Diastolic Dysfunction with Normal Heart T2* in Thalassemia Major with Heart Failure. a Single Centre Study
Authors: Mancuso Luigi , Vitrano Angela , Sacco Massimiliano , Mancuso Andrea , Ledda Antonietta , Maggio Aurelio 
Published: October 2019

Background Heart failure (HF) is the most important cause of death in Thalassemia Major (TM) patients, and results from iron overload which determines progressive systolic dysfunction of the left ventricle. T2* Magnetic Resonance Imaging (CMR) is the only non-invasive tool for detecting and quantifying myocardial iron storage.We had observed that a large number of Thalassemia patients recently observed at our Centre develops a different form of HF, with evidence of diastolic dysfunction and often in absence of systolic dysfunction.

Methods We evaluated the clinical, electrocardiographic, echocardiographic and Doppler data of 16 adult Thalassemia patients with HF observed at our Centre between 2008 and 2016, together with the data obtained by means of T2* CMR. All statistical analyses were descriptive. Results are provided as means ± standard deviations, medians with interquartile ranges (IQR), and percentages.

Results Table 1 describes demographics, T2* and Echo-Doppler data of 16 TM patients. The 31.2% were females and the mean age was 44.2±5.7 years.One patient presented systolic dysfunction of the left ventricle whereas the others had echocardiographic and Doppler evidence of diastolic dysfunction. Systolic dysfunction of the right ventricle was also found in 81.25% of cases.

Furthermore, 30.75% of cases had T2* values consistent with significant risk for heart failure (≤14 ms), whereas the others had normal values. In 68.75% of the cases ECG showed inversion of T wave beyond V2 lead, and low voltages.

Conclusions Most of the patients with heart failure recently observed at our Centre had diastolic dysfunction of the left ventricle with normal systolic function, and impairment of systolic function of the right ventricle, and normal values of cardiacT2*. In 68.75% of cases ECG showed inversion of T wave beyond V2 lead and low voltages. Limitations of this study can be summarizes in: a) small number of cases (16 pts); b) Evidence of normal values of T2* values in most patients does not exclude an iron overload in precedent years. However patients with HF due to systolic dysfunction usually show low or very low values; c) a possible bias of this study may be linked to the Centre where this study has been performed. Our Centre is the Reference Centre of Sicilian Region for Thalassemia patients. This implies the possibility of a very strict surveillance of chelation therapy with frequent evaluations of the data of T2* in order to improve at best the treatment with chelation therapy. It is possible that this, at least in part, might prevent the onset of the classical form of systolic dysfunction of the left ventricle due to iron overload, and that in these patients, differently than in patients followed up in other centres, different forms of HF noit linked to cardiac iron overload may occur: that is heart failure preserved ejection fraction (HFpEF), with prevalent left ventricular diastolic dysfunction.

Table 1. Demographics, Echo-Doppler and T2* data Table 1. Demographics, Echo-Doppler and T2* data

Disclosures

No relevant conflicts of interest to declare.