Abstract
SETTING—Tertiary adult congenital cardiac referral centre. DESIGN—Retrospective cross sectional analysis. OBJECTIVES—To report our 20 year experience with adult Fontan operations, and to compare late outcome in patients with single ventricle with definitive aortopulmonary or cavopulmonary shunt palliation. PATIENTS AND MAIN OUTCOME MEASURES—Patients older than 18 years undergoing Fontan operation between 1 January 1982 and 31 December 1998 were identified. Mortality and late outcome were derived from hospital records. These patients were compared with a cohort of 50 adults with single ventricle who had not undergone a Fontan operation. RESULTS—61 adults, median age 36 years (range 18-47 years), with a median follow up of 10 years (range 0-21 years) were identified. Actuarial survival was 80% at one year, 76% at five years, 72% at 10 years, and 67% at 15 years. Compared with before the Fontan operation, more patients were in New York Heart Association (NYHA) functional class I or II at the latest follow up (80% v 58%, p < 0.001). Systolic ventricular function deteriorated during follow up such that 34% had moderate to severe ventricular dysfunction at the latest follow up compared with 5% before Fontan (p < 0.001). Arrhythmia increased with time (10% before Fontan v 57% after 10 years, p < 0.001). Fontan patients had improved NYHA functional class, ventricular function, atrioventricular regurgitation, and fewer arrhythmias than the non-Fontan group at the latest follow up. CONCLUSION—The Fontan operation in adults has acceptable early and late mortality. Functional class, systolic ventricular function, atrioventricular regurgitation, and arrhythmia deteriorate late after surgery but to a lesser degree than in non-Fontan patients with a single ventricle. Keywords: Fontan operation; univentricular heart; cavopulmonary shunt; aortopulmonary shunt
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- Cheung Y. F., Penny D. J., Redington A. N. Serial assessment of left ventricular diastolic function after Fontan procedure. Heart. 2000 Apr;83(4):420–424. doi: 10.1136/heart.83.4.420. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Colucci W. S., Ribeiro J. P., Rocco M. B., Quigg R. J., Creager M. A., Marsh J. D., Gauthier D. F., Hartley L. H. Impaired chronotropic response to exercise in patients with congestive heart failure. Role of postsynaptic beta-adrenergic desensitization. Circulation. 1989 Aug;80(2):314–323. doi: 10.1161/01.cir.80.2.314. [DOI] [PubMed] [Google Scholar]
- Driscoll D. J., Offord K. P., Feldt R. H., Schaff H. V., Puga F. J., Danielson G. K. Five- to fifteen-year follow-up after Fontan operation. Circulation. 1992 Feb;85(2):469–496. doi: 10.1161/01.cir.85.2.469. [DOI] [PubMed] [Google Scholar]
- Fishberger S. B., Wernovsky G., Gentles T. L., Gauvreau K., Burnett J., Mayer J. E., Jr, Walsh E. P. Factors that influence the development of atrial flutter after the Fontan operation. J Thorac Cardiovasc Surg. 1997 Jan;113(1):80–86. doi: 10.1016/s0022-5223(97)70402-1. [DOI] [PubMed] [Google Scholar]
- Fontan F., Baudet E. Surgical repair of tricuspid atresia. Thorax. 1971 May;26(3):240–248. doi: 10.1136/thx.26.3.240. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Fontan F., Kirklin J. W., Fernandez G., Costa F., Naftel D. C., Tritto F., Blackstone E. H. Outcome after a "perfect" Fontan operation. Circulation. 1990 May;81(5):1520–1536. doi: 10.1161/01.cir.81.5.1520. [DOI] [PubMed] [Google Scholar]
- Gates R. N., Laks H., Drinkwater D. C., Jr, Lam L., Blitz A., Child J. S., Perloff J. K. The Fontan procedure in adults. Ann Thorac Surg. 1997 Apr;63(4):1085–1090. doi: 10.1016/s0003-4975(96)01256-8. [DOI] [PubMed] [Google Scholar]
- Gatzoulis M. A., Munk M. D., Williams W. G., Webb G. D. Definitive palliation with cavopulmonary or aortopulmonary shunts for adults with single ventricle physiology. Heart. 2000 Jan;83(1):51–57. doi: 10.1136/heart.83.1.51. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Humes R. A., Mair D. D., Porter C. B., Puga F. J., Schaff H. V., Danielson G. K. Results of the modified Fontan operation in adults. Am J Cardiol. 1988 Mar 1;61(8):602–604. doi: 10.1016/0002-9149(88)90772-2. [DOI] [PubMed] [Google Scholar]
- Jahangiri M., Ross D. B., Redington A. N., Lincoln C., Shinebourne E. A. Thromboembolism after the Fontan procedure and its modifications. Ann Thorac Surg. 1994 Nov;58(5):1409–1414. doi: 10.1016/0003-4975(94)91924-0. [DOI] [PubMed] [Google Scholar]
- Kirklin J. K., Blackstone E. H., Kirklin J. W., Pacifico A. D., Bargeron L. M., Jr The Fontan operation. Ventricular hypertrophy, age, and date of operation as risk factors. J Thorac Cardiovasc Surg. 1986 Dec;92(6):1049–1064. [PubMed] [Google Scholar]
- Kopf G. S., Laks H., Stansel H. C., Hellenbrand W. E., Kleinman C. S., Talner N. S. Thirty-year follow-up of superior vena cava-pulmonary artery (Glenn) shunts. J Thorac Cardiovasc Surg. 1990 Nov;100(5):662–671. [PubMed] [Google Scholar]
- Mair D. D., Hagler D. J., Julsrud P. R., Puga F. J., Schaff H. V., Danielson G. K. Early and late results of the modified Fontan procedure for double-inlet left ventricle: the Mayo Clinic experience. J Am Coll Cardiol. 1991 Dec;18(7):1727–1732. doi: 10.1016/0735-1097(91)90511-7. [DOI] [PubMed] [Google Scholar]
- Marcelletti C., Corno A., Giannico S., Marino B. Inferior vena cava-pulmonary artery extracardiac conduit. A new form of right heart bypass. J Thorac Cardiovasc Surg. 1990 Aug;100(2):228–232. [PubMed] [Google Scholar]
- Mayer J. E., Jr, Helgason H., Jonas R. A., Lang P., Vargas F. J., Cook N., Castaneda A. R. Extending the limits for modified Fontan procedures. J Thorac Cardiovasc Surg. 1986 Dec;92(6):1021–1028. [PubMed] [Google Scholar]
- McCarthy K., Tang A. T., Dalrymple-Hay M. J., Haw M. P. Ventricular thrombosis and systemic embolism in bodybuilders: etiology and management. Ann Thorac Surg. 2000 Aug;70(2):658–660. doi: 10.1016/s0003-4975(00)01572-1. [DOI] [PubMed] [Google Scholar]
- Moodie D. S., Ritter D. G., Tajik A. H., McGoon D. C., Danielson G. K., O'Fallon W. M. Long-term follow-up after palliative operation for univentricular heart. Am J Cardiol. 1984 Jun 1;53(11):1648–1651. doi: 10.1016/0002-9149(84)90595-2. [DOI] [PubMed] [Google Scholar]
- Peters N. S., Somerville J. Arrhythmias after the Fontan procedure. Br Heart J. 1992 Aug;68(2):199–204. doi: 10.1136/hrt.68.8.199. [DOI] [PMC free article] [PubMed] [Google Scholar]
- de Leval M. R., Kilner P., Gewillig M., Bull C. Total cavopulmonary connection: a logical alternative to atriopulmonary connection for complex Fontan operations. Experimental studies and early clinical experience. J Thorac Cardiovasc Surg. 1988 Nov;96(5):682–695. [PubMed] [Google Scholar]