61.13 Perioperative Outcomes Following Cardiovascular Surgery in Patients with 22q11.2 deletion

Posted on January 18, 2016

M. E. Mitchell1,2, D. Menhke2, P. Simpson2, M. Nugent2, A. Tomita-Mitchell2, N. Ghanayem1,2, S. Clarke1,2 1Children’s Hospital Of Wisconsin,Milwaukee, WI, USA 2Medical College Of Wisconsin,Milwaukee, WI, USA

Introduction:
22q11.2 is the most common deletion syndrome with an incidence of 1:4000. Up to 75% of patients with 22q11.2 deletion syndrome (22qDS) have congenital heart disease and need of congenital heart surgery. The known associated anomalies within the endocrine, neurologic, hematologic, and airway systems can have affects on their perioperative morbidity and mortality following cardiovascular surgery. Our study was to look at our single center institution perioperative morbidity in patients with 22qDS following cardiovascular surgery.

Methods:
This was a single center retrospective chart review of 99 patients that had cardiovascular surgery at Children's Hospital of Wisconsin between 2004-2011 and were in the Congenital Heart Disease Tissue Bank. Patients with 22qDS and matched controls, for age and type of surgery were identified. Data from the perioperative period was obtained and analysis was performed using classification trees.

Results:
Patients with 22qDS and a RACHS score < 2 had more cardiopulmonary bypass time > 150 min (p < 0.001). There was an increased risk of post-operative neurologic injury in patients with 22qDS that had a cardiopulmonary bypass time > 150 min (p<0.004). Cardiopulmonary bypass time was > 150 min in patients with 22qDS, higher RACHS scores, and no prenatal diagnosis (p<0.001). Patients with 22qDS had > 5 days of mechanical ventilation similar to patients with RACHS scores > 3 and cardiopulmonary bypass time > 150 min.

Conclusion:
This study shows there are increased post-operative morbidities in patients with 22qDS related to cardiopulmonary bypass, neurologic injury, and mechanical ventilation. Prenatal diagnosis also plays a role in length of cardiopulmonary bypass in patients with 22qDS. This suggests that further investigation with a larger cohort of patients with 22qDS is needed.