USA: A cross-sectional, multicenter study has shed light on a concerning association between elevated tricuspid regurgitation velocity (TRV) and the prevalence of cerebrovascular and kidney diseases in children diagnosed with sickle cell disease (SCD). The findings, published in Pediatric Blood & Cancer, underscore the importance of vigilant monitoring and early intervention strategies for pediatric patients grappling with this inherited blood disorder.

According to the study, approximately 1 in 5 children with sickle cell disease in two large clinical cohorts experienced elevated tricuspid regurgitation velocity, a surrogate marker for pulmonary hypertension.
Evidence from the study revealed pediatric patients with a history of cerebrovascular disease (CVD) and chronic kidney disease (CKD) showed a higher prevalence of abnormal TRV, indicating the need for an echocardiogram in clinical assessment.
Sickle cell disease, characterized by abnormal hemoglobin molecules in red blood cells, is known for its diverse array of complications, including organ damage and impaired blood flow. Among these complications, cerebrovascular and kidney diseases stand out due to their severity and potential long-term impact on patients’ health.
Tricuspid regurgitation velocity, measured by echocardiography, is a surrogate marker for pulmonary hypertension. Not many pediatric studies have considered the association between TRV and surrogate markers of end-organ disease.
Led by Chibuzo Ilonze, University of Alabama at Birmingham, Birmingham, Alabama, USA, the cross-sectional study evaluated the prevalence of elevated TRV ≥2.5 m/s and its associations with renal and cerebrovascular outcomes in children with SCD 1–21 years of age in two large sickle cell cohorts, the Sickle Cell Clinical Research and Intervention Program (SCCRIP) cohort, and the University of Alabama at Birmingham (UAB) sickle cell cohort at St. Jude Children's Research Hospital.
The researchers hypothesized that patients with SCD and elevated TRV would have higher odds of having either persistent albuminuria or cerebrovascular disease.
The study revealed the following findings:
One hundred and sixty-six children from the UAB cohort (mean age: 13.49 ± 4.47 years) and 325 children from the SCCRIP cohort (mean age: 13.41 ± 3.99 years) were identified with echocardiograms.
The prevalence of an elevated TRV was 21% in both UAB and SCCRIP cohorts.
Elevated TRV was significantly associated with cerebrovascular disease (odds ratio [OR] 1.88) and persistent albuminuria (OR 1.81) after adjusting for age, sex, treatment, and site.
In conclusion, the researchers showed that elevated TRV is associated with a history of CVD and albuminuria. The findings suggest that adolescent patients may benefit from universal echocardiography performed at a steady state if they have been identified with either cerebrovascular disease or albuminuria.
As the medical community continues to unravel the complexities of sickle cell disease, studies like this serve as critical milestones in advancing our understanding of its pathophysiology and informing evidence-based strategies for patient management. Moving forward, concerted efforts are warranted to integrate these findings into clinical practice guidelines and enhance the holistic care provided to children battling this chronic condition.
"Future prospective studies are needed to determine if children with surrogate makers of cardio-renal or cardiac-CVD disease are at risk of increased morbidity such as exercise intolerance, poor quality of life, and early mortality," the researchers wrote.
Reference:
Ilonze, C., Rai, P., Galadanci, N., Zahr, R., Okhomina, V. I., Kang, G., Padmanabhan, D., Lebensburger, J., & Alishlash, A. S. Association of elevated tricuspid regurgitation velocity with cerebrovascular and kidney disease in children with sickle cell disease. Pediatric Blood & Cancer, e31002. https://doi.org/10.1002/pbc.31002