Perioperative management of patients with DiGeorge syndrome undergoing cardiac surgery.
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.


METHODS
Retrospective cohort study.


METHODS
Major academic tertiary institution.


METHODS
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.


METHODS
None.


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.
Department of Anesthesiology, Mayo Clinic, Rochester, MN; Department of Anaesthesia, National University Hospital, National University Health System, Republic of Singapore.
Tze Yeng
TY
Yeoh
Department of Anesthesiology, Mayo Clinic, Rochester, MN.
Federica
F
Scavonetto
Department of Anesthesiology, Mayo Clinic, Rochester, MN.
Ryan J
RJ
Hamlin
Division of Cardiovascular Surgery, Mayo Clinic, Rochester, MN.
Harold M
HM
Burkhart
Department of Anesthesiology, Mayo Clinic, Rochester, MN.
Juraj
J
Sprung
Department of Anesthesiology, Mayo Clinic, Rochester, MN. Electronic address: weingarten.toby@mayo.edu.
Toby N
TN
Weingarten
D006348:Cardiac Surgical Procedures; D002648:Child; D002675:Child, Preschool; D004062:DiGeorge Syndrome / Q000175:diagnosis / Q000601:surgery / Q000628:therapy*; D019468:Disease Management; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D015994:Incidence; D007223:Infant; D008297:Male; D008910:Minnesota / Q000453:epidemiology; D019990:Perioperative Care / Q000379:methods*; D011183:Postoperative Complications / Q000453:epidemiology / Q000517:prevention & control*; D011379:Prognosis; D012189:Retrospective Studies
DiGeorge syndrome; anesthesia; cardiovascular surgical procedures; hypocalcemia; immunosuppression; infection