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Left Ventricular Structural and Functional Changes in Children With β-Thalassemia and Sickle Cell Disease:

Mohsen S Elalfy1, Omneya Ibrahim Youssef, Marwa M R Deghedy

  • 1Department of Paediatrics, Faculty of Medicine, Ain Shams University, Cairo, Egypt.

Journal of Pediatric Hematology/Oncology
|March 2, 2018
PubMed

Insights

Sleep-disordered breathing (SDB) is common in children with beta-thalassemia and sickle cell disease (SCD). SDB is linked to significant left ventricular (LV) structural and functional changes in these patients.

Area of Science:

  • Cardiology
  • Hematology
  • Sleep Medicine

Background:

  • Cardiovascular complications are prevalent in beta-thalassemia and sickle cell disease (SCD).
  • The impact of sleep-disordered breathing (SDB) on cardiovascular health in these pediatric populations is not fully understood.

Purpose of the Study:

  • To investigate left ventricular (LV) structural and functional changes in children with beta-thalassemia and SCD.
  • To determine the association between sleep-disordered breathing (SDB) and these cardiovascular alterations.

Main Methods:

  • One hundred pediatric patients with beta-thalassemia or SCD were assessed using the Pittsburgh Sleep Quality Index.
  • Patients with positive scores underwent polysomnography and tissue Doppler echocardiography.
  • Comparison was made with age- and sex-matched healthy controls.

Main Results:

  • Sleep-disordered breathing (SDB) was detected in 73% of beta-thalassemia patients and 46% of SCD patients.
  • SDB was associated with increased LV mass index, diastolic dysfunction, and pulmonary hypertension in both groups.
  • Lower sleep oxygen saturation correlated with adverse LV structural and functional parameters in both beta-thalassemia and SCD.

Conclusions:

  • Sleep-disordered breathing (SDB) is a common comorbidity in children with beta-thalassemia and SCD.
  • SDB is significantly associated with detrimental left ventricular (LV) structural and functional changes, including increased LV mass, diastolic dysfunction, and pulmonary hypertension.
  • These findings highlight the importance of screening for SDB in pediatric patients with these hematologic disorders.

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