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Deletion of 22q11 chromosome is associated with postoperative morbidity after unifocalisation surgery

Published online by Cambridge University Press:  30 August 2018

Andrew Koth Open the ORCID record for Andrew Koth [Opens in a new window] ,
Doug Sidell ,
Holly Bauser-Heaton ,
Lisa Wise-Faberowski ,
Frank L. Hanley ,
Doff B. McElhinney  and
Ritu Asija
Andrew Koth*
Affiliation:
Division of Pediatric Cardiology, Stanford Hospital and Clinics, Stanford, CA, USA
Doug Sidell
Affiliation:
Division of Pediatric Cardiology, Stanford Hospital and Clinics, Stanford, CA, USA
Holly Bauser-Heaton
Affiliation:
Division of Pediatric Cardiology, Stanford Hospital and Clinics, Stanford, CA, USA
Lisa Wise-Faberowski
Affiliation:
Department of Anesthesia, Stanford University, Stanford, CA, USA
Frank L. Hanley
Affiliation:
Division of Cardiothoracic Surgery, Stanford University, Stanford, CA, USA
Doff B. McElhinney
Affiliation:
Division of Cardiothoracic Surgery, Stanford University, Stanford, CA, USA
Ritu Asija
Affiliation:
Division of Pediatric Cardiology, Stanford Hospital and Clinics, Stanford, CA, USA
*
Author for correspondence: A. Koth, MD, Division of Pediatric Cardiology, 750 Welch Road, Suite 325, Palo Alto, CA 94304, USA. Tel: 206 794 4501; Fax: 650 724 4922; E-mail: akoth@stanford.edu
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Abstract

Background

A 22q11 chromosome deletion is common in patients with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals. We sought to determine whether 22q11 chromosome deletion is associated with increased postoperative morbidity after unifocalisation surgery.

Methods

We included all patients with this diagnosis undergoing primary or revision unifocalisation ± ventricular septal defect closure at our institution from 2008 to 2016, and we excluded patients with unknown 22q11 status. Demographic and surgical data were collected. We compared outcomes between those with 22q11 chromosome deletion and those without using non-parametric analysis.

Results

We included 180 patients, 41% of whom were documented to have a chromosome 22q11 deletion. Complete unifocalisation was performed in all patients, and intracardiac repair was performed with similar frequency regardless of 22q11 chromosome status. Duration of mechanical ventilation was longer in 22q11 deletion patients. This difference remained significant after adjustment for delayed sternal closure and/or intracardiac repair. Duration of ICU stay was longer in patients with 22q11 deletion, although no longer significant when adjusted for delayed sternal closure and intracardiac repair. Finally, length of hospital stay was longer in 22q11-deleted patients, but this difference was not significant on unadjusted or adjusted analysis.

Conclusion

Children with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals and 22q11 deletion are at risk for greater prolonged mechanical ventilation after unifocalisation surgery. Careful attention should be given to the co-morbidities of this population in the perioperative period to mitigate risks that may complicate the postoperative course.

Keywords

CHDdel22q11DiGeorge syndrometetralogy of Fallotpulmonary atresia

Information

Type
Original Article
Information
Cardiology in the Young , Volume 29 , Issue 1 , January 2019 , pp. 19 - 22
Copyright
© Cambridge University Press 2018 

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Footnotes

Cite this article: Koth A, Sidell D, Bauser-Heaton H, Wise-Faberowski L, Hanley FL, McElhinney DB, Asija R. (2018) Deletion of 22q11 chromosome is associated with postoperative morbidity after unifocalisation surgery. Cardiology in the Young29: 19–22. doi: 10.1017/S1047951118001427

References

1. Doell, C, Bernet, V, Molinari, L, Beck, I, Balmer, C, Latal, B. Children with genetic disorders undergoing open-heart surgery: are they at increased risk for postoperative complications? Pediatr Crit Care Med 2011; 12: 539–544.Google Scholar
2. Simsic, JM, Coleman, K, Maher, KO, Cuadrado, A, Kirshbom, PM. Do neonates with genetic abnormalities have an increased morbidity and mortality following cardiac surgery? Congenit Heart Dis 2009; 4: 160–165.Google Scholar
3. Alsoufi, B. The effect of noncardiac and genetic abnormalities on outcomes following neonatal congenital heart surgery. Semin Thorac Cardiovasc Surg 2016; 28: 114–117.Google Scholar
4. Carotti, A, Digilio, MC, Piacentini, G, Saffirio, C, Di Donato, RM, Marino, B. Cardiac defects and results of cardiac surgery in 22q11.2 deletion syndrome. Dev Disabil Res Rev 2008; 14: 35–42.Google Scholar
5. Digilio, MC, Marino, B, Grazioli, S, Agostino, D, Giannotti, A, Dallapiccola, B. Comparison of occurrence of genetic syndromes in ventricular septal defect with pulmonic stenosis (classic tetralogy of Fallot) versus ventricular septal defect with pulmonic atresia. Am J Cardiol 1996; 77: 1375–1376.Google Scholar
6. Pierpont, ME, Basson, CT, Benson, DW Jr, et al. Genetic basis for congenital heart defects: current knowledge: a scientific statement from the American Heart Association Congenital Cardiac Defects Committee, Council on Cardiovascular Disease in the Young: endorsed by the American Academy of Pediatrics. Circulation 2007; 115: 3015–3038.Google Scholar
7. Maeda, J, Yamagishi, H, Matsuoka, R, et al. Frequent association of 22q11.2 deletion with tetralogy of Fallot. Am J Med Genet 2000; 92: 269–272.Google Scholar
8. Brenner, MK, Clarke, S, Mahnke, DK, et al. Effect of 22q11.2 deletion on bleeding and transfusion utilization in children with congenital heart disease undergoing cardiac surgery. Pediatr Res 2016; 79: 318–324.Google Scholar
9. Mercer-Rosa, L, Pinto, N, Yang, W, Tanel, R, Goldmuntz, E. 22q11.2 Deletion syndrome is associated with perioperative outcome in tetralogy of Fallot. J Thorac Cardiovasc Surg 2013; 146: 868–873.Google Scholar
10. McDonald, R, Dodgen, A, Goyal, S, et al. Impact of 22q11.2 deletion on the postoperative course of children after cardiac surgery. Pediatr Cardiol 2013; 34: 341–347.Google Scholar
11. Alsoufi, B, McCracken, C, Shashidharan, S, Deshpande, S, Kanter, K, Kogon, B. The impact of 22q11.2 deletion syndrome on surgical repair outcomes of conotruncal cardiac anomalies. Ann Thorac Surg 2017; 104: 1597–1604.Google Scholar
12. O’Byrne, ML, Yang, W, Mercer-Rosa, L, et al. 22q11.2 deletion syndrome is associated with increased perioperative events and more complicated postoperative course in infants undergoing infant operative correction of truncus arteriosus communis or interrupted aortic arch. J Thorac Cardiovasc Surg 2014; 148: 1597–1605.Google Scholar
13. Carotti, A, Albanese, SB, Filippelli, S, et al. Determinants of outcome after surgical treatment of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. J Thorac Cardiovasc Surg 2010; 140: 1092–1103.Google Scholar
14. Mahle, WT, Crisalli, J, Coleman, K, et al. Deletion of chromosome 22q11.2 and outcome in patients with pulmonary atresia and ventricular septal defect. Ann Thorac Surg 2003; 76: 567–571.Google Scholar
15. Chen, MY, Chiu, SN, Wang, JK, et al. Genetic syndromes and outcome after surgical repair of pulmonary atresia and ventricular septal defect. Ann Thorac Surg 2012; 94: 1627–1633.Google Scholar
16. Carotti, A, Marino, B, Di Donato, RM. Influence of chromosome 22q11.2 microdeletion on surgical outcome after treatment of tetralogy of Fallot with pulmonary atresia. J Thorac Cardiovasc Surg 2003; 126: 1666–1667.Google Scholar
17. Bauser-Heaton, H, Borquez, A, Han, B, et al. Programmatic approach to management of tetralogy of Fallot with major aortopulmonary collateral arteries: a 15-year experience with 458 patients. Circ Cardiovasc Interv 2017; 10: 10.Google Scholar
18. Asija, R, Hanley, FL, Roth, SJ. Postoperative respiratory failure in children with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals: a pilot study. Pediatr Crit Care Med 2013; 14: 384–389.Google Scholar
19. Malhotra, SP, Hanley, FL. Surgical management of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals: a protocol-based approach. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2009; 12: 145–151.Google Scholar
20. Sidell, DR, Koth, AM, Bauser-Heaton, H, et al. Bronchoscopy in children with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals. Pediatr Pulmonol 2017; 52: 1599–1604.Google Scholar
21. Perri, G, Albanese, SB, Carotti, A. Airway complications after single-stage unifocalization for pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries. J Card Surg 2015; 30: 453–458.Google Scholar
22. McElhinney, DB, Jacobs, I, McDonald-McGinn, DM, Zackai, EH, Goldmuntz, E. Chromosomal and cardiovascular anomalies associated with congenital laryngeal web. Int J Pediatr Otorhinolaryngol 2002; 66: 23–27.Google Scholar
23. Leopold, C, De Barros, A, Cellier, C, Drouin-Garraud, V, Dehesdin, D, Marie, JP. Laryngeal abnormalities are frequent in the 22q11 deletion syndrome. Int J Pediatr Otorhinolaryngol 2012; 76: 36–40.Google Scholar
24. Huang, RY, Shapiro, NL. Structural airway anomalies in patients with DiGeorge syndrome: a current review. Am J Otolaryngol 2000; 21: 326–330.Google Scholar