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Updated: Sep 12, 2025

A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy
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Cardiovascular disease in Sickle cell: mechanisms, diagnostics and therapeutic advances

Elio Haroun1, Ankit Agrawal1, Aro Daniela Arockiam1

  • 1Department of Cardiovascular Medicine, Heart, Vascular and Thoracic Institute, Cleveland Clinic, Cleveland, Ohio, USA.

Heart (British Cardiac Society)
|August 8, 2025
PubMed

Insights

Cardiovascular complications in sickle cell disease (SCD) are a major cause of early death. This review details SCD

Area of Science:

  • Cardiology
  • Hematology
  • Genetics

Background:

  • Cardiovascular complications significantly contribute to morbidity and mortality in sickle cell disease (SCD).
  • These complications are often underdiagnosed and underappreciated, necessitating a comprehensive review of current knowledge.
  • Understanding the unique pathophysiological mechanisms in SCD is crucial for effective management.

Purpose of the Study:

  • To synthesize current knowledge on cardiovascular manifestations in SCD.
  • To highlight emerging diagnostic tools and their findings.
  • To present practical algorithms for risk stratification, monitoring, and management.

Main Methods:

  • Comprehensive literature review of cardiovascular complications in SCD.
  • Emphasis on unique pathophysiological mechanisms.
  • Integration of multimodality imaging, updated guidelines, and clinical insights.

Main Results:

  • Spectrum of cardiovascular manifestations detailed: myocardial dysfunction, pulmonary hypertension, cardiac iron overload, arrhythmias, myocardial infarction, stroke, and sudden death.
  • Emerging diagnostic tools like cardiac MRI (T2* mapping, ECV) and speckle-tracking echocardiography reveal a distinct phenotype.
  • Phenotype characterized by restrictive cardiomyopathy and high-output heart failure.

Conclusions:

  • Early recognition and personalized therapy are essential for improved cardiovascular outcomes in SCD.
  • Multimodality imaging and updated management strategies are key.
  • This review serves as a comprehensive resource for clinicians managing SCD patients.

Keywords:
CARDIOMYOPATHYDIASTOLIC DYSFUNCTIONPulmonary Arterial Hypertension

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