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Variation in care for children undergoing the Fontan operation for hypoplastic left heart syndrome

Published online by Cambridge University Press:  26 November 2019

Aaron W. Eckhauser
Affiliation:
Divisions of Cardiothoracic Surgery and Pediatric Cardiology, University of Utah, Primary Children’s Hospital, Salt Lake City, UT, USA
Maria I. Van Rompay
Affiliation:
New England Research Institutes, Watertown, MA, USA
Chitra Ravishankar
Affiliation:
Division of Pediatric Cardiology, Children’s Hospital of Philadelphia, Philadelphia, PA, USA
Jane W. Newburger
Affiliation:
Department of Cardiology, Boston Children’s Hospital, Harvard Medical School, Boston, MA, USA
S. Ram Kumar
Affiliation:
Heart Institute, Children’s Hospital of Los Angeles, Department of Surgery, Keck School of Medicine, University of Southern California, Los Angeles, CA, USA
Christian Pizarro
Affiliation:
Nemours Cardiac Center, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA
Nancy Ghanayem
Affiliation:
Division of Pediatric Critical Care, Baylor College of Medicine, Texas Children’s Hospital, Houston, TX, USA
Felicia L. Trachtenberg
Affiliation:
New England Research Institutes, Watertown, MA, USA
Kristin M. Burns
Affiliation:
Division of Cardiovascular Sciences, National Heart, Lung and Blood Institute, Bethesda, MD, USA
Garick D. Hill
Affiliation:
Division of Cardiology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, USA
Andrew M. Atz
Affiliation:
Division of Cardiology, Medical University of South Carolina, Charleston, SC, USA
Michelle S. Hamstra
Affiliation:
Division of Cardiology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, USA
Mjaye Mazwi
Affiliation:
Department of Critical Care Medicine, Hospital for Sick Children, University of Toronto, Toronto, ON, Canada.
Patsy Park
Affiliation:
Division of Pediatric Cardiology, Duke University, Durham, NC, USA
Marc E. Richmond
Affiliation:
Division of Pediatric Cardiology, Columbia University College of Physicians & Surgeons, New York, NY, USA
Michael Wolf
Affiliation:
Division of Pediatric Cardiology, Emory University School of Medicine, Atlanta, GA, USA
Jeffrey D. Zampi
Affiliation:
Division of Pediatric Cardiology, University of Michigan, C.S. Mott Children’s Hospital, Ann Arbor, MI, USA
Jeffrey P. Jacobs
Affiliation:
Division of Cardiovascular Surgery, Johns Hopkins University and All Children’s Hospital, St. Petersburg, FL, USA
L. LuAnn Minich
Affiliation:
Divisions of Cardiothoracic Surgery and Pediatric Cardiology, University of Utah, Primary Children’s Hospital, Salt Lake City, UT, USA
Corresponding

Abstract

Background:

The Single Ventricle Reconstruction Trial randomised neonates with hypoplastic left heart syndrome to a shunt strategy but otherwise retained standard of care. We aimed to describe centre-level practice variation at Fontan completion.

Methods:

Centre-level data are reported as median or median frequency across all centres and range of medians or frequencies across centres. Classification and regression tree analysis assessed the association of centre-level factors with length of stay and percentage of patients with prolonged pleural effusion (>7 days).

Results:

The median Fontan age (14 centres, 320 patients) was 3.1 years (range from 1.7 to 3.9), and the weight-for-age z-score was −0.56 (−1.35 + 0.44). Extra-cardiac Fontans were performed in 79% (4–100%) of patients at the 13 centres performing this procedure; lateral tunnels were performed in 32% (3–100%) at the 11 centres performing it. Deep hypothermic circulatory arrest (nine centres) ranged from 6 to 100%. Major complications occurred in 17% (7–33%). The length of stay was 9.5 days (9–12); 15% (6–33%) had prolonged pleural effusion. Centres with fewer patients (<6%) with prolonged pleural effusion and fewer (<41%) complications had a shorter length of stay (<10 days; sensitivity 1.0; specificity 0.71; area under the curve 0.96). Avoiding deep hypothermic circulatory arrest and higher weight-for-age z-score were associated with a lower percentage of patients with prolonged effusions (<9.5%; sensitivity 1.0; specificity = 0.86; area under the curve 0.98).

Conclusions:

Fontan perioperative practices varied widely among study centres. Strategies to decrease the duration of pleural effusion and minimise complications may decrease the length of stay. Further research regarding deep hypothermic circulatory arrest is needed to understand its association with prolonged pleural effusion.

Type
Original Article
Copyright
© Cambridge University Press 2019 

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