Reference ranges for biventricular volumes and ejection fraction and for left ventricular mass in adult thalassemia intermedia patients without myocardial iron overload

Abstract

Purpose: Thalassemia intermedia (TI) patients have significantly higher cardiac output and volumes with respect to thalassemia major patients. This study aimed to establish the ranges for normal biventricular volumes and ejection fraction (EF) and for left ventricular (LV) mass assessed by cardiovascular magnetic resonance (CMR) in TI. Methods: Among the 294 adult TI patients enrolled in the MIOT network, we selected 68 patients with no known risk factors or history of cardiac disease, normal electrocardiogram, no myocardial iron overload or fibrosis. Biventricular parameters were quantitatively evaluated by cine images using MASS® software. LV and right ventricular (RV) end-diastolic volume (EDV), end-systolic volume (ESV) and stroke volume (SV) were normalized by body surface area (EDVI, ESVI, SVI), as well as the LV mass. (Tabel Presented) Results: The selected patients had a mean age of 36.5±9.2 years and 37 were males. Biventricular volumes indexes (with the exception of the RV ESVI) and LV mass were significantly larger in males than in females while the EFs were not different. Biventricular parameters are detailed in Table 1 with differentiation for sex. Table 1 reports also the cut-off of normality defined as mean - 2 standard deviation (SD) for the volumes and the LV mass and as mean - 1 SD for the EF (considering the high cardiac output state in anemic patients). Conclusions: Reference ranges for biventricular volumes and function specific to adult TI patients were defined. These new reference ranges are important for avoiding a misdiagnosis of cardiomyopathy in TI patients.