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Published on: July 18, 2014
Outcomes of Patients With Sickle Cell Disease and Trait After Congenital Heart Disease Surgery
Amrit Misra1, Ryan Halas2, Daisuke Kobayashi2
1Department of Cardiology, Boston Children's Hospital, Boston, Massachusetts.
Insights
Patients with sickle cell disease (SCD) and sickle cell trait (SCT) undergoing heart surgery had similar operative mortality to controls. These findings suggest safe surgical outcomes for SCD and SCT patients with appropriate perioperative care.
Area of Science:
- Cardiovascular Surgery
- Hematology
- Pediatric Cardiology
Background:
- Sickle cell disease (SCD) is a genetic blood disorder that can lead to multiorgan complications.
- Congenital heart disease (CHD) requires surgical intervention, sometimes in patients with SCD or sickle cell trait (SCT).
Purpose of the Study:
- To assess and compare perioperative outcomes in patients with SCD or SCT undergoing operations for CHD.
- To determine if SCD or SCT impacts mortality and morbidity in pediatric cardiac surgery patients.
Main Methods:
- Retrospective review of The Society of Thoracic Surgeons Congenital Heart Surgery Database (2014-2019).
- Inclusion of patients with SCD, SCT, and a control group.
- One-to-one propensity score matching for comparative analysis of outcomes.
Main Results:
- Operative mortality, surgery duration, and bypass times were comparable across SCD, SCT, and control groups.
- The SCD group showed a higher rate of postsurgical cardiac arrest compared to the matched control group (5.5% vs. 0%).
- No other significant differences in postoperative complications were observed between the SCD/SCT groups and their respective controls.
Conclusions:
- Cardiothoracic procedures in patients with SCD or SCT demonstrate similar operative mortality to control patients.
- Patients with SCD or SCT can undergo cardiac surgery without an increased risk of mortality, though unique perioperative management may be necessary.
Background:
Sickle cell disease (SCD) is a hemoglobinopathy that can cause multiorgan dysfunction. This study assessed the perioperative outcomes of patients undergoing operations for congenital heart disease who had SCD or sickle cell trait (SCT).
Methods:
We performed a retrospective review of patients with SCD or SCT who had records in The Society of Thoracic Surgeons Congenital Heart Surgery Database between 2014 and 2019. The primary outcome was operative mortality. Secondary outcomes included postoperative complications. One-to-one propensity score matching was performed between the SCD and SCT groups and the control group for further analysis.
Results:
Our study population consisted of 73, 411, and 36 501 patients in the SCD, SCT, and control groups, respectively. Median (25%-75% interquartile range) age at surgery was 2.8 (0.4-9.7), 0.60 (0.2-3.1), and 0.70 (0.2-6.4) years in the SCD, SCT, and control cohorts, respectively. Operative mortality, surgery duration, cardiopulmonary bypass time, and cross-clamp time were not significantly different among the 3 groups. The SCD group had a higher rate of postsurgical cardiac arrest than its propensity score-matched control group (5.5% vs 0%, P < .05); otherwise, there were no statistically significant differences in the outcomes between the SCD and SCT groups and their respective matched control groups.
Conclusions:
Operative mortality after cardiothoracic procedures in patients with SCD or SCT appeared similar to our control patients. While these patients may require unique perioperative management, they can undergo cardiac surgery without an observed increase in mortality.
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