Cardiac Chamber Quantification by Echocardiography in Adults With Sickle Cell Disease: Need Attention to Eccentric

Mahmut B Koyuncu1, Anil Tombak2, Ozcan Orscelik3

  • 1Hematology, Adana City Research and Training Hospital, Adana, TUR.

Cureus
|July 19, 2021
PubMed

Insights

Sickle cell anemia patients with eccentric hypertrophy show the lowest survival rates. Early cardiac disorder detection and treatment are crucial for reducing mortality in sickle cell cardiomyopathy.

Area of Science:

  • Cardiology
  • Hematology
  • Medical Imaging

Background:

  • Sickle cell anemia (SCA) is a prevalent hemoglobinopathy globally.
  • Cardiovascular diseases are the leading cause of mortality in SCA patients.
  • Cardiac remodeling, including hypertrophy, develops early and leads to sickle cell cardiomyopathy.

Purpose of the Study:

  • To determine the relationship between cardiac remodeling types and survival in SCA patients.
  • To identify factors influencing left ventricular mass in SCA.
  • To investigate the prognostic value of different cardiac remodeling patterns.

Main Methods:

  • Echocardiographic measurements of left ventricular mass index (LVMI) and relative wall thickness (RWT).
  • Categorization of 146 SCA patients into normal, concentric remodeling (CR), concentric hypertrophy (CH), and eccentric hypertrophy (EH) groups.
  • Logistic regression analysis to identify predictors of LVMI.

Main Results:

  • Hemoglobin S (HbS) and ferritin levels independently predicted LVMI.
  • Prevalence of remodeling types: normal (38.4%), CR (16.4%), CH (14.4%), EH (30.8%).
  • Overall mortality was 21.2%, with EH patients exhibiting significantly lower survival rates (log-rank p < 0.001).

Conclusions:

  • Cardiac death is a major cause of mortality in SCA.
  • Eccentric hypertrophy is associated with the poorest survival outcomes.
  • Early detection and management of cardiac abnormalities are vital for improving survival in SCA patients.

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