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Echocardiografic abnormalities in patients with sickle cell/β-thalassemia do not depend on the β-thalassemia

Bruno Deltreggia Benites1, Ianara Silva Cisneiros1, Stephany Oliveira Bastos1

  • 1Universidade Estadual de Campinas (UNICAMP), Campinas, SP, Brazil.

Hematology, Transfusion and Cell Therapy
|May 16, 2019
PubMed

Insights

Cardiac involvement in sickle cell/β-thalassemia is linked to multi-organ issues, not the specific thalassemia type. Older patients with myocardial remodeling face higher stroke risk, needing careful management.

Area of Science:

  • Cardiology
  • Hematology
  • Genetics

Background:

  • Sickle cell/β-thalassemia is a complex hemoglobinopathy.
  • Cardiac involvement is a known complication, but its determinants are not fully understood.

Purpose of the Study:

  • To investigate echocardiographic findings in Brazilian patients with sickle cell/β-thalassemia.
  • To explore relationships between cardiac parameters, clinical status, and laboratory findings.

Main Methods:

  • Echocardiographic assessment of cardiac structure and function.
  • Correlation analysis with clinical and laboratory data, including creatinine, transaminases, and bilirubin.
  • Comparison of cardiac parameters between Sβ0 and Sβ+ phenotypes.

Main Results:

  • Left atrial and left ventricular dilation observed in 19.5% and 11% of patients, respectively.
  • Altered ventricular geometry correlated significantly with elevated serum creatinine, hepatic transaminases, and bilirubin.
  • Stroke patients were older and showed increased left ventricular posterior wall thickness, left ventricular mass, and left atrial/aortic ratio.

Conclusions:

  • Cardiac involvement in sickle cell/β-thalassemia is not phenotype-dependent.
  • Myocardial remodeling is associated with multi-organ impairment.
  • Older patients with cardiac remodeling have a higher stroke risk, necessitating vigilant management of associated factors.
Abstract

Keywords:
Beta-thalassemiaEchocardiographySickle cell disease

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