Related Experiment Video

Updated: Jul 7, 2026

Detection of Residual Donor Erythroid Progenitor Cells after Hematopoietic Stem Cell Transplantation for Patients with Hemoglobinopathies
11:59

Detection of Residual Donor Erythroid Progenitor Cells after Hematopoietic Stem Cell Transplantation for Patients with Hemoglobinopathies

Published on: September 6, 2017

Early impairment of myocardial function in young patients with beta-thalassemia major

Damiano Magrì1, Susanna Sciomer, Francesco Fedele

  • 1Dipartimento di Scienze Cardiovascolari, Respiratorie e Morfologiche, Università degli Studi di Roma La Sapienza, Rome, Italy. damiano.magri@libero.it

European Journal of Haematology
|February 21, 2008
PubMed

Insights

Young patients with beta-thalassemia major (TM) show impaired heart function, detectable by advanced ultrasound (TDI/SI). Deferiprone may offer better myocardial protection than deferoxamine in TM patients.

Area of Science:

  • Cardiology
  • Hematology
  • Medical Imaging

Background:

  • Heart failure from iron overload is a primary cause of mortality in beta-thalassemia major (TM).
  • Early detection of cardiac dysfunction is crucial for managing TM patients.

Purpose of the Study:

  • To compare myocardial function in young, asymptomatic TM patients and healthy controls using echocardiography (TDI/SI) and cardiac MRI.
  • To assess the impact of different iron chelation therapies (deferoxamine vs. deferiprone) on cardiac function in TM.

Main Methods:

  • Echocardiography with tissue Doppler imaging (TDI) and strain imaging (SI) was performed on 30 TM patients and 30 controls.
  • Cardiac MRI with T2* technique was used to assess myocardial iron levels.
  • TM patients were subgrouped based on iron chelation therapy (deferoxamine or deferiprone).

Main Results:

  • TM patients exhibited lower systolic myocardial velocities (Sm) and systolic strain (S) in the left and right ventricles compared to controls.
  • Myocardial function and T2* values were better in patients treated with deferiprone versus deferoxamine.
  • Advanced ultrasound parameters correlated well with MRI findings.

Conclusions:

  • Even in young, asymptomatic, and well-chelated TM patients, myocardial function is impaired.
  • Advanced echocardiographic techniques (TDI and SI) can effectively detect cardiac dysfunction in TM.
  • Deferiprone may offer superior myocardial protection in TM patients, warranting further investigation.
Abstract

More Related Videos

Intramyocardial Cell Delivery: Observations in Murine Hearts08:12

Intramyocardial Cell Delivery: Observations in Murine Hearts

Published on: January 24, 2014

Related Concept Videos

Myocarditis I: Introduction01:21

Myocarditis I: Introduction

Myocarditis is inflammation of the myocardium, which is the muscular layer of the heart.EtiologyMyocarditis has a diverse etiology, including a wide range of infectious and non-infectious causes:Infectious CausesViral: Common viruses include Coxsackie A and B, adenovirus, parvovirus B19, enteroviruses, and influenza A.Bacterial: Examples include infections caused by Streptococcus, Staphylococcus, and Mycoplasma species.Rickettsial: Infections like Rocky Mountain spotted fever can result in...
Cardiomyopathy III: Hypertrophic Cardiomyopathy01:29

Cardiomyopathy III: Hypertrophic Cardiomyopathy

Hypertrophic cardiomyopathy, or HCM, is an autosomal dominant genetic disorder characterized by asymmetric left ventricular hypertrophy without ventricular dilation. It is more common in men and is typically diagnosed in young, athletic adults.EtiologyHCM is primarily genetic and is caused by mutations in genes encoding sarcomeric proteins. Researchers have identified over 1400 mutations across at least 11 different genes. Among these, the most frequently occurring mutations are found in the...
Cardiomyopathy IV: Restrictive Cardiomyopathy01:29

Cardiomyopathy IV: Restrictive Cardiomyopathy

Restrictive cardiomyopathy (RCM) is a rare heart muscle disease characterized by impaired ventricular filling due to stiffened ventricular walls, leading to significant diastolic dysfunction.EtiologyRestrictive cardiomyopathy can arise from both inherited and acquired diseases, many of which are systemic. It is categorized into four main types: infiltrative, storage, non-infiltrative, and endomyocardial diseases.Infiltrative diseases, such as amyloidosis, lead to RCM by depositing amyloid...
Cardiomyopathy II: Dilated Cardiomyopathy01:30

Cardiomyopathy II: Dilated Cardiomyopathy

Dilated cardiomyopathy, or DCM, is a progressive myocardial disorder characterized by ventricular chamber dilation and contractile dysfunction.EtiologyVarious factors can cause DCM, including hypertension and heavy alcohol intake, which contribute to the weakening and enlargement of the heart muscle. Viral infections, such as Coxsackievirus B, adenoviruses, and influenza, can lead to DCM by causing inflammation and damage to heart tissue. Certain chemotherapeutic agents, including daunorubicin,...
Cardiomyopathy I: Introduction and Classification01:25

Cardiomyopathy I: Introduction and Classification

Cardiomyopathy, or CMP, is a group of diseases affecting the myocardial structure, impairing its ability to pump blood effectively. This condition can lead to arrhythmias, heart failure, or sudden cardiac death.Cardiomyopathies are classified into primary and secondary categories:Primary Cardiomyopathy refers to conditions involving only the heart muscle that are often idiopathic (of unknown cause) or genetic. They primarily affect the myocardium without the involvement of other systemic...
Myocarditis III: Medical Management01:14

Myocarditis III: Medical Management

Myocarditis: Comprehensive Medical ManagementMyocarditis, the heart muscle inflammation, requires a comprehensive medical management strategy that addresses the underlying cause, provides supportive care, manages symptoms, and reduces cardiac workload.Infections and Autoimmune CausesAdminister appropriate antimicrobial therapy when an infectious agent causes myocarditis. For instance, penicillin treats infections caused by Group A Streptococcus. In cases where autoimmune processes are...