Guideline recommendations for heart complications in thalassemia major

Abstract

Thalassemia major is an inherited hemoglobin disorder resulting in a chronic hemolytic anemia. Transfusion therapy together with elevated gastrointestinal absorption of iron determines iron overload, which causes most of the mortality and morbidity associated with the disease. Heart complications represent the leading cause of mortality in this disease, although, because of an improvement in chelation treatment, an important and progressive increase of life expectancy mainly as a result of a reduction in mortality due to cardiac dysfunction has been demonstrated in recent years. Clinical pictures of heart damage range from the involvement of the ventricles to pulmonary hypertension or symptomatic ventricular or supra-ventricular arrhythmias. For this reason, the possibility of having specific recommendations is noteworthy. These recommendations outline the definition, the follow-up and the treatment of the main heart complications in this group of patients. The identification of topics and the nomination of the committee were made on behalf of the Society for the Study of Thalassemia and Hemoglobinopathies (SoSTE). The document obtained the auspices of ANMCO, SIC, SIRM and the Cardiovascular Magnetic Resonance Working Groups of the ANMCO, SIC and SIRM. All recommendations provided in this document have been performed according to the American Cardiology College (ACC) and American Heart Association (AHA) guidelines. Moreover, the recommendations were reviewed by two external referees before the definitive approval.

Influence of myocardial fibrosis and blood oxygenation on heart T2* values in thalassemia patients

“…MRI represents the only noninvasive technique to quantify myocardial iron burden (30). In particular, iron overload in MRI is associated with an effect termed susceptibility-induced relaxation (31).…”

Single region of interest versus multislice T2* MRI approach for the quantification of hepatic iron overload

“…MRI is the only technique that quantitatively and noninvasively assesses both myocardial and liver burden (4). MRI multi-echo T2-star (T2*) sequences allow rapid monitoring of iron deposition, exploiting the fact that paramagnetic iron compounds produce variability in susceptibility, shortening the T2* relaxation time (5).…”

A novel hemoglobin-binding peptide reduces cell-free hemoglobin in murine hemolytic anemia

“…Indeed, intravascular hemolysis has been linked to pulmonary hypertension (19,24,26,45,59), systemic vasculopathies (27,28,50,52,53,60), and cardiovascular complications (11,25,30,39). Although such studies have suggested that CF-Hb likely plays important roles in vascular disease, the actual contribution of CF-Hb to disease pathology has drawn controversy, particularly in sickle cell disease (SCD) (9,18).…”