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Longitudinal Analysis of Echocardiographic Abnormalities in Children With Sickle Cell Disease
Jamie K Harrington1, Usha Krishnan, Zhezhen Jin
1*Department of Pediatrics, Division of Pediatric Cardiology ‡Department of Pediatrics §Department of Surgery, Division of Cardiothoracic Surgery ∥Department of Pediatrics, Division of Hematology/Oncology/Stem Cell Transplantation, College of Physicians & Surgeons †Department of Biostatistics, Mailman School of Public Health, Columbia University, New York, NY.
Insights
Cardiac abnormalities are common in children with sickle cell disease (SCD), appearing early and increasing with age. This progression may indicate disease severity in pediatric patients.
Area of Science:
- Pediatric Cardiology
- Hematology
- Cardiovascular Research
Background:
- Echocardiographic cardiac abnormalities are noted in children with sickle cell disease (SCD).
- Longitudinal studies tracking these abnormalities throughout childhood in SCD patients are limited.
Purpose of the Study:
- To investigate the longitudinal progression of echocardiographic abnormalities in pediatric patients with sickle cell disease (SCD).
- To identify factors associated with the development of cardiac abnormalities in this population.
Main Methods:
- Retrospective analysis of 829 echocardiograms from pediatric SCD patients at steady-state.
- Evaluation of left heart parameters (dimensions, mass, fractional shortening) and right ventricular pressure (tricuspid regurgitation gradient).
- Definition of abnormal parameters included specific thresholds for LV parameters and TR gradient.
Main Results:
- Echocardiographic abnormalities were detected by age 5, with cumulative incidence increasing throughout childhood.
- Positive associations with cardiac abnormalities included age, male gender, HbSS/Sβ thalassemia genotype, elevated WBC, platelets, total bilirubin, and history of pain crises/acute chest syndrome.
- Lower hemoglobin levels were negatively associated with cardiac abnormalities.
Conclusions:
- Cardiac abnormalities in children with SCD emerge early and progress with age.
- The high cumulative incidence suggests cardiac abnormalities may serve as a marker for disease severity in pediatric SCD.
- Further research is warranted to understand the long-term implications and management strategies.
Background:
Cardiac abnormalities have been described in echocardiograms of children with sickle cell disease (SCD). However, longitudinal studies investigating progression of echocardiographic abnormalities across the pediatric age spectrum in SCD are lacking.
Methods:
A retrospective longitudinal analysis of 829 echocardiograms from pediatric patients with SCD at steady-state was performed. Left heart parameters included left ventricular end-systolic, end-diastolic diameters, fractional shortening, and mass. Right ventricular pressure was estimated by tricuspid regurgitation gradient. Tricuspid regurgitation gradient ≥25 mm Hg, a z-score ≥2 for LV parameters and ≤-2 for left ventricular fractional shortening were considered abnormal.
Results:
Kaplan-Meier analysis revealed that echocardiographic abnormalities were detected by 5 years of age, and the cumulative incidence progressively increased throughout childhood. Age, male gender, HbSS and Sβ thalassemia genotype, white blood cell count, platelet count, total bilirubin, admissions for pain crises and acute chest syndrome were positively, whereas hemoglobin was negatively associated with cardiac abnormalities.
Conclusion:
Cardiac abnormalities began early in childhood and progressively increased with age. Our study highlights the high cumulative incidence of cardiac abnormalities in children with SCD, which could represent a marker of disease severity.
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