A case-control study of bleeding risk in children with 22q11.2 deletion syndrome undergoing cardiac surgery

Previous research suggests that individuals with 22q11.2 deletion syndrome (DS) have an increased risk of bleeding following cardiac surgery. However, current guidelines for management of patients with 22q11.2DS do not provide specific recommendations for perioperative management. This study sought...

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Main Authors: T. Blaine Crowley, Ian Campbell, Abinaya Arulselvan, David Friedman, Elaine H. Zackai, Tracy R. Geoffrion, Char Witmer, J. William Gaynor, Donna M. McDonald-McGinn, Michele P. Lambert
Format: Article
Language:English
Published: Taylor & Francis Group 2024-12-01
Series:Platelets
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Online Access:https://www.tandfonline.com/doi/10.1080/09537104.2023.2290108
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author T. Blaine Crowley
Ian Campbell
Abinaya Arulselvan
David Friedman
Elaine H. Zackai
Tracy R. Geoffrion
Char Witmer
J. William Gaynor
Donna M. McDonald-McGinn
Michele P. Lambert
author_facet T. Blaine Crowley
Ian Campbell
Abinaya Arulselvan
David Friedman
Elaine H. Zackai
Tracy R. Geoffrion
Char Witmer
J. William Gaynor
Donna M. McDonald-McGinn
Michele P. Lambert
author_sort T. Blaine Crowley
collection DOAJ
description Previous research suggests that individuals with 22q11.2 deletion syndrome (DS) have an increased risk of bleeding following cardiac surgery. However, current guidelines for management of patients with 22q11.2DS do not provide specific recommendations for perioperative management. This study sought to identify specific risk factors for bleeding in this patient population. Examine the factors determining bleeding and transfusion requirements in patients with 22q11.2DS undergoing cardiac surgery. This was a single center review of patients who underwent cardiac surgery at the Children’s Hospital of Philadelphia from 2000 to 2016. Data was extracted from the medical record. Frequency of bleeding events, laboratory values, and transfusion requirements were compared. We included 226 patients with 22q11.2DS and 506 controls. Bleeding events were identified in 13 patients with 22q11.2DS (5.8%) and 27 controls (5.3%). Platelet counts were lower among patients with 22q11.2DS than in control patients, but not statistically different comparing bleeding to not bleeding. Patients with 22q11.2DS received more transfusions (regardless of bleeding status). However, multivariate analysis showed only procedure type was associated with increased risk of bleeding (p = .012). The overall risk of bleeding when undergoing cardiac surgery is not different in patients with 22q11.2DS compared to non-deleted patients. Though platelet counts were lower in patients with 22q11.2DS, only procedure type was significantly associated with an increased risk of bleeding.
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spelling doaj-art-ad61591e3c2d4b56a2097ce4d2f856b12025-08-20T02:27:11ZengTaylor & Francis GroupPlatelets0953-71041369-16352024-12-0135110.1080/09537104.2023.2290108A case-control study of bleeding risk in children with 22q11.2 deletion syndrome undergoing cardiac surgeryT. Blaine Crowley0Ian Campbell1Abinaya Arulselvan2David Friedman3Elaine H. Zackai4Tracy R. Geoffrion5Char Witmer6J. William Gaynor7Donna M. McDonald-McGinn8Michele P. Lambert9Division of Human Genetics, Children’s Hospital of Philadelphia, Philadelphia, PA, USADivision of Human Genetics, Children’s Hospital of Philadelphia, Philadelphia, PA, USADivision of Hematology, Children’s Hospital of Philadelphia, Philadelphia, PA, USADepartment of Pathology and Laboratory Medicine, Children’s Hospital of Philadelphia, Philadelphia, PA, USADivision of Human Genetics, Children’s Hospital of Philadelphia, Philadelphia, PA, USADepartment of Surgery, Children’s Wisconsin, Milwaukee, WI, USADivision of Hematology, Children’s Hospital of Philadelphia, Philadelphia, PA, USADivision of Cardiothoracic Surgery, Children’s Hospital of Philadelphia, Philadelphia, PA, USADivision of Human Genetics, Children’s Hospital of Philadelphia, Philadelphia, PA, USADivision of Hematology, Children’s Hospital of Philadelphia, Philadelphia, PA, USAPrevious research suggests that individuals with 22q11.2 deletion syndrome (DS) have an increased risk of bleeding following cardiac surgery. However, current guidelines for management of patients with 22q11.2DS do not provide specific recommendations for perioperative management. This study sought to identify specific risk factors for bleeding in this patient population. Examine the factors determining bleeding and transfusion requirements in patients with 22q11.2DS undergoing cardiac surgery. This was a single center review of patients who underwent cardiac surgery at the Children’s Hospital of Philadelphia from 2000 to 2016. Data was extracted from the medical record. Frequency of bleeding events, laboratory values, and transfusion requirements were compared. We included 226 patients with 22q11.2DS and 506 controls. Bleeding events were identified in 13 patients with 22q11.2DS (5.8%) and 27 controls (5.3%). Platelet counts were lower among patients with 22q11.2DS than in control patients, but not statistically different comparing bleeding to not bleeding. Patients with 22q11.2DS received more transfusions (regardless of bleeding status). However, multivariate analysis showed only procedure type was associated with increased risk of bleeding (p = .012). The overall risk of bleeding when undergoing cardiac surgery is not different in patients with 22q11.2DS compared to non-deleted patients. Though platelet counts were lower in patients with 22q11.2DS, only procedure type was significantly associated with an increased risk of bleeding.https://www.tandfonline.com/doi/10.1080/09537104.2023.2290108Congenital heart diseaseDiGeorge syndromepostoperative hemorrhagesurgical blood lossthrombocytopenia
spellingShingle T. Blaine Crowley
Ian Campbell
Abinaya Arulselvan
David Friedman
Elaine H. Zackai
Tracy R. Geoffrion
Char Witmer
J. William Gaynor
Donna M. McDonald-McGinn
Michele P. Lambert
A case-control study of bleeding risk in children with 22q11.2 deletion syndrome undergoing cardiac surgery
Platelets
Congenital heart disease
DiGeorge syndrome
postoperative hemorrhage
surgical blood loss
thrombocytopenia
title A case-control study of bleeding risk in children with 22q11.2 deletion syndrome undergoing cardiac surgery
title_full A case-control study of bleeding risk in children with 22q11.2 deletion syndrome undergoing cardiac surgery
title_fullStr A case-control study of bleeding risk in children with 22q11.2 deletion syndrome undergoing cardiac surgery
title_full_unstemmed A case-control study of bleeding risk in children with 22q11.2 deletion syndrome undergoing cardiac surgery
title_short A case-control study of bleeding risk in children with 22q11.2 deletion syndrome undergoing cardiac surgery
title_sort case control study of bleeding risk in children with 22q11 2 deletion syndrome undergoing cardiac surgery
topic Congenital heart disease
DiGeorge syndrome
postoperative hemorrhage
surgical blood loss
thrombocytopenia
url https://www.tandfonline.com/doi/10.1080/09537104.2023.2290108
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