The Fontan procedure for tricuspid atresia: early and late results of a 25-year experience with 216 patients.

Published on Mar 1, 2001in Journal of the American College of Cardiology20.589
· DOI :10.1016/S0735-1097(00)01164-5
Douglas D. Mair53
Estimated H-index: 53
(Mayo Clinic),
Francisco J. Puga73
Estimated H-index: 73
(Mayo Clinic)
+ 0 AuthorsGordon K. Danielson10
Estimated H-index: 10
(Mayo Clinic)
Sources
Abstract
OBJECTIVES We assessed the operative and late mortality and the present clinical status of 216 patients with tricuspid atresia who had a nonfenestrated Fontan procedure performed at the Mayo Clinic in the 25-year period 1973 to 1998. BACKGROUND The Fontan operation eliminates the systemic hypoxemia and ventricular volume overload characteristic of prior forms of palliation. However, it originally did so at the cost of systemic venous and right atrial hypertension, and the long-term effects of this “price” were unknown when the procedure was initially proposed. METHODS We reviewed the clinical records of the 216 patients retrospectively. These were arbitrarily grouped into early (1973 through 1980), middle (1981 through 1987) and late (1988 through 1997) surgical eras. Patient outcome was also analyzed according to age at surgery. Operative and late mortality rates were determined and present clinical status was ascertained in 167 of 171 surviving patients. RESULTS Overall survival was 79%. Operative mortality steadily declined and was 2% (one of 58 patients) during the most recent decade. Late survival also continues to improve. Age at operation had no effect on operative mortality, and late mortality was significantly increased only in patients who were operated on at age 18 years or older. Eighty-nine percent of surviving patients are currently in New York Heart Association class I or II. CONCLUSIONS The initial 25-year experience with the nonfenestrated Fontan procedure for tricuspid atresia has been gratifying, with most survivors now leading lives of good quality into adulthood. These results justify continued application of this procedure for children born with tricuspid atresia.
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#2V. Mohan Reddy (UCSF: University of California, San Francisco)H-Index: 59
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Abstract Background: Among the modifications of the Fontan operation, the extracardiac approach may offer the greatest potential for optimizing early postoperative ventricular and pulmonary vascular function, insofar as it can be performed with short periods of normothermic partial cardiopulmonary bypass and without cardioplegic arrest in most cases. In this study, we reviewed our experience with the extracardiac conduit Fontan operation, with a focus on early postoperative outcomes. Methods and...
132 CitationsSource
#1Kritvikrom Durongpisitkul (Loyola University Medical Center)H-Index: 1
#1Kritvikrom Durongpisitkul (Mayo Clinic)H-Index: 3
Last. David J. Driscoll (Loyola University Medical Center)H-Index: 1
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Background—The objectives of our study were to determine the frequency of supraventricular tachyarrhythmias (SVTAs) among modifications of the Fontan operation and identify risk factors for developing SVTA. Methods and Results—The population consisted of all patients who had any modification of the Fontan operation at the Mayo Clinic between 1985 and 1993. Clinically significant SVTAs were those requiring initiation or change of antiarrhythmic treatment, and they were divided into early SVTAs (<...
146 CitationsSource
#1Amy M. Kelly (Mayo Clinic)H-Index: 2
#2Robert H. FeldtH-Index: 31
Last. Gordon K. Danielson (Mayo Clinic)H-Index: 10
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Protein-losing enteropathy (PLE) is a serious complication of the Fontan operation and is associated with pronounced mortality. Medical management of PLE has been only partially successful. A recent report noted dramatic improvement in patients with PLE within 3 weeks of subcutaneous administration of heparin. We report a case of reversal of PLE with resolution of clinical symptoms and normalization of serum albumin, total protein, and fecal α 1 ,-antitrypsin values after several months of hepar...
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#1Daphne T. Hsu (Columbia University)H-Index: 54
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Background A completed Fontan circulation is the goal in the management of patients with single-ventricle physiology. To achieve this end, a two-stage rather than a single-stage approach is carried out routinely at many centers. Some groups have advocated baffle fenestration for virtually all patients to minimize post-Fontan complications. Other centers perform single-stage Fontan operations and do not fenestrate. Thus controversies have arisen regarding the indications for the staged procedure ...
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#1Jon P. Donnelly (UM: University of Michigan)H-Index: 2
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Abstract We studied three pediatric patients with protein-losing enteropathy in conjunction with univentricular hearts and right atrial to pulmonary artery anastomosis (Fontan operation) before and during heparin therapy. Each patient showed dramatic improvements in symptoms, marked elevations in serum albumin levels, and quantitative reversal of enteric protein loss within a few weeks of beginning therapy. These findings suggest that heparin may be an important treatment for this poorly underst...
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Background Atrial distension after Fontan operation may predispose to arrhythmia. Modifications aimed at decreasing the extent of right atrial distension (total cavopulmonary connection, TCPC) have been associated with a lower incidence of early arrhythmia, but serial evaluation has not been performed. Methods and Results All 119 patients undergoing TCPC between March 1987 and December 1993 were enrolled in a prospective study to evaluate the incidence and determinants of arrhythmia by use of am...
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#1Robert H. Feldt (Mayo Clinic)H-Index: 31
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Abstract Patients were observed after the Fontan operation to determine the frequency and severity of protein-losing enteropathy. A total of 427 patients who survived for 30 days after the Fontan operation, performed between 1973 and January 1987, were analyzed and, thus far, protein-losing enteropathy has developed in 47 of 427. The cumulative risk for the development of protein-losing enteropathy by 10 years was 13.4% among 30-day survivors, and 5-year survival after the diagnosis was 46%. Hem...
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#1Thomas M. Zellers (UTSW: University of Texas Southwestern Medical Center)H-Index: 21
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Protein-losing enteropathy occurs in up to 10% of patients following the modified Fontan procedure. Treatment is still controversial. We describe a female adolescent who developed protein-losing enteropathy 4 years after a modified Fontan procedure. Treatment with oral prednisone attenuated the protein loss with subsequent normalization of her serum total protein and albumin levels. Discontinuation of prednisone therapy was associated with relapse, which was again treated successfully with low-d...
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Abstract The purpose of this study was to determine the effects of newer Fontan modifications (lateral tunnel with or without fenestration) and patient's age at surgery on the incidence and impact of symptomatic postoperative early and intermediate arrhythmias. Modifications to the Fontan procedure are used to decrease postoperative complications, and the Fontan procedure is now being performed on younger patients to reduce age-related changes in ventricular function. A retrospective review was ...
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