Hostname: page-component-77c89778f8-fv566 Total loading time: 0 Render date: 2024-07-22T15:17:11.275Z Has data issue: false hasContentIssue false

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
*
Author for correspondence: A. W. Eckhauser, MD, MS, Divisions of Cardiothoracic Surgery and Pediatric Cardiology, University of Utah, Primary Children’s Hospital, 100 N. Mario Capecchi Dr., Salt Lake City, UT 84113, USA. Tel: +1 801 662 5566; Fax: +1 801 662 5571; E-mail: aaron.eckhauser@hsc.utah.edu

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 

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

Ohye, RG, Sleeper, LA, Mahony, L, et al. Comparison of shunt types in the Norwood procedure for single-ventricle lesions. N Engl J Med 2010; 362: 19801992.CrossRefGoogle ScholarPubMed
Newburger, JW, Sleeper, LA, Gaynor, JW, et al. Transplant-free survival and interventions at 6 years in the Single Ventricle Reconstruction trial. Circulation 2018; 137: 22462253.CrossRefGoogle Scholar
Eckhauser, A, Pasquali, SK, Ravishankar, C, et al. Variation in care for infants undergoing the Stage II palliation for hypoplastic left heart syndrome. Cardiol Young 2018; 28: 11091115.CrossRefGoogle ScholarPubMed
Pasquali, SK, Ohye, RG, Lu, M, et al. Variation in perioperative care across centers for infants undergoing the Norwood procedure. J Thorac Cardiovasc Surg 2012; 144: 915921.CrossRefGoogle ScholarPubMed
Ohye, RG, Gaynor, JW, Ghanayem, NS, et al. Design and rationale of a randomized trial comparing the Blalock–Taussig and right ventricle–pulmonary artery shunts in the Norwood procedure. J Thorac Cardiovasc Surg 2008; 136: 968975.CrossRefGoogle ScholarPubMed
Jacobs, ML, O’Brien, SM, Jacobs, JP, et al. An empirically based tool for analyzing morbidity associated with operations for congenital heart disease. J Thorac Cardiovasc Surg 2013; 145: 10461057e1.CrossRefGoogle ScholarPubMed
Ravishankar, C, Gerstenberger, E, Sleeper, LA, et al. Factors affecting Fontan length of stay: results from the Single Ventricle Reconstruction trial. J Thorac Cardiovasc Surg 2016; 151: 669675e1.CrossRefGoogle ScholarPubMed
Alsaied, T, Bokma, JP, Engel, ME, et al. Factors associated with long-term mortality after Fontan procedures: a systematic review. Heart 2017; 103: 104110.CrossRefGoogle ScholarPubMed
Hirsch, JC, Goldberg, C, Bove, EL, et al. Fontan operation in the current era: a 15-year single institution experience. Ann Surg 2008; 248: 402410.Google ScholarPubMed
Iyengar, AJ, Winlaw, DS, Galati, JC, et al. Trends in Fontan surgery and risk factors for early adverse outcomes after Fontan surgery: the Australia and New Zealand Fontan Registry experience. J Thorac Cardiovasc Surg 2014; 148: 566575.CrossRefGoogle ScholarPubMed
Pace Napoleone, C, Oppido, G, Angeli, E, Giardini, A, Resciniti, E, Gargiulo, G. Results of the modified Fontan procedure are not related to age at operation. Eur J Cardiothorac Surg 2010; 37: 645650.CrossRefGoogle Scholar
Rogers, LS, Glatz, AC, Ravishankar, C, et al. 18 years of the Fontan operation at a single institution: results from 771 consecutive patients. J Am Coll Cardiol 2012; 60: 10181025.CrossRefGoogle Scholar
Salvin, JW, Scheurer, MA, Laussen, PC, et al. Factors associated with prolonged recovery after the fontan operation. Circulation 2008; 118 (14 Suppl): S171S176.CrossRefGoogle ScholarPubMed
Stewart, RD, Pasquali, SK, Jacobs, JP, et al. Contemporary Fontan operation: association between early outcome and type of cavopulmonary connection. Ann Thorac Surg 2012; 93: 12541260; discussion 61.CrossRefGoogle ScholarPubMed
Tweddell, JS, Nersesian, M, Mussatto, KA, et al. Fontan palliation in the modern era: factors impacting mortality and morbidity. Ann Thorac Surg 2009; 88: 12911299.CrossRefGoogle ScholarPubMed
van der Ven, JPG, van den Bosch, E, Bogers, A, Helbing, WA. State of the art of the Fontan strategy for treatment of univentricular heart disease. F1000Res 2018; 114.CrossRefGoogle Scholar
Lo Rito, M, Al-Radi, OO, Saedi, A, et al. Chylothorax and pleural effusion in contemporary extracardiac fenestrated fontan completion. J Thorac Cardiovasc Surg 2018; 155: 20692077.CrossRefGoogle ScholarPubMed
Fedderly, RT, Whitstone, BN, Frisbee, SJ, Tweddell, JS, Litwin, SB. Factors related to pleural effusions after Fontan procedure in the era of fenestration. Circulation 2001; 104 (12 Suppl 1): I148I151.CrossRefGoogle ScholarPubMed
Wallace, MC, Jaggers, J, Li, JS, et al. Center variation in patient age and weight at Fontan operation and impact on postoperative outcomes. Ann Thorac Surg 2011; 91: 14451452.CrossRefGoogle ScholarPubMed
Supplementary material: File

Eckhauser et al. supplementary material

Eckhauser et al. supplementary material

Download Eckhauser et al. supplementary material(File)
File 63.2 KB