Extracorporeal membrane oxygenation in children with heart disease and del22q11 syndrome: a review of the Extracorporeal Life Support Organization Registry

Abstract

The study objective was to evaluate outcomes among children with del22q11 (DiGeorge) syndrome supported on ECMO for heart disease. The ELSO registry database was queried to include all children <18 years undergoing heart surgery for either common atrio-ventricular canal, tetralogy of Fallot, truncus arteriosus or transposition of the great vessels and interrupted aortic arch and requiring ECMO, from 1998-2011. The outcomes evaluated included mortality, ECMO duration and length of hospital stay in patients with del22q11 syndrome and with no del22q11 syndrome. Eighty-eight ECMO runs occurred in children with del22q11 syndrome while 2694 ECMO runs occurred in children without del22q11 syndrome. For patients with heart defects receiving ECMO, del22q11 syndrome did not confer a significant mortality risk or an increased risk of infectious complications before or while on ECMO support. Neither the duration of ECMO nor mechanical ventilation prior to ECMO deployment were prolonged in patients with del22q11 syndrome compared to the controls.

Keywords

extracorporeal membrane oxygenation congenital heart disease del22q11 syndrome children tetralogy of Fallot interrupted aortic arch truncus arteriosus outcomes

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References

Harris

Francannet

Pradat

Robert

The epidemiology of cardiovascular defects, part 2: a study based on data from three large registries of congenital malformations. Pediatr Cardiol 2003; 24: 222–235.

Tezenas du Montcel

Mendizabal

Ayme

. Prevalence of 22q11 microdeletion. J Med Genet 1996; 33: 719.

Jatana

Gillis

Webster

Adès

LC.

Deletion 22q11.2 syndrome: implications for the intensive care physician. Pediatr Crit Care Med 2007; 8: 459–463.

Ryan

Goodship

Wilson

. Spectrum of clinical features associated with interstitial chromosome 22q11 deletions: a European collaborative study. J Med Genet 1997; 34: 798–804.

Van Mierop

LHS

Kutsche

LM.

Cardiovascular anomalies in DiGeorge syndrome and importance of neural crest as a possible pathogenetic factor. Am J Cardiol 1986; 58: 133–137.

Goldmuntz

Clark

Mitchell

. Frequency of 22q11 deletions in patients with conotruncal defects. J Am Coll Cardiol 1998; 32: 492–498.

Maeda

Yamagishi

Matsuoka

. Frequent association of 22q11.2 deletion with tetralogy of Fallot. Am J Med Genet 2000; 92: 269–272.

Michielon

Marino

Oricchio

. Impact of DEL22q11, trisomy 21, and other genetic syndromes on surgical outcome of conotruncal heart defects. J Thorac Cardiovasc Surg 2009; 138: 565–570.

Simsic

Coleman

Maher

. Do neonates with genetic abnormalities have an increased morbidity and mortality following cardiac surgery? Congenit Heart Dis 2009; 4: 160–165.

10.

McDonald

Dodgen

Goyal

. Impact of 22q11.2 deletion on the postoperative course of children after cardiac surgery. Pediatr Cardiol. 2013; 34: 341–347.

11.

Carotti

Marino

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.

12.

Carotti

Albanese

Filippelli

. 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.

13.

Mahle

Crisalli

Coleman

. Deletion of chromosome 22q11.2 and outcome in patients with pulmonary atresia and ventricular septal defect. Ann Thorac Surg 2003; 76: 567–571.

14.

Marmon

Balsara

Chen

: Congenital cardiac anomalies associated with the DiGeorge syndrome: a neonatal experience. Ann Thorac Surg 1984; 38: 146–150.

15.

Yeng Yeoh

Scavonetto

Hamlin

. Perioperative management of patients with DiGeorge syndrome undergoing cardiac surgery. J Cardiothorac Vasc Anesth 2014; 28: 995–1001.

16.

Kyburz

Bauersfeld

Schinzel

. The fate of children with microdeletion 22q11.2 syndrome and congenital heart defect: clinical course and cardiac outcome. Pediatr Cardiol 2008; 29: 76–83.