What does this research mean for the field?

Subtle left ventricular systolic and diastolic dysfunction develops early in young, asymptomatic patients with thalassemia intermedia despite normal cardiac iron concentrations, with left ventricular remodeling playing a principal role in this impairment. Novelty: ClaimNovelty.INCREMENTAL. Consensus alignment: ConsensusAlignment.NEUTRAL.

June 17, 2022Open Access

Subclinical Left Ventricular Dysfunction in Children and Adolescence With Thalassemia Intermedia

RTRoya Isa TafreshiIran University of Medical Sciences MRMohammad RadgoodarziIran University of Medical Sciences KRKadijeh Arjmandi RafsanjaniIran University of Medical Sciences

Structured PICO

Does thalassemia intermedia cause subclinical left ventricular dysfunction in asymptomatic children and adolescents compared to healthy controls?

Population

28 asymptomatic children and adolescents (age 7-21 years, mean age 13.6 ± 5.7 years) with thalassemia intermedia (TI), NYHA functional class I, normal renal and thyroid function, absence of congenital or acquired heart disease, no regular transfusions or chelating agents, and normal cardiac iron load (T2* > 20 ms).

Comparator

35 age-matched healthy control children referred for investigation of a heart murmur.

Outcome

Left ventricular systolic and diastolic function assessed by tissue Doppler imaging (TDI) indices including peak systolic myocardial velocity (s'), E/e' ratio, myocardial performance index (MPI), and myocardial acceleration during isovolumic contraction (IVA).surrogate

Tissue Doppler imaging reveals early, subclinical left ventricular systolic and diastolic dysfunction in young, asymptomatic patients with thalassemia intermedia despite normal cardiac iron levels and normal ejection fraction.

Abstract

Background Cardiac complications are important causes of morbidity in patients with thalassemia intermedia (TI). We aimed to assess left ventricular (LV) function, using new tissue Doppler imaging (TDI) indices, in order to diagnose early ventricular impairment in asymptomatic children and adolescence with the TI. Materials and Methods We investigated possible differences in echocardiographic systolic and diastolic parameters between a population of 28 asymptomatic patients (mean age, 13.6 ± 5.7 years) and 35 age-matched healthy control members. All of them underwent 2-D, pulsed Doppler, and tissue Doppler echocardiographic studies for the assessment of the LV mass, Trans-mitral velocities, mitral annular systolic and diastolic velocities, myocardial performance index (MPI), and myocardial acceleration during isovolumic contraction (IVA). The cardiac iron load was estimated by magnetic resonance imaging T2*. Results Left ventricular hypertrophy (LVH) was found in 13 (46.4%) patients. We found significantly reduced TDI-derived peak systolic myocardial velocity (s′) in patients, whereas no significant difference was identified between the patients and control group members when the IVA was compared. The ratio of peak mitral inflow velocity to annular early diastolic velocity (E/e′) of the mitral valve as an index of the diastolic function was significantly higher in patients (9 ± 1 vs. 6 ± 1, p 0.05). Choosing a TDI-derived MPI 0.33 as a cutoff point, the global LV dysfunction was detected with a sensitivity of 78% and a specificity of 80%. The patients with LVH significantly exhibited higher values of TDI-MPI and lower values of s′ velocity and IVA when compared against the subjects with normal LV mass. Conclusion Subtle LV systolic and diastolic dysfunction develops early in young patients with the TI who have normal cardiac iron concentration. Moreover, LV remodeling as a main cardiac adaptive response plays a principal role in developing myocardial impairment.

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