Perioperative management of patients with DiGeorge syndrome undergoing cardiac surgery.

Abstract

OBJECTIVE:

DiGeorge syndrome is a genetic disorder with multisystem involvement resulting in craniofacial and cardiac anomalies and parathyroid and immune system dysfunction. This study describes perioperative management of a large cohort of patients with DiGeorge syndrome undergoing cardiac surgery.

DESIGN:

Retrospective cohort study.

SETTING:

Major academic tertiary institution.

PARTICIPANTS:

The medical records of patients diagnosed with DiGeorge syndrome and undergoing cardiac surgery at this institution, from January 1, 1976, to July 31, 2012, were reviewed for phenotypic characteristics and intraoperative and postoperative complications, with specific attention to hemodynamic instability, perioperative perturbations of plasma calcium homeostasis, and airway difficulty.

INTERVENTIONS:

None.

MEASUREMENTS AND MAIN RESULTS:

Sixty-two patients underwent 136 cardiac surgical procedures; 47 patients (76%) had multiple operations. Sternotomies for reoperations often were complex (8 complicated by vascular injury or difficulty achieving hemostasis and 5 requiring bypass before sternotomy). Two patients had persistent hypocalcemia intraoperatively, requiring infusion of calcium chloride, and hypocalcemia developed postoperatively in 8 patients. Prolonged mechanical ventilation (>24 hours) was required after 48 procedures (35%), and 25 (18%) required prolonged inotropic support (>72 hours). Infectious complications occurred after 31 procedures (23%). There was no in-hospital or 30-day mortality.

CONCLUSIONS:

Patients with DiGeorge syndrome often have complex cardiac anomalies that require surgical repair. The postoperative course is notable for the frequent need for prolonged respiratory and hemodynamic support. Patients can develop hypocalcemia and may require calcium supplementation. Immunodeficiencies may be associated with the increased rate of postoperative infections and may dictate the need for specific transfusion management practices.

• Authors: Tze Yeng Yeoh, Federica Scavonetto, Ryan J Hamlin, Harold M Burkhart, Juraj Sprung, Toby N Weingarten
• Journal: Journal of cardiothoracic and vascular anesthesia (J Cardiothorac Vasc Anesth) Vol. 28 Issue 4 Pg. 983-9 (Aug 2014) ISSN: 1532-8422 [Electronic] United States
• PMID: 24461359 (Publication Type: Journal Article)
• Copyright: © 2014 Elsevier Inc. All rights reserved.

Topics

• Cardiac Surgical Procedures
• Child
• Child, Preschool
• DiGeorge Syndrome (diagnosis, surgery, therapy)
• Disease Management
• Female
• Follow-Up Studies
• Humans
• Incidence
• Infant
• Male
• Minnesota (epidemiology)
• Perioperative Care (methods)
• Postoperative Complications (epidemiology, prevention & control)
• Prognosis
• Retrospective Studies