Author + information
- Arushi Dhar,
- Abena O. Appiah-Kubi,
- Banu Aygun and
- Elizabeth C. Mitchell
Published studies have reported echocardiographic abnormalities such as left ventricular (LV) hypertrophy, LV dilation and pulmonary hypertension (PH) in sickle cell disease (SCD). Hydroxyurea (HU) is the only approved pharmacological therapy for SCD. Longitudinal assessment of echocardiographic abnormalities in children with SCD receiving treatment with HU is lacking.
Single center retrospective longitudinal analysis of echocardiograms performed on children with SCD, prior to and after initiation of HU from January 2010 - June 2017. Echocardiographic data included LV end-systolic and end-diastolic diameters with corresponding z-scores, LV end-diastolic volume and mass (measured by 5/6th area*length method) and z-scores, LV fractional shortening, LV ejection fraction and tricuspid valve regurgitation velocity (TR). TR ≥ 2.5 m/s, z-score ≥ 2 for LV parameters and ≤ - 2 for LV fractional shortening and ejection fraction were considered abnormal.
Serial echocardiograms were analyzed on 64 patients, for a total of 181 echocardiograms; 40 patients (62.5%) were on HU for ≤3 years. LV dilation, LV hypertrophy and elevated TR velocities all improved significantly with HU (see table 1). Patients who were on HU for ≤3 years showed a significantly greater rate of decrease in TR velocity than those who were on HU for >3 years (p=0.029).
Echocardiographic abnormalities in children with SCD are common and appear to decrease over the course of treatment with HU.
Poster Hall, Hall F
Sunday, March 17, 2019, 3:45 p.m.-4:30 p.m.
Session Title: Non Invasive Imaging: Echo 4
Abstract Category: 28. Non Invasive Imaging: Echo
Presentation Number: 1271-334
- 2019 American College of Cardiology Foundation