Modified Fontan operation in patients with anomalies of systemic and pulmonary venous connection

M. Ruzmetov, Mark Rodefeld, P. Vijay, Mark Turrentine, John Brown

Research output: Contribution to journalArticle

6 Citations (Scopus)

Abstract

The presence of anomalies of the systemic and pulmonary venous connection associated with single ventricle anomalies has been considered a contraindication for the Fontan operation (FO). The aim of this study is to outline the technical considerations associated with the presence of anomalies of systemic and/or pulmonary venous connections and to identify the risk factors for mortality in this group of patients undergoing the modified FO. Between 1989 and 2004, 63 patients (median age, 3.2 years) with anomalous systemic or pulmonary venous connection underwent a Fontan procedure at our institution. Nine patients had a combination of anomalous systemic and pulmonary venous connection, 49 patients had anomalous drainage only from the systemic circulation, and 5 patients had isolated anomalies of pulmonary venous return. Visceral heterotaxy syndrome was diagnosed in 25 patients. Previous palliative operations had been performed in 51 patients (81%). There was 1 early death, and 2 patients required take down of Fontan procedures. Two patients required reoperation for revision of the atrial baffle. At a mean follow-up of 4.6 ± 3.4 years, there have been 5 late deaths (8%) and 45 patients (71%) have undergone Fontan completion. Actuarial survival was 92% at 1 year and 91% at 5 and 10 years-not significantly different from the overall survival of the Fontan patients. We conclude that the modified FO can be successfully performed in patients with anomalous systemic or pulmonary venous connections, including those with visceral heterotaxy syndrome, with morbidity and mortality rates that do not differ significantly from those achieved in all patients with normal connections.

Original languageEnglish
Pages (from-to)608-613
Number of pages6
JournalPediatric Cardiology
Volume26
Issue number5
DOIs
StatePublished - Oct 2005

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Fontan Procedure
Lung
Heterotaxy Syndrome
Scimitar Syndrome
Survival
Mortality
Reoperation

Keywords

  • Congenital heart disease
  • Fontan operation
  • Heterotaxy syndrome

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Pediatrics, Perinatology, and Child Health

Cite this

Modified Fontan operation in patients with anomalies of systemic and pulmonary venous connection. / Ruzmetov, M.; Rodefeld, Mark; Vijay, P.; Turrentine, Mark; Brown, John.

In: Pediatric Cardiology, Vol. 26, No. 5, 10.2005, p. 608-613.

Research output: Contribution to journalArticle

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