt created.

Key Words: heart defects, congenital • Fontan procedure • enteropathy • edema

This article has been cited by other articles:

				R. J. Sommer, Z. M. Hijazi, and J. F. Rhodes Pathophysiology of Congenital Heart Disease in the Adult: Part III: Complex Congenital Heart Disease Circulation, March 11, 2008; 117(10): 1340 - 1350. [Full Text] [PDF]

				A. M. Ostrow, H. Freeze, and J. Rychik Protein-Losing Enteropathy After Fontan Operation: Investigations Into Possible Pathophysiologic Mechanisms Ann. Thorac. Surg., August 1, 2006; 82(2): 695 - 700. [Abstract] [Full Text] [PDF]

				V. M. Tak, S. Al-Jureidini, I. Balfour, M. Hohenberg, B. Kountzman, and A. C. Fiore An Alternative Method to Fenestrate the Extracardiac Fontan Conduit in a Patient with Atrial Situs Inversus and Levocardia Ann. Thorac. Surg., February 1, 2006; 81(2): 746 - 748. [Abstract] [Full Text] [PDF]

				M. Petko, R. J. Myung, G. Wernovsky, M. I. Cohen, J. Rychik, S. C. Nicolson, J. W. Gaynor, and T. L. Spray Surgical reinterventions following the Fontan procedure Eur. J. Cardiothorac. Surg., August 1, 2003; 24(2): 255 - 259. [Abstract] [Full Text] [PDF]

				T.-Y. Hsia, S. Khambadkone, A.N. Redington, and M.R. de Leval Effect of fenestration on the sub-diaphragmatic venous hemodynamics in the total-cavopulmonary connection Eur. J. Cardiothorac. Surg., June 1, 2001; 19(6): 785 - 792. [Abstract] [Full Text] [PDF]

				M. I. Cohen, L. A. Rhodes, G. Wernovsky, J. W. Gaynor, T. L. Spray, and J. Rychik Atrial pacing: An alternative treatment for protein-losing enteropathy after the Fontan operation J. Thorac. Cardiovasc. Surg., March 1, 2001; 121(3): 582 - 583. [Full Text] [PDF]

				L. D. Thompson, E. Petrossian, D. B. McElhinney, N. A. Abrikosova, P. Moore, V. M. Reddy, and F. L. Hanley Is it necessary to routinely fenestrate an extracardiac Fontan? J. Am. Coll. Cardiol., August 1, 1999; 34(2): 539 - 544. [Abstract] [Full Text] [PDF]

				J Therrien, G D Webb, and M A Gatzoulis Reversal of protein losing enteropathy with prednisone in adults with modified Fontan operations: long term palliation or bridge to cardiac transplantation? Heart, August 1, 1999; 82(2): 241 - 243. [Abstract] [Full Text] [PDF]

				J. J. Lamberti and K. C. Uzark The Fontan operation Ann. Thorac. Surg., May 1, 1999; 67(5): 1523 - 1524. [Full Text] [PDF]

				A. C. Lardo, S. A. Webber, I. Friehs, P. J. del Nido, and E. G. Cape FLUID DYNAMIC COMPARISON OF INTRA-ATRIAL AND EXTRACARDIAC TOTAL CAVOPULMONARY CONNECTIONS J. Thorac. Cardiovasc. Surg., April 1, 1999; 117(4): 697 - 704. [Abstract] [Full Text] [PDF]