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

  • Andrew M. Atz (a1), Thomas G. Travison (a2), Brian W. McCrindle (a3), Lynn Mahony (a4), Andrew C. Glatz (a5), Aditya K. Kaza (a6), Roger E. Breitbart (a7), Steven D. Colan (a7), Jonathan R. Kaltman (a8), Renee Margossian (a7), Sara K. Pasquali (a9), Yanli Wang (a2) and Welton M. Gersony (a10)...



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.


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).


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.


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.


Corresponding author

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:


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