04 October 2021

Hydroxyurea therapy could improve or even reverse cardiac complications in people with sickle cell anaemia (SCA), according to a new US study.

The researchers, whose findings are published in the journal Blood Advances, say their discovery should mean the introduction of early screening for sickle cell-related cardiac abnormalities, even among those who do not show any symptoms.

The team evaluated data from 100 children and young adults, aged between three and 22, who were receiving care for SCA at Cohen Children’s Medical Center’s Pediatric Sickle Cell Program in New York.

Half of the participants had enlargement or thickening of the left ventricle (LV) of the heart, while pulmonary hypertension was also reported. Diastolic dysfunction can cause heart disease and heart failure if left unchecked.

Study author Dr Arushi Dhar, of Cohen Children’s Heart Center, said: “Our study shows once again that cardiac abnormalities are extremely common in children and young adults living with sickle cell anaemia.

“Anaemia reduces the oxygen-carrying capacity of blood in the body. As a result, in people living with SCA, the heart enlarges, thickens, and works harder at rest.”

The study showed promising new evidence that treatment with hydroxyurea could improve and even reverse cardiac abnormalities in SCA patients.

The research team noted that 60 of the study participants were taking hydroxyurea and that there was no difference in the level of LV enlargement for that group of patients compared to the group not taking the drug.

However, cardiac outcomes were better among individuals taking hydroxyurea for a longer time.

Dr Dhar said: “When we compared the group that had been on hydroxyurea treatment for less than one year to the group that had been on treatment for more than one year, we found that the people who had been treated for longer were less likely to have an enlarged heart or a thickened heart.

“This finding set the stage for the next phase of our research, where we discovered that cardiac measures were actually improving over time on hydroxyurea.”

This was a longitudinal analysis that assessed echocardiograms performed over time on the patients receiving hydroxyurea.

The researchers found the longer a patient took hydroxyurea, the less likely they were to show LV enlargement or thickening. They found 34 patients who had an abnormal LV mass when first tested, and of these, 18 had a normal LV at their most recent visit.

“We need very close cardiac monitoring of children and young adults with SCA,” said Dr Dhar. “Even for asymptomatic individuals, screening should start at a young age so we can catch any abnormalities early and start treatment with hydroxyurea as soon as possible.”


Dhar A, Leung TM, Appiah-Kubi A, Gruber D, Aygun B, Serigano O, Mitchell E.  (2021) “Longitudinal analysis of cardiac abnormalities in pediatric patients with sickle cell anemia and effect of hydroxyurea therapy.” Blood Advances, doi: 10.1182/bloodadvances.2021005076

Link: https://ashpublications.org/bloodadvances/article/doi/10.1182/bloodadvances.2021005076/476912/Longitudinal-analysis-of-cardiac-abnormalities-in

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