Right ventricular abnormalities in sickle cell anemia: evidence of a progressive increase in pulmonary vascular

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Evaluation of Right Ventricular Function in Experimental Models of Pulmonary Arterial Hypertension
10:03

Evaluation of Right Ventricular Function in Experimental Models of Pulmonary Arterial Hypertension

Published on: June 27, 2025

Naveen Qureshi1, James J Joyce, Ning Qi

  • 1Department of Pediatrics, David Geffen School of Medicine at UCLA, Torrance and Los Angeles, CA, USA. nqureshi@mail.cho.org

The Journal of Pediatrics
|July 25, 2006
PubMed

Insights

Sickle cell anemia (SCA) increases right ventricle (RV) mass and pulmonary hypertension risk in children. However, RV preload and systolic function remain stable during childhood in SCA patients.

Area of Science:

  • Pediatric Cardiology
  • Hematology
  • Cardiovascular Research

Background:

  • Sickle cell anemia (SCA) is a genetic blood disorder with known cardiac complications.
  • The right ventricle (RV) is susceptible to changes in sickle cell anemia due to altered hemodynamics and pulmonary vascular resistance.

Purpose of the Study:

  • To evaluate the impact of sickle cell anemia on right ventricle structure and function in children.
  • To determine if right ventricle abnormalities progress during childhood in SCA patients.

Main Methods:

  • Echocardiographic assessment of 32 children with SCA compared to healthy controls.
  • Key RV parameters measured: diastolic area index, fractional area change, free-wall mass index, heart rate-corrected ejection time (ETc), and tricuspid regurgitation (TR) gradient.

Main Results:

  • Children with SCA exhibited elevated RV ETc, diastolic area index, and free-wall mass index compared to controls.
  • RV fractional area change was similar between SCA patients and controls.
  • RV free-wall mass index increased with age in SCA patients, and 16% of subjects showed TR gradients indicative of pulmonary hypertension.

Conclusions:

  • Right ventricle preload and systolic function are not adversely affected during childhood in SCA.
  • Progressive increases in RV mass index and the incidence of pulmonary hypertension are observed in children with SCA, correlating with increased pulmonary vascular resistance.
Abstract

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