Perioperative management of patients with DiGeorge syndrome undergoing cardiac surgery

Tze Yeng Yeoh, Federica Scavonetto, Ryan J. Hamlin, Harold M. Burkhart, Juraj Sprung, Toby N. Weingarten

Research output: Contribution to journalArticle

6 Citations (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)983-989
Number of pages7
JournalJournal of Cardiothoracic and Vascular Anesthesia
Volume28
Issue number4
DOIs
StatePublished - Aug 2014

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DiGeorge Syndrome
Thoracic Surgery
Hypocalcemia
Sternotomy
Hemodynamics
Cardiac Surgical Procedures
Calcium
Calcium Chloride
Inborn Genetic Diseases
Vascular System Injuries
Practice Management
Intraoperative Complications
Hemostasis
Reoperation
Artificial Respiration
Medical Records
Immune System
Homeostasis
Cohort Studies
Retrospective Studies

Keywords

  • DiGeorge syndrome
  • anesthesia
  • cardiovascular surgical procedures
  • hypocalcemia
  • immunosuppression
  • infection

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Anesthesiology and Pain Medicine

Cite this

Perioperative management of patients with DiGeorge syndrome undergoing cardiac surgery. / Yeng Yeoh, Tze; Scavonetto, Federica; Hamlin, Ryan J.; Burkhart, Harold M.; Sprung, Juraj; Weingarten, Toby N.

In: Journal of Cardiothoracic and Vascular Anesthesia, Vol. 28, No. 4, 08.2014, p. 983-989.

Research output: Contribution to journalArticle

Yeng Yeoh, Tze ; Scavonetto, Federica ; Hamlin, Ryan J. ; Burkhart, Harold M. ; Sprung, Juraj ; Weingarten, Toby N. / Perioperative management of patients with DiGeorge syndrome undergoing cardiac surgery. In: Journal of Cardiothoracic and Vascular Anesthesia. 2014 ; Vol. 28, No. 4. pp. 983-989.
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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.",
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N2 - 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.

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