Sickle Cell Diastolic Cardiomyopathy and Mortality Risk: A Novel Echocardiographic Framework for Prognostic...

Area of Science:

• Cardiology
• Hematology
• Echocardiography

Background:

• Cardiovascular complications are the primary cause of mortality in sickle cell anemia (SCA).
• Current echocardiographic assessments for diastolic dysfunction (DD) may not adequately capture the unique hemodynamics in SCA.
• Refining DD assessment is crucial for improving risk stratification and treatment in SCA patients.

Purpose of the Study:

• To evaluate left ventricular diastolic parameters in SCA patients compared to healthy controls.
• To assess the association of lateral e' velocity (e' lat), E/e' ratio, and indexed left atrial volume (LAVi) with clinical outcomes in SCA.
• To refine the echocardiographic diagnosis and prognostic value of DD in young SCA patients.

Main Methods:

• Analysis of data from the French multicentric Etendard cohort (n=379) and comparison with the Copenhagen City Heart Study (CCHS) controls (n=672).
• Focus on left ventricular diastolic parameters: lateral e' velocity (e' lat), E/e' ratio, and indexed left atrial volume (LAVi).
• 12-year follow-up to assess the association of these parameters with clinical outcomes, particularly mortality.

Main Results:

• SCA patients exhibited earlier impaired diastolic function compared to controls.
• In young SCA patients (≤38 years), e' lat < 11 cm/s was significantly associated with prognosis (p=0.01).
• Young SCA patients with DD (e' lat < 11 cm/s) had a fourfold increased 12-year mortality rate (16% vs. 4%, p<0.001) and correlated with hemolysis markers.

Conclusions:

• Lateral e' velocity below 11 cm/s is a valuable prognostic marker for diastolic dysfunction in young SCA patients.
• This finding helps refine DD evaluation and risk stratification in SCA.
• The study highlights the link between DD, hemolysis, and systemic vasculopathy in SCA cardiac impairment.