Sickle Cell Diastolic Cardiomyopathy and Mortality Risk: A Novel Echocardiographic Framework for Prognostic Stratification
Key result
Diastolic dysfunction linked to ~4x higher 12-year mortality in young sickle cell anemia patients.
- P<0.001
- n=1,051
Why the study?
Hemodynamic conditions unique to sickle cell anemia challenge current recommendations for assessing diastolic function, creating a need to refine echocardiographic definitions of diastolic dysfunction for risk stratification.
Does echocardiographic assessment of lateral e' velocity predict 12-year mortality in young patients with sickle cell anemia?
Population
379 sickle cell anemia patients and 672 age- and sex-matched controls
Comparison
French multicentric Etendard cohort patients vs Copenhagen City Heart Study controls
Design
Multicentric cohort study
Follow-up
12-year follow-up
Authors
Discussion
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Member takes
Implication
May aid risk stratification in young SCA; leaves open whether screening improves outcomes.
Study Design
Type
Cohort (n=1,051)
Multicenter
Yes
Structured PICO
Does echocardiographic assessment of lateral e' velocity predict 12-year mortality in young patients with sickle cell anemia?
P
Population
1,051 individuals, comprising 379 patients with sickle cell anemia (SCA) from the French multicentric Etendard cohort (including 252 young patients aged ≤ 38 years) and 672 age- and sex-matched controls from the Copenhagen City Heart Study.
I
Intervention
Echocardiographic assessment of left ventricular diastolic parameters, specifically lateral e' velocity (e' lat), E/e' ratio, and indexed left atrial volume (LAVi).
C
Comparator
Age- and sex-matched control group from the Copenhagen City Heart Study (CCHS).
O
Outcome
Mortality over a 12-year follow-up.hard clinical
Main Result
Absolute Event Rate: 16% vs 4%
p-value: p=<0.001
In young patients with sickle cell anemia, a lateral e' velocity below 11 cm/s is a strong prognostic marker associated with a fourfold increased 12-year mortality risk.
Cite This Study
Simon et al. (2025) conducted a cohort in Sickle cell anemia (n=1,051). Diastolic dysfunction (lateral e' velocity < 11 cm/s) vs. SCA patients without diastolic dysfunction was evaluated on 12-year mortality rate (p=<0.001). Young sickle cell anemia patients with diastolic dysfunction (lateral e' velocity < 11 cm/s) had a fourfold increased 12-year mortality rate compared to those without (16% vs. 4%, p<0.001).