Hostname: page-component-586b7cd67f-dlnhk Total loading time: 0 Render date: 2024-11-21T06:22:02.641Z Has data issue: false hasContentIssue false
  • English
  • Français

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

Published online by Cambridge University Press:  24 July 2012

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  and
Renee Margossian
...Show all authors
Andrew M. Atz*
Affiliation:
Division of Cardiology, Department of Pediatrics, Medical University of South Carolina, Charleston, South Carolina, United States of America
Thomas G. Travison
Affiliation:
New England Research Institutes, Watertown, Massachusetts, United States of America
Brian W. McCrindle
Affiliation:
Division of Cardiology, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Canada
Lynn Mahony
Affiliation:
Department of Pediatrics, University of Texas Southwestern, Dallas, Texas, United States of America
Andrew C. Glatz
Affiliation:
Division of Cardiology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, United States of America
Aditya K. Kaza
Affiliation:
Department of Surgery, University of Utah, Salt Lake City, Utah, United States of America
Roger E. Breitbart
Affiliation:
Department of Cardiology, Children's Hospital Boston, Boston, Massachusetts, United States of America
Steven D. Colan
Affiliation:
Department of Cardiology, Children's Hospital Boston, Boston, Massachusetts, United States of America
Jonathan R. Kaltman
Affiliation:
National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland, United States of America
Renee Margossian
Affiliation:
Department of Cardiology, Children's Hospital Boston, Boston, Massachusetts, United States of America
Sara K. Pasquali
Affiliation:
Department of Pediatrics, Duke University Medical Center, Durham, North Carolina, United States of America
Yanli Wang
Affiliation:
New England Research Institutes, Watertown, Massachusetts, United States of America
Welton M. Gersony
Affiliation:
Division of Pediatrics, Columbia University Medical Center, New York, United States of America
*
Correspondence to: Dr A. M. Atz, MD, Division of Cardiology, Department of Pediatrics, Medical University of South Carolina, MSC 915, Room 601 Children's Hospital, Charleston, South Carolina 29425, United States of America. Tel: +1 843 792 3292; Fax: +1 843 792 1978; E-mail: [email protected]
Get access Rights & Permissions [Opens in a new window]

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.

Keywords

Congenital heart diseaseFontanfunctionally univentricular heartquality of life
Type
Original Articles
Information
Cardiology in the Young , Volume 23 , Issue 3 , June 2013 , pp. 335 - 343
Copyright
Copyright © Cambridge University Press 2012 

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1. Khairy, P, Poirier, N, Mercier, LA. Univentricular heart. Circulation 2007; 115: 800–812.Google Scholar
2. Bridges, ND, Jonas, RA, Mayer, JE, Flanagan, MF, Keane, JF, Castaneda, AR. Bidirectional cavopulmonary anastomosis as interim palliation for high-risk Fontan candidates. Early results. Circulation 1990; 82(5 Suppl): IV170–IV176.Google ScholarPubMed
3. Norwood, WI, Jacobs, ML. Fontan's procedure in two stages. Am J Surg 1993; 166: 548–551.Google Scholar
4. Anderson, PA, Sleeper, LA, Mahony, L, et al. Contemporary outcomes after the Fontan procedure: a Pediatric Heart Network multicenter study. J Am Coll Cardiol 2008; 52: 85–98.Google Scholar
5. Sleeper, LA, Anderson, P, Hsu, DT, et al. Design of a large cross-sectional study to facilitate future clinical trials in children with the Fontan palliation. Am Heart J 2006; 152: 427–433.CrossRefGoogle ScholarPubMed
6. Paridon, SM, Mitchell, PD, Colan, SD, et al. A cross-sectional study of exercise performance during the first 2 decades of life after the Fontan operation. J Am Coll Cardiol 2008; 52: 99–107.Google Scholar
7. Sluysmans, T, Colan, SD. Theoretical and empirical derivation of cardiovascular allometric relationships in children. J Appl Physiol 2005; 99: 445–457.CrossRefGoogle ScholarPubMed
8. Kuczmarski, RJ, Odgen, CL, Grummer-Strawn, LM. CDC Growth Charts: United States. Advance Data from Vital and Health Statistics; No. 314. National Center for Health Statistics, Hyattsville, Maryland, 2000.Google Scholar
9. McCrindle, BW, Zak, V, Sleeper, LA, et al. Laboratory measures of exercise capacity and ventricular characteristics and function are weakly associated with functional health status after Fontan procedure. Circulation 2010; 121: 34–42.Google Scholar
10. Atz, AM, Zak, V, Breitbart, RE, et al. Factors associated with serum brain natriuretic peptide levels after the Fontan procedure. Congenit Heart Dis 2011; 6: 313–321.CrossRefGoogle ScholarPubMed
11. Anderson, PA, Breitbart, RE, McCrindle, BW, et al. The Fontan patient: inconsistencies in medication therapy across seven pediatric heart network centers. Pediatr Cardiol 2010; 31: 1219–1228.Google Scholar
12. Lamberti, JJ, Spicer, RL, Waldman, JD, et al. The bidirectional cavopulmonary shunt. J Thorac Cardiovasc Surg 1990; 100: 22–29.CrossRefGoogle ScholarPubMed
13. d'Udekem, Y, Iyengar, AJ, Cochrane, AD, et al. The Fontan procedure: contemporary techniques have improved long-term outcomes. Circulation 2007; 116 (11 Suppl) I157–I164.Google Scholar
14. Attanavanich, S, Limsuwan, A, Vanichkul, S, Lertsithichai, P, Ngodngamthaweesuk, M. Single-stage versus two-stage modified Fontan procedure. Asian Cardiovasc Thorac Ann 2007; 15: 327–331.Google Scholar
15. Uemura, H, Yagihara, T, Kawashima, Y, et al. What factors affect ventricular performance after a Fontan-type operation? J Thorac Cardiovasc Surg 1995; 110: 405–415.CrossRefGoogle ScholarPubMed
16. Sluysmans, T, Sanders, SP, van, d V, et al. Natural history and patterns of recovery of contractile function in single left ventricle after Fontan operation. Circulation 1992; 86: 1753–1761.Google Scholar
17. Mahle, WT, Wernovsky, G, Bridges, ND, Linton, AB, Paridon, SM. Impact of early ventricular unloading on exercise performance in preadolescents with single ventricle Fontan physiology. J Am Coll Cardiol 1999; 34: 1637–1643.CrossRefGoogle ScholarPubMed
18. Landgraf, JM, Abetz, L, Ware, JE. The Child Health Questionnaire (CHQ): A User's Manual. The Health Institute, New England Medical Center, Boston, Massachusetts, 1996.Google Scholar
19. Cohen, MI, Bridges, ND, Gaynor, JW, et al. Modifications to the cavopulmonary anastomosis do not eliminate early sinus node dysfunction. J Thorac Cardiovasc Surg 2000; 120: 891–900.Google Scholar
20. Cohen, MI, Wernovsky, G, Vetter, VL, et al. Sinus node function after a systematically staged Fontan procedure. Circulation 1998; 98(19 Suppl): II352–II358.Google Scholar