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High-risk single ventricle palliation in children with Down syndrome: single institution experience

Published online by Cambridge University Press:  25 March 2014

Yinn K. Ooi ,
Pranava Sinha ,
Marcin Gierdalski  and
Ashraf Harahsheh
Yinn K. Ooi
Affiliation:
Department of Pediatrics, Division of Cardiology, Children’s National Health System, The George Washington University School of Medicine, Washington, District of Columbia, United States of America
Pranava Sinha
Affiliation:
Department of Cardiovascular Surgery, Children’s National Health System, The George Washington University School of Medicine, Washington, District of Columbia, United States of America
Marcin Gierdalski
Affiliation:
Department of Cardiovascular Surgery, Children’s National Health System, The George Washington University School of Medicine, Washington, District of Columbia, United States of America
Ashraf Harahsheh*
Affiliation:
Department of Pediatrics, Division of Cardiology, Children’s National Health System, The George Washington University School of Medicine, Washington, District of Columbia, United States of America
*
Correspondence to: Dr A. Harahsheh, MD, FACC, FAAP, Assistant Professor, Department of Pediatric Cardiology, George Washington University Center for Heart, Lung and Kidney Disease, Children’s National Health System 111 Michigan Ave, N.W. Washington, DC 20010, United States of America. Tel: 202 476 2020; Fax: 202 476 5700; E-mail: AHarahsh@childrensnational.org
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Abstract

Background: Of the children with Down syndrome 40–50% have cardiac defects and the majority of these cardiac defects are amenable to biventricular repair. The outcome of single ventricle palliation is improving; nonetheless, there are limited data on Down syndrome patients with associated high-risk factors undergoing single ventricle palliation. Our aim was to study the outcomes of children with Down syndrome and high-risk factors on the single ventricle palliation pathway. Methods: A retrospective study on all patients with Down syndrome on the single ventricle palliation pathway from 2005 until 2011 was conducted. Operative, clinical, echocardiographic, haemodynamic data, and follow-up data were reviewed. Results: A total of 310 patients underwent at least one single ventricle surgical intervention. Of those, eight patients had Down syndrome, five of which had associated risk factors – low birth weight, high pulmonary vascular resistance, pulmonary vein stenosis, significant atrioventricular valve regurgitation, and extracardiac anomalies. Mortality in the high-risk group was 80% (4/5), compared with 33% (1/3) in the non-high-risk patients. Overall, after a median follow-up period of 138 days (8–576 days), only 37.5% (3/8) of patients were alive. Conclusion: Despite many improvements in the care of single ventricle patients, the fate of those with Down syndrome and associated high-risk factors remains poor. Further multicentre longer-term studies are needed to validate and quantify the cumulative effects of negative prognostic factors in this complex group of patients.

Keywords

Norwoodpulmonary artery bandingsurgerypediatrics and congenital cardiac defect
Type
Original Articles
Information
Cardiology in the Young , Volume 25 , Issue 3 , March 2015 , pp. 539 - 543
Copyright
© Cambridge University Press 2014 

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Footnotes

*

Yinn K. Ooi and Ashraf Harahsheh were involved in the analysis of data and drafting of the manuscript. Marcin Gierdalski was involved in data gathering and analysis of data. Pranava Sinha provided critical revision of the manuscript for important intellectual content.

References

1. Canfield, MA, Honein, MA, Yuskiv, N, et al. National estimates and race/ethnic-specific variation of selected birth defects in the United States, 1999–2001. Birth Defects Res A Clin Mol Teratol 2006; 76: 747–756.Google ScholarPubMed
2. Rowe, RD, Uchida, IA. Cardiac malformation in mongolism: a prospective study of 184 mongoloid children. Am J Med 1961; 31: 726–735.CrossRefGoogle ScholarPubMed
3. van Trotsenburg, AS, Heymans, HS, Tijssen, JG, de Vijlder, JJ, Vulsma, T. Comorbidity, hospitalization, and medication use and their influence on mental and motor development of young infants with Down syndrome. Pediatrics 2006; 118: 1633–1639.CrossRefGoogle ScholarPubMed
4. Baraona, F, Gurvitz, M, Landzberg, MJ, Opotowsky, AR. Hospitalizations and mortality in the United States for adults with Down syndrome and congenital heart disease. Am J Cardiol 2013; 111: 1046–1051.CrossRefGoogle ScholarPubMed
5. Fudge, JC Jr., Li, S, Jaggers, J, et al. Congenital heart surgery outcomes in Down syndrome: analysis of a national clinical database. Pediatrics 2010; 126: 315–322.CrossRefGoogle ScholarPubMed
6. Lacour-Gayet, F, Clarke, D, Jacobs, J, et al. The Aristotle score: a complexity-adjusted method to evaluate surgical results. Eur J Cardiothorac Surg 2004; 25: 911–924.CrossRefGoogle Scholar
7. O’Brien, SM, Jacobs, JP, Clarke, DR, et al. Accuracy of the Aristotle Basic Complexity score for classifying the mortality and morbidity potential of congenital heart surgery operations. Ann Thorac Surg 2007; 84: 2027–2037; discussion 37.CrossRefGoogle ScholarPubMed
8. Jenkins, KJ, Gauvreau, K, Newburger, JW, Spray, TL, Moller, JH, Iezzoni, LI. Consensus-based method for risk adjustment for surgery for congenital heart disease. J Thorac Cardiovasc Surg 2002; 123: 110–118.Google ScholarPubMed
9. Jacobs, JP, Mavroudis, C, Jacobs, ML, et al. What is operative mortality? Defining death in a surgical registry database: a report of the STS Congenital Database Taskforce and the Joint EACTS-STS Congenital Database Committee. Ann Thorac Surg 2006; 81: 1937–1941.Google Scholar
10. Ashburn, DA, McCrindle, BW, Tchervenkov, CI, et al. Outcomes after the Norwood operation in neonates with critical aortic stenosis or aortic valve atresia. J Thorac Cardiovasc Surg 2003; 125: 1070–1082.Google ScholarPubMed
11. Vida, VL, Bacha, EA, Larrazabal, A, et al. Surgical outcome for patients with the mitral stenosis-aortic atresia variant of hypoplastic left heart syndrome. J Thorac Cardiovasc Surg 2008; 135: 339–346.CrossRefGoogle ScholarPubMed
12. Kelleher, DK, Laussen, P, Teixeira-Pinto, A, Duggan, C. Growth and correlates of nutritional status among infants with hypoplastic left heart syndrome (HLHS) after stage 1 Norwood procedure. Nutrition 2006; 22: 237–244.CrossRefGoogle ScholarPubMed
13. Anderson, JB, Beekman, RH 3rd, Border, WL, et al. Lower weight-for-age z score adversely affects hospital length of stay after the bidirectional Glenn procedure in 100 infants with a single ventricle. J Thorac Cardiovasc Surg 2009; 138: 397–404.CrossRefGoogle ScholarPubMed
14. Sathanandam, SK, Polimenakos, AC, Roberson, DA, et al. Mitral stenosis and aortic atresia in hypoplastic left heart syndrome: survival analysis after stage I palliation. Ann Thorac Surg 2010; 90: 1599–1607; discussion 607–608.Google ScholarPubMed
15. Hehir, DA, Dominguez, TE, Ballweg, JA, et al. Risk factors for interstage death after stage 1 reconstruction of hypoplastic left heart syndrome and variants. J Thorac Cardiovasc Surg 2008; 136: 94–99; 9.Google ScholarPubMed
16. Gupta-Malhotra, M, Larson, VE, Rosengart, RM, Guo, H, Moller, JH. Mortality after total cavopulmonary connection in children with the Down syndrome. Am J Cardiol 2010; 105: 865–868.CrossRefGoogle ScholarPubMed
17. Wada, N, Takahashi, Y, Ando, M, Park, IS, Sasaki, T. Single ventricle repair in children with Down’s syndrome. Gen Thorac Cardiovasc Surg 2008; 56: 104–108.Google ScholarPubMed
18. Campbell, RM, Adatia, I, Gow, RM, Webb, GD, Williams, WG, Freedom, RM. Total cavopulmonary anastomosis (Fontan) in children with Down’s syndrome. Ann Thorac Surg 1998; 66: 523–526.CrossRefGoogle ScholarPubMed
19. Hickey, EJ, Caldarone, CA, Blackstone, EH, et al. Critical left ventricular outflow tract obstruction: the disproportionate impact of biventricular repair in borderline cases. J Thorac Cardiovasc Surg 2007; 134: 1429–1436; discussion 36–37.Google ScholarPubMed
20. Beaton, AZ, Pike, JI, Stallings, C, Donofrio, MT. Predictors of repair and outcome in prenatally diagnosed atrioventricular septal defects. J Am Soc Echocardiogr 2013; 26: 208–216.CrossRefGoogle ScholarPubMed
21. 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.CrossRefGoogle ScholarPubMed