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A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy
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Evaluation of cardiac sequelae in patients with sickle cell anemia

Hamid Amoozgar1, Leila Dastgheib, Vahid Seifi

  • 1Division of Pediatric Cardiology, Shiraz University of Medical Sciences, Shiraz, Iran.

Hellenic Journal of Cardiology : HJC = Hellenike Kardiologike Epitheorese
|May 30, 2015
PubMed

Insights

Sickle cell anemia in children causes a volume-overloaded heart with enlarged left ventricular dimensions, but without frequent systolic dysfunction or pulmonary hypertension.

Area of Science:

  • Cardiology
  • Hematology
  • Pediatrics

Background:

  • Sickle cell anemia (SCA) is a genetic blood disorder.
  • Cardiac complications are a significant concern in SCA patients.

Purpose of the Study:

  • To evaluate cardiac function in children with SCA using echocardiography.
  • To identify echocardiographic changes associated with SCA.

Main Methods:

  • A case-control study comparing 44 SCA patients with 44 healthy controls.
  • Utilized M-mode, 2D, Doppler, and pulse tissue-Doppler echocardiography.

Main Results:

  • SCA patients showed increased early diastolic trans-tricuspid peak flow velocity.
  • Significantly greater systolic, early, and late diastolic velocities in mitral and tricuspid annuli were observed in SCA patients.
  • Increased left ventricular diameter, interventricular septum, and posterior wall diameter were noted in SCA patients, with no difference in ejection fraction.

Conclusions:

  • SCA in children leads to a volume-overloaded heart and increased left ventricular dimensions.
  • Left ventricular systolic dysfunction and pulmonary hypertension were infrequent in this pediatric SCA cohort.
Abstract

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