Multiparametric Cardiac Magnetic Resonance Assessment in Sickle Beta Thalassemia

Laura Pistoia1,2, Antonella Meloni2,3, Vincenzo Positano2,3

  • 1Unità Operativa Complessa Ricerca Clinica, Fondazione G. Monasterio CNR-Regione Toscana, 56124 Pisa, Italy.

PubMed

Insights

Cardiac function in sickle beta thalassemia patients is similar to sickle cell anemia patients but differs from healthy individuals. New reference values for cardiac parameters in sickle beta thalassemia are needed.

Area of Science:

  • Cardiology
  • Hematology
  • Medical Imaging

Background:

  • Cardiac involvement in sickle beta thalassemia (Sβ-thal) is understudied.
  • Cardiovascular magnetic resonance (CMR) is crucial for assessing cardiac function and iron overload.

Purpose of the Study:

  • To evaluate cardiac function and myocardial iron overload in Sβ-thal patients using CMR.
  • To compare cardiac parameters between Sβ-thal, sickle cell anemia (SCA), and healthy individuals.

Main Methods:

  • 111 Sβ-thal patients, 46 SCA patients, and 111 healthy volunteers were studied.
  • CMR assessed biventricular function, myocardial iron overload (T2*), and fibrosis (LGE).

Main Results:

  • Sβ-thal and SCA patients showed similar cardiac morphology, function, iron overload, and fibrosis.
  • Sβ-thal patients had altered bi-atrial and biventricular parameters compared to healthy subjects, with lower LV ejection fraction.
  • SCA patients had higher left atrial area and LV stroke volume index than Sβ-thal patients.

Conclusions:

  • CMR analysis confirms phenotypic similarity between Sβ-thal and SCA patients.
  • Sβ-thal patients exhibit significant cardiac morphological and functional differences from healthy individuals.
  • Establishing Sβ-thal/SCA-specific reference values for cardiac parameters is recommended.