Two-dimensional Strain Echocardiography in the Evaluation of Ventricular Function in Patients with Newly Diagnosed Hereditary Hemochromatosis

J. Martín Aladio, Florencia Yamasato, Ariel K. Saad, Juan Sordá, Oscar Grosso, Esteban González Ballerga, Jorge Daruich, Sara Berensztein


Background: Hereditary hemochromatosis is a genetic disorder characterized by systemic iron overload which can involve theheart in advanced stages. Early myocardial involvement has not been thoroughly investigated.Objective: The aim of our study was to evaluate the performance of conventional Doppler-echocardiography and 2D strainechocardiography in a group of patients with newly diagnosed hereditary hemochromatosis vs. a control group.Methods: Patients with newly diagnosed hereditary hemochromatosis without treatment were included. All the patientsunderwent conventional Doppler echocardiography and 2D strain echocardiography with evaluation of left ventricular longitudinal,radial and circumferential strain, twist and torsion.Results: Twenty-three male patients with hereditary hemochromatosis (46±18 years) and 20 male controls (45±15 years)were included. There were no differences in ventricular dimensions and wall thickness. Left ventricular ejection fractionwas lower in patients with hereditary hemochromatosis (59±4% vs. 62±4%; p=0.01). There were no differences in MAPSE,TAPSE and left ventricular s’ velocity. There was a significant decrease in global strain in patients with hereditary hemochromatosis,with greater involvement of radial strain (37±12 % vs. 55±17%; p=0.01) and circumferential strain (-19.5±2.8% vs.-22.5±2.8%; p=0.001), and less involvement of longitudinal strain (-19±1.8% vs. -21.1±2.5%; p=0.04). Myocardial rotationshowed lower twist (17.7±13° vs. 25±7°; p=0.03) and lower torsion (2.3±1.8°/cm vs. 3.3±1.1 °/cm; p=0.03). There was nocorrelation between the different strain parameters and iron metabolism.Conclusions: Echocardiography, and particularly 2D strain analysis can detect early abnormalities of ventricular mechanicsin asymptomatic patients with systemic iron overload due to hereditary hemochromatosis.


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