What echocardiographic profiles are seen in adults with Sickle Cell Disease?
Echocardiography in adults with sickle cell disease (SCD) often reveals distinct patterns of heart structure and function. A 2021 cluster analysis of 379 patients identified three main profiles: one with high cardiac output and enlarged heart chambers, one with mild left heart remodeling and higher pulmonary artery pressure, and one with near-normal findings. These profiles are linked to different clinical outcomes, such as risk of pulmonary hypertension and survival.
What the research says
A 2021 study of 379 adults with SCD used cluster analysis to group patients based on echocardiographic variables 9. Cluster 1 (123 patients) had the lowest cardiac output, mild left heart remodeling, mild diastolic dysfunction, and higher tricuspid regurgitation velocity (TRV). These patients were mostly female and had the most functional limitation. Cluster 2 (102 patients) had the highest cardiac output and the most enlarged heart chambers, with diastolic function and TRV similar to Cluster 1. They also had higher blood pressure and more severe anemia. Cluster 3 (154 patients) had mild left heart remodeling, normal diastolic function, and the lowest TRV; they were younger with higher hemoglobin levels 9. Right heart catheterization in 94 patients showed that Cluster 1 had most cases of pre-capillary pulmonary hypertension, Cluster 2 had post-capillary pulmonary hypertension, and Cluster 3 had no pulmonary hypertension 9. Over 11 years of follow-up, 41 patients died (11%), with Cluster 2 having the worst prognosis 9.
A separate cross-sectional study of 57 adults with SCD in Colombia found that most had preserved left ventricular size and function (median ejection fraction 63%) and normal right ventricular function (mean TAPSE 25.4 mm) 1. However, elevated NT-proBNP levels (median 491 pg/mL) suggested some cardiac stress 1.
A case report of a 20-year-old with acute chest syndrome showed that point-of-care ultrasound (POCUS) can detect acute pulmonary hypertension (TR jet velocity >4 m/s) and guide treatment; after exchange transfusion, TR jet velocity improved 2. This highlights that echocardiographic profiles can change rapidly during acute illness.
What to ask your doctor
- What echocardiographic profile do I have, and what does it mean for my heart health?
- Should I have regular echocardiograms to monitor for changes in heart structure or function?
- What is my tricuspid regurgitation velocity (TRV) and does it suggest pulmonary hypertension?
- How does my hemoglobin level or anemia severity relate to my heart findings?
- Are there any treatments or lifestyle changes that could help if my heart shows signs of strain?
This question is drawn from common patient questions about Cardiology and answered using cited medical research. We do not provide individualized advice.