Cardiac performance and quality of life in patients who have undergone the Fontan procedure with and without prior superior cavopulmonary connection

Andrew M. Atz, Thomas G. Travison, Brian W. McCrindle, Lynn Mahony, Andrew C. Glatz, Aditya K. Kaza, Roger E. Breitbart, Steven D. Colan, Jonathan R. Kaltman, Renee Margossian, Sara K. Pasquali, Yanli Wang, Welton M. Gersony

Research output: Contribution to journalArticle

7 Scopus citations

Abstract

Background A superior cavopulmonary connection is commonly performed before the Fontan procedure in patients with a functionally univentricular heart. Data are limited regarding associations between a prior superior cavopulmonary connection and functional and ventricular performance late after the Fontan procedure. Methods We compared characteristics of those with and without prior superior cavopulmonary connection among 546 subjects enrolled in the Pediatric Heart Network Fontan Cross-Sectional Study. We further compared different superior cavopulmonary connection techniques: bidirectional cavopulmonary anastomosis (n equals 229), bilateral bidirectional cavopulmonary anastomosis (n equals 39), and hemi-Fontan (n equals 114). Results A prior superior cavopulmonary connection was performed in 408 subjects (75%); the proportion differed by year of Fontan surgery and centre (p-value less than 0.0001 for each). The average age at Fontan was similar, 3.5 years in those with superior cavopulmonary connection versus 3.2 years in those without (p-value equals 0.4). The type of superior cavopulmonary connection varied by site (p-value less than 0.001) and was related to the type of Fontan procedure. Exercise performance, echocardiographic variables, and predominant rhythm did not differ by superior cavopulmonary connection status or among superior cavopulmonary connection types. Using a test of interaction, findings did not vary according to an underlying diagnosis of hypoplastic left heart syndrome. Conclusions After controlling for subject and era factors, most long-term outcomes in subjects with a prior superior cavopulmonary connection did not differ substantially from those without this procedure. The type of superior cavopulmonary connection varied significantly by centre, but late outcomes were similar.

Original languageEnglish (US)
Pages (from-to)335-343
Number of pages9
JournalCardiology in the Young
Volume23
Issue number3
DOIs
StatePublished - Jun 1 2013

Keywords

  • Congenital heart disease
  • Fontan
  • functionally univentricular heart
  • quality of life

ASJC Scopus subject areas

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

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    Atz, A. M., Travison, T. G., McCrindle, B. W., Mahony, L., Glatz, A. C., Kaza, A. K., Breitbart, R. E., Colan, S. D., Kaltman, J. R., Margossian, R., Pasquali, S. K., Wang, Y., & Gersony, W. M. (2013). Cardiac performance and quality of life in patients who have undergone the Fontan procedure with and without prior superior cavopulmonary connection. Cardiology in the Young, 23(3), 335-343. https://doi.org/10.1017/S1047951112001175