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Haematological determinants of cardiac involvement in adults with sickle cell disease
Thibaud Damy1,2,3,4,5,6, Diane Bodez1,2,3,4,5,6, Anoosha Habibi4,7
1AP-HP, Department of Cardiology, Henri Mondor Teaching Hospital, 51 Avenue Maréchal de Lattre de Tassigny, Creteil F-94000, France.
Insights
Sickle cell disease (SCD) patients with cardiac dilation show links to poorer blood health, including low hemoglobin and high red blood cell rigidity. Tricuspid regurgitant velocity and left ventricular dysfunction predict mortality in SCD.
Area of Science:
- Cardiology
- Hematology
- Genetics
Background:
- Cardiac involvement is a frequent complication of sickle cell disease (SCD).
- Identifying hematological determinants and prognostic markers for cardiac complications in SCD is crucial for patient management.
- Previous studies highlight the need for deeper investigation into the relationship between blood parameters and cardiac function in SCD.
Purpose of the Study:
- To identify hematological factors associated with cardiac involvement in sickle cell disease patients.
- To determine the impact of these hematological factors on the prognosis of cardiac involvement in SCD.
- To establish predictive markers for mortality in SCD patients with cardiac complications.
Main Methods:
- A longitudinal observational study involving 656 sickle cell disease patients (SS or S-β(0)-thalassemia).
- Data collected included blood workup and echocardiograms, analyzed for left ventricular and left atrial dimensions, cardiac index, ejection fraction, and tricuspid regurgitant velocity.
- Statistical analysis identified independent hematological determinants of cardiac abnormalities and their association with mortality.
Main Results:
- Cardiac dilation and elevated cardiac index were associated with lower hemoglobin, fetal hemoglobin, and red blood cell counts, alongside higher lactate dehydrogenase, bilirubin, and dense red blood cells.
- Low fetal hemoglobin and red blood cell count were linked to high cardiac index.
- Tricuspid regurgitant velocity ≥ 2.5 m/s and left ventricular ejection fraction <55% were significant predictors of mortality, with a four-fold increased risk when both were present.
Conclusions:
- Cardiac dilation and elevated cardiac index in SCD are linked to hematological variables indicating hemolysis, red blood cell rigidity, and blood viscosity.
- Tricuspid regurgitant velocity ≥ 2.5 m/s and left ventricular dysfunction are critical predictors of mortality in sickle cell disease patients.
- These findings underscore the importance of monitoring hematological parameters for assessing cardiac risk and prognosis in SCD.
Aims:
Cardiac involvement is common in sickle cell disease (SCD). Studies are needed to establish haematological determinants of this involvement and prognostic markers. The aim of the study was to identify haematological factors associated with cardiac involvement in SCD and their impact on prognosis.
Methods And Results:
This longitudinal observational study was performed on 1780 SCD patients with SS or S-β(0)-thalassemia referred to our centre. Six hundred fifty-six met our inclusion criteria (availability of a blood-workup and echocardiogram obtained <1 year apart, no heart valve surgery and no current pregnancy). Median age was 31 (interquartile range, 25-40) years, and median haemoglobin (Hb) was 87 (80-95)g/L. Left ventricular (LV) dilation, left atrial dilation, cardiac index (CI) >4 L/min/m(2), LV ejection fraction <55%, and tricuspid regurgitant velocity (TRV) ≥2.5 m/s were found in 35, 78, 23, 8.5, and 17% of patients, respectively. Compared with other patients, those in the fourth quartiles (Q4) of LV end-diastolic dimension index (LVEDDind) and left atrial dimension index (LADind) and those with high CI had significantly lower Hb, % foetal Hb (HbF), and red blood cell (RBC) counts; and significantly higher lactate dehydrogenase, bilirubin, and %dense RBCs. Independent haematologic determinants of Q4 LVEDDind and LADind were low RBC count and %HbF; high %dense RBCs were associated with LADind. Low %HbF and RBC count were associated with high CI. High %dense RBCs or no α-thalassemia gene deletion was associated with greater severity of anaemia and cardiac dilation and with higher CI. During the median follow-up of 48 (32-59) months, 50 (7.6%) patients died. Tricuspid regurgitant velocity ≥ 2.5 m/s was a predictor of mortality. The risk of death increased four-fold when left ventricular ejection fraction <55% was present also (P = 0.0001).
Conclusion:
Cardiac dilation and CI elevation in patients with SCD are associated with haematologic variables reflecting haemolysis, RBC rigidity, and blood viscosity. Tricuspid regurgitant velocity ≥ 2.5 and LV dysfunction (even mild) predict mortality.
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