Improving outcomes in functional single ventricle and total anomalous pulmonary venous connection.

Published

Journal Article (Review)

BACKGROUND: We have previously reported that the outcome of infants with functional single ventricle and total anomalous pulmonary venous connection is poor relative to that of other single ventricle patients. Younger age at initial operation and obstructed total anomalous pulmonary venous connection were found to be risk factors for mortality. A review of our recent experience was undertaken to determine whether results in these patients are improving. METHODS: Medical records of 18 patients admitted after 1997 were reviewed (group B) and compared with the previous group of 73 patients admitted between 1984 and 1997 (group A). Data were analyzed using a Cox proportional hazards model. RESULTS: Median age at first operation was the same for both groups. The incidence of obstructed total anomalous pulmonary venous connection was not significantly different between groups (29% versus 33%, p = 0.70). Early survival is significantly improved for group B compared with group A (p = 0.015). Only group and younger age at initial operation were found to be risk factors for mortality. In the current group, 5 patients have undergone superior cavopulmonary connection with one death, 3 have undergone Fontan completion with no deaths, and heart or heart-lung transplantation was performed in 5 patients with two deaths. CONCLUSIONS: Early survival in patients with single ventricle and total anomalous pulmonary venous connection has improved significantly in recent years, but intermediate survival is still approximately 50%. Selective management using staged reconstructive surgery and transplantation may result in improved survival. Further study may identify risk factors for mortality and improve selection of appropriate patients for each therapy.

Full Text

Duke Authors

Cited Authors

  • Lodge, AJ; Rychik, J; Nicolson, SC; Ittenbach, RF; Spray, TL; Gaynor, JW

Published Date

  • November 2004

Published In

Volume / Issue

  • 78 / 5

Start / End Page

  • 1688 - 1695

PubMed ID

  • 15511457

Pubmed Central ID

  • 15511457

Electronic International Standard Serial Number (EISSN)

  • 1552-6259

Digital Object Identifier (DOI)

  • 10.1016/j.athoracsur.2004.04.057

Language

  • eng

Conference Location

  • Netherlands