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Improving results of the Fontan procedure in patients with heterotaxy syndrome
Cited 49 time in
Web of Science
Cited 56 time in Scopus
- Authors
- Issue Date
- 2006-09-26
- Publisher
- Elsevier
- Citation
- Ann Thorac Surg. 2006 Oct;82(4):1245-51.
- Keywords
- Arrhythmias, Cardiac/etiology ; Child ; Child, Preschool ; Female ; Heart Defects, Congenital/mortality/*surgery ; Humans ; Infant ; Male ; Prognosis ; Reoperation ; Retrospective Studies ; Survival Analysis ; Syndrome ; Treatment Outcome ; Fontan Procedure/adverse effects
- Abstract
- BACKGROUND: The Fontan operation in patients with heterotaxy syndrome has been associated with a poor prognosis. We studied whether the outcome of those patients with heterotaxy syndrome improved compared with those who did not have the syndrome after an extracardiac Fontan operation. METHODS: A retrospective review was performed on 185 patients who had undergone an extracardiac Fontan operation between 1996 and 2005. RESULTS: Sixty-two of the patients had heterotaxy syndrome (33.5%). Forty-one had right isomerism and 21 had left isomerism. Heterotaxy syndrome was commonly associated with a morphologic right ventricle (59.7%), a common atrioventricular valve (72.6%), an interrupted inferior vena cava (25.8%), a separate hepatic vein (30.6%), and extracardiac pulmonary venous drainage (16.1%). The hospital mortality rate was higher in the heterotaxy syndrome than nonheterotaxy (4.8% vs 2.4%; p = 0.05). Eight-year survivals were 91.9 +/- 3.2% in the nonheterotaxy group and 89.3 +/- 4.2 % in the heterotaxy group (p = 0.39). At 8 years, freedom from reoperation was 90.2 +/- 3.2% in the nonheterotaxy group and 78.5 +/- 6.2% in the heterotaxy group (p = 0.15). The outcomes (other than those of arrhythmia) were no different between the two groups. The incidences of early and late postoperative arrhythmia were 29.0% and 25.4%, respectively, in heterotaxy patients, and 15.4% and 10.8% in nonheterotaxy patients (p < 0.05). Bradyarrhythmia was found to be more common. During follow-up, atrioventricular valve regurgitation of more than mild was more common in heterotaxy patients (33.9% vs 18.9%; p = 0.05). CONCLUSIONS: Midterm outcomes after an extracardiac Fontan operation in heterotaxy and nonheterotaxy patients are similar, except arrhythmia and atrioventricular valve regurgitation.
- ISSN
- 1552-6259 (Electronic)
- Language
- English
- URI
- http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=16996916
https://hdl.handle.net/10371/13086
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