Hostname: page-component-586b7cd67f-t7czq Total loading time: 0 Render date: 2024-11-24T06:39:39.679Z Has data issue: false hasContentIssue false

The bidirectional Glenn and Fontan procedures—integrated management of the patient with a functionally single ventricle

Published online by Cambridge University Press:  19 August 2008

Richard D. Mainwaring*
Affiliation:
The Cardiac Institute, Children's Hospital–San Diego, San Diego
John J. Lamberti
Affiliation:
The Cardiac Institute, Children's Hospital–San Diego, San Diego
John W. Moore
Affiliation:
The Cardiac Institute, Children's Hospital–San Diego, San Diego
*
Dr. Richard D. Mainwaring, 3030 Children's Way, Suite 310, San Diego, CA 92123, USA. Fax 619-974-8032.

Abstract

The Fontan procedure provides a means of separating the systemic and pulmonary circulations in patients with congenital heart defects characterized by a functionally single ventricle. The procedure has undergone numerous changes since its introduction 25 years ago. Technical modifications have now increased its applicability to virtually all forms of univentricular atrioventricular connection, as well as many hearts with biventricular connections. More recently, philosophical changes have been introduced to address increased rates of morbidity and mortality observed in certain subsets of patients. These changes appear to have improved short- and midterm results. The purpose of this review is to offer our strategy for management of such patients. We will discuss the interrelationship between the bidirectional Glenn and Fontan procedures. We will also focus on a number of observations concerning the possible role of accessory pulmonary blood flow, aortopulmonary collateral arteries, and endocrinologic changes.

Type
Review Article
Copyright
Copyright © Cambridge University Press 1996

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1.Fontan, F, Mounicot, SB, Baudet, E, Simmoneau, J, Gordo, J, Gouffrant, JM. “Correction” de l'atresie tricuspidienne: a rapport de deux cas “corriges” par l'utilisation d'une technique chir-urgicale nouvelle. Ann Chir Thorac Cardiovasc 1971; 10:3947.Google Scholar
2.Mair, DD, Hagler, DJ, Puga, FJ, Schaff, HV, Danielson, GK.Fontan operation in 1,976 patients with tricuspid atresia: results in a proposed new index for patient selection. Circulation 1990; 82(PartII): 164169.Google Scholar
3.de Leval, MR, Kilner, P, Gewillig, M, Bull, C. Total cavopulmo-naiy connection: a logical alternative to atriopulmonary connection for complex Fontan operations. J Thorac Cardiovasc Surg 1988; 96: 682695.CrossRefGoogle ScholarPubMed
4.Mayer, JE Jr, Helgason, H, Jonas, RA, Lang, P, Fargas, FJ, Cook, N, Castaneda, AR. Extending the limits for modified Fontan procedures. J Thorac Cardiovasc Surg 1986; 92: 10211028.CrossRefGoogle ScholarPubMed
5.Mayer, JE Jr, Bridges, ND, Lock, JE, Hanley, FL, Jonas, RA, Castenada, AR. Factors associated with marked reduction in mortality for Fontan operations in patients with single ventricle. J Thorac Cardiovasc Surg 1992; 103: 443452.CrossRefGoogle ScholarPubMed
6.Jacobs, ML, Norwood, WI. Fontan operation: influence of modifications on morbidity and mortality. Ann Thorac Surg 1994; 58: 945952.CrossRefGoogle ScholarPubMed
7.Chin, AJ, Franklin, WH, Andrews, BAA, Norwood, WI. Changes in early geometry after Fontan operation. Ann Thorac Surg 1993; 56: 13591365.CrossRefGoogle ScholarPubMed
8.Castaneda, AR. From Glenn to Fontan a continuing evolution. Circulation 1992; 86(Supp II): II 80II 84.Google Scholar
9.Mainwaring, RD, Lamberti, JJ, Carter, TL Jr. Bidirectional Glenn procedure inpatients under four months of age. J Am Coll Cardiol (Special Issue) 1994; 191A. [Abstract]Google Scholar
10.Reddy, VM, Liddicoat, JR, and Hanley, FL. Primary bidirectional Glenn in patients between one and four months of age. J Am Coll Cardiol (Special Issue) 1994; 8A. [Abstract]Google Scholar
11.Mainwaring, RD, Lamberti, JJ, Uzark, K. The bidirectional Glenn: palliation of the univentricular heart. In: Karp, RB, Laks, H, Wechsler, AS (eds). Advances in Cardiac Surgery, Volume 5. Mosby-Year Book, Inc., St. Louis, 1994, pp 115140.Google Scholar
12.Mainwaring, RD, Lamberti, JJ, Uzark, K, Spicer, RL. Bidirectional Glenn: is accessory pulmonary blood flow good or bad? Circulation 1995; 92(Suppl II): II294II297.CrossRefGoogle ScholarPubMed
13.Moore, JW, Spicer, RL, Perry, JW, Mathewson, JW, Kirkpatrick, SE, George, L, Uzark, K, Mainwaring, RD, Lamberti, JJ. Percutaneous use of scents to correct pulmonary artery stenosis in infants and young children after cavo-pulmonary anastomosis. Am Heart J 1995; 130: 12451249.CrossRefGoogle Scholar
14.Spicer, RL, Uzark, K, Mainwaring, RD, Lamberti, JJ. Palliation of univentricular heart: predictors of outcome. J Am Coll Cardiol 1993; 21: 81A. [Abstract]Google Scholar
15.Spicer, RL, Uzark, K, Cocalis, MW, Moore, JW, Mainwaring, RD, Lamberti, JJ. Aortopulmonary collaterals and prolonged pleural effusions after modified Fontan procedure. Pediatr Cardiol 1994; 15: 256. [Abstract]Google Scholar
16.Barber, G, Helton, JG, Aglira, BA, Chin, AJ, Murphy, JD, Pigott, JD, Norwood, WI. The significance of tricuspid regurgitation in hypoplastic left heart syndrome. Am Heart J 1988; 116: 15631567.CrossRefGoogle ScholarPubMed
17.Humes, RA, Feldt, RH, Porter, CJ, Julsrud, PR, Puga, FJ, Danielson, GK. The modified Fontan operation for asplenia and polysplenia syndromes. J Thorac Cardiovasc Surg 1988; 96:212218.CrossRefGoogle ScholarPubMed
18.Amato, JJ, Cotroneo, JV, Galdieri, RJ, Alboloiras, E, Antillon, J, Vogel, RL. Experience with the polytetrafluoroethylene surgical membrane for pericardia! closure in operations for congenital cardiac defects. J Thorac Cardiovasc Surg 1989; 97: 929934.CrossRefGoogle Scholar
19.Miura, T, Hiramatsu, T, Forbess, JM, Mayer, JE Jr. Effects of elevated coronary sinus pressure on coronary blood flow and left ventricular function: implications after Fontan operation. Circulation 1994; 90(Part II): 11420.Google Scholar
20.Laks, H, Pearl, J, Wu, A, Haas, G, George, B. Experience with the Fontan procedure including use of an adjustable intra-atrial communication. In: Crupi, G, Parenzan, L, Anderson, R (eds). Perspectives in Pediatric Cardiac Surgery, Volume 2. Futura, Mount Kisco, New York, 1989, pp 205208.Google Scholar
21.Kopf, GS, Kleinman, CS, Hijazi, ZM, Fahey, JT, Dewar, ML, Hellenbrand, WE. Fenestrated Fontan operation with delayed transcatheter closure of atrial septal defect. J Thorac Cardiovasc Surg 1992; 103: 10391048.CrossRefGoogle ScholarPubMed
22.Ing, F, Montoya, C, Hsu, D, Quaegebeur, J, Gersony, W. The Non-fenestrated Fontan: hospital course and outcome. J Am Coll Cardiol 1993; 21: 324A. [Abstract]Google Scholar
23.Laks, H. The partial Fontan procedure: a new concept and its clinical application. Circulation 1990; 82: 866867. [Editorial]CrossRefGoogle ScholarPubMed
24.Bridges, ND, Lock, JE, Castenada, AR. Baffle fenestration with subsequent transcatheter closure: modification of the Fontan operation for patients at increased risk. Circulation 1990; 82: 16811689.CrossRefGoogle ScholarPubMed
25.Harake, B, Kuhn, MA, Jarmakani, JM, Laks, H, Al-Khatib, You, Elami, A, Williams, RG. Acute hemodynamic effects of adjustable atrial septal defect closure in the lateral tunnel Fontan procedure. J Am Coll Cardiol 1994; 23: 16711676.CrossRefGoogle ScholarPubMed
26.Stewart, JM, Gewitz, MH, Clark, BJ, Seligman, KP, Romano, A, Zeballos, GA, Chang, A, Murdison, K, Woolf, PK, Norwood, WI. The Role of vasopressin and atrial natriuretic factor in postoperative fluid retention after the Fontan procedure. J Thorac Cardiovasc Surg 1991; 102: 821829.CrossRefGoogle ScholarPubMed
27.Mainwaring, RD, Lamberti, JJ, Moore, JW, Billman, GF, Nelson, JC. A Comparison of the hormonal response following bidirectional Glenn and Fontan procedures. Ann Thorac Surg 57: 5964, 1994.CrossRefGoogle Scholar
28.Mainwaring, RD, Lamberti, JJ, Carter, TL, Moore, JW, Nelson, JC. Renin, angiotensin II, and the development of effusions following bidirectional Glenn and Fontan procedures. J Card Surg 1995; 10: 111118.CrossRefGoogle ScholarPubMed
29.Mainwaring, RD, Lamberti, JJ, Carter, TL, Nelson, JC. Reduction in triiodothyronine levels following modified Fontan procedure. J Card Surg 1994; 9: 322331.CrossRefGoogle ScholarPubMed
30.Salter, DR, Dyke, CM, Wechsler, AS. Triiodothyronine (T3) and cardiovascular therapeutics: a review. J Card Surg 1992; 7: 363374.CrossRefGoogle ScholarPubMed