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

  • Richard D. Mainwaring (a1), John J. Lamberti (a1) and John W. Moore (a1)


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.


Corresponding author

Dr. Richard D. Mainwaring, 3030 Children's Way, Suite 310, San Diego, CA 92123, USA. Fax 619-974-8032.


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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.
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. 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.
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.
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.
6.Jacobs, ML, Norwood, WI. Fontan operation: influence of modifications on morbidity and mortality. Ann Thorac Surg 1994; 58: 945952.
7.Chin, AJ, Franklin, WH, Andrews, BAA, Norwood, WI. Changes in early geometry after Fontan operation. Ann Thorac Surg 1993; 56: 13591365.
8.Castaneda, AR. From Glenn to Fontan a continuing evolution. Circulation 1992; 86(Supp II): II 80II 84.
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]
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]
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.
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.
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.
14.Spicer, RL, Uzark, K, Mainwaring, RD, Lamberti, JJ. Palliation of univentricular heart: predictors of outcome. J Am Coll Cardiol 1993; 21: 81A. [Abstract]
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]
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.
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.
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.
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.
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.
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.
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]
23.Laks, H. The partial Fontan procedure: a new concept and its clinical application. Circulation 1990; 82: 866867. [Editorial]
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.
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.
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.
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.
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.
29.Mainwaring, RD, Lamberti, JJ, Carter, TL, Nelson, JC. Reduction in triiodothyronine levels following modified Fontan procedure. J Card Surg 1994; 9: 322331.
30.Salter, DR, Dyke, CM, Wechsler, AS. Triiodothyronine (T3) and cardiovascular therapeutics: a review. J Card Surg 1992; 7: 363374.


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

  • Richard D. Mainwaring (a1), John J. Lamberti (a1) and John W. Moore (a1)


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