22q11.2 Deletion Status and Perioperative Outcomes for Tetralogy of Fallot with Pulmonary Atresia and Multiple Aortopulmonary Collateral Vessels

Laura Mercer-Rosa, Okan U. Elci, Nelangi M. Pinto, Ronn E. Tanel, Elizabeth Goldmuntz

Research output: Contribution to journalArticle

Abstract

Deletion of 22q11.2 (del22q11) is associated with adverse outcomes in patients with tetralogy of Fallot (TOF). We sought to investigate its contribution to perioperative outcome in patients with a severe form of TOF characterized by pulmonary atresia (PA) or severe pulmonary stenosis (PS) and major aortopulmonary collateral arteries (MAPCAS). We conducted a retrospective review of patients with TOF/MAPCAS who underwent staged surgical reconstruction between 1995 and 2006. Groups were compared according to 22q11.2 deletion status using t-tests or the Wilcoxon Rank sum test. We included 26 subjects, 24 of whom survived the initial operation. Of those, 21 subjects had known deletion status and constitute the group for this analysis [15 with no deletion present (ND) and 6 del22q11 subjects]. There was no difference with respect to occurrence of palliative procedure prior to initial operation, or to timing of closure of the ventricular septal defect (VSD). Other than higher prevalence of prematurity (50%) in the del22q11 group versus no prematurity in the ND, the groups were comparable in terms of pre-operative characteristics. The intra- and post-operative course outcomes (length of cardiopulmonary bypass, use of vasopressors, duration of intensive care and length of hospital stay, tube-feeding) were also comparable. Although the del22q11 had longer mechanical ventilation than the ND, this difference was not significant [68 h (range 4–251) vs. 45 h (range 3–1005), p = 0.81]. In this detailed comparison of a small patient cohort, 22q11.2 deletion syndrome was not associated with adverse perioperative outcomes in patients with TOF, PA, and MAPCAS when compared to those without 22q11.2 deletion syndrome. These results are relevant to prenatal and neonatal pre-operative counseling and planning.

LanguageEnglish (US)
Pages1-5
Number of pages5
JournalPediatric Cardiology
DOIs
StateAccepted/In press - Mar 8 2018

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Pulmonary Atresia
Tetralogy of Fallot
DiGeorge Syndrome
Arteries
Nonparametric Statistics
Length of Stay
Pulmonary Valve Stenosis
Ventricular Heart Septal Defects
Enteral Nutrition
Critical Care
Cardiopulmonary Bypass
Artificial Respiration
Counseling

Keywords

  • 22q11.2 deletion
  • Aortopulmonary collateral vessels
  • DiGeorge syndrome
  • Outcome
  • Tetralogy of Fallot

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Cardiology and Cardiovascular Medicine

Cite this

22q11.2 Deletion Status and Perioperative Outcomes for Tetralogy of Fallot with Pulmonary Atresia and Multiple Aortopulmonary Collateral Vessels. / Mercer-Rosa, Laura; Elci, Okan U.; Pinto, Nelangi M.; Tanel, Ronn E.; Goldmuntz, Elizabeth.

In: Pediatric Cardiology, 08.03.2018, p. 1-5.

Research output: Contribution to journalArticle

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