Why was this study done?
Heart disease is the main cause of death in people with sickle cell disease. We wanted to find out if chronic hemolysis (nonstop breakdown of red blood cells) causes people with sickle cell disease and other blood disorders to develop pulmonary hypertension (high blood pressure in the lungs) and other medical problems. Pulmonary hypertension is closely associated with heart failure.
The study’s main goals were to find out:
- If chronic hemolysis is connected to the development of pulmonary hypertension
- The number of children or adults with hemolysis who have pulmonary hypertension
- The spleen’s role in the development of pulmonary hypertension
- If blood and urine test results can be related to the development of pulmonary hypertension
- If there are genes in the blood that are important in developing pulmonary hypertension
- If blood tests, urine tests and a heart ultrasound change over a two year period
When was this study done?
The study opened in March 2009 and closed in June 2016.
What did the study consist of?
The study involved two blood samples, two urine samples and two echocardiograms (heart ultrasounds) done two years apart.
What did we learn from this study?
When we looked at two key measures of heart function based on the echocardiograms, there were important changes. Measuring the elevated tricuspid valve regurgitation jet velocity (TRV) is a way to find out if there is build-up of pressure in the lungs. TRV is related to a higher risk of dying in adulthood. We found out that TRV elevation is influenced by the degree of anemia a person with sickle cell disease has. Also, measuring global longitudinal strain (GLS) is a way to find out how well the heart is working. This test is done with an echocardiogram. GLS can reveal changes in heart function for patients who have sickle cell disease. In our study, GLS of the heart’s right ventricle (RVGLS) was higher in children with sickle cell disease than in healthy individuals. Abnormal RVGLS has been linked to serious medical problems and death in patients who have heart disease. The increased RVGLS points to an early response of the right ventricle to heart problems.
What are the next research steps as a result of this study?
Long-term follow-up is needed to find out if GLS can help us identify heart problems early on in patients with sickle cell disease.
How does this study affect my child?
Every individual with sickle cell disease should receive life-long follow-up care. Please speak with your St. Jude doctor about specific guidelines that apply to your child.
For more information
Please talk with your child’s St. Jude doctor about questions or concerns you have as a result of this study.
Publications generated from this study:
Ventricular global longitudinal strain is altered in children with sickle cell disease. Whipple NS, Naik RJ, Kang G, Moen J, Govindaswamy SD, Fowler JA, Dowdy J, Penkert R, Joshi VM, Hankins JS. Br J Haematol. 2018 Dec;183(5):796-806. https://ncbi.nlm.nih.gov/pubmed/30450553
Elevated tricuspid regurgitation velocity in congenital hemolytic anemias: Prevalence and laboratory correlates. Yates AM, Joshi VM, Aygun B, Moen J, Smeltzer MP, Govindaswamy D, Dowdy J, Cotton A, Kang G, Ware RE, Hankins JS. Pediatr Blood Cancer. 2019 Jul;66(7). https://www.ncbi.nlm.nih.gov/pubmed/30907497