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Cardiac manifestations in sickle cell disease varies with patient genotype

Paul Guedeney1, François Lionnet2, Alexandre Ceccaldi1

  • 1Sorbonne Universités, UPMC Univ Paris 06, Institut de Cardiologie (AP-HP), Centre Hospitalier Universitaire Pitié-Salpêtrière, Institute of Cardiometabolism and Nutrition (ICAN), INSERM UMRS 1166, ACTION Study Group, Paris, France.

British Journal of Haematology
|April 21, 2018
PubMed

Insights

Cardiac involvement differs between sickle cell anaemia (SCA) and Haemoglobin SC (HbSC) disease. SCA shows more left ventricular hypertrophy, while HbSC disease

Area of Science:

  • Cardiology
  • Hematology
  • Genetics

Background:

  • Cardiac involvement is well-defined in sickle cell anaemia (SCA).
  • Cardiac manifestations in Haemoglobin SC (HbSC) disease remain largely uncharacterized.
  • Understanding genotypic differences in cardiac complications is crucial for patient management.

Purpose of the Study:

  • To compare cardiac features in patients with HbSC disease versus SCA patients.
  • To identify determinants of cardiac involvement in both sickle cell genotypes.
  • To establish genotype-specific echocardiographic findings and indications.

Main Methods:

  • Echocardiography was used to assess left ventricular ejection fraction (LVEF), left ventricle mass index (LVMi), cardiac index, and peak tricuspid regurgitation velocity (TRV).
  • LV filling pressures were evaluated using the E/e' ratio.
  • Sixty HbSC patients were compared to 60 SCA patients and 60 age/gender-matched controls.

Main Results:

  • Both HbSC and SCA patients showed higher LVMi than controls; LV hypertrophy was significantly more prevalent in SCA (45%) than HbSC (5%).
  • SCA patients had elevated cardiac index and TRV, while HbSC patients' cardiac index and TRV were similar to controls.
  • Both genotypes exhibited higher E/e' ratios than controls, indicating diastolic dysfunction. In SCA, cardiac involvement correlated with anaemia/haemolysis; in HbSC, it linked to hypertension/obesity.

Conclusions:

  • Cardiac involvement and its underlying causes differ significantly between HbSC disease and SCA.
  • SCA is associated with more pronounced left ventricular hypertrophy.
  • Genotype-specific considerations are essential for echocardiographic assessment and interpretation in sickle cell disorders.

Keywords:
cardiologyhaemoglobinopathiessickle cell anaemiasickle cell disease

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