Revista Argentina de Cardiología | 2019
Ecografía Doppler strain 2D en la evaluación de la función ventricular en pacientes con diagnóstico reciente de hemocromatosis hereditaria
Abstract
espanolIntroduccion: La hemocromatosis hereditaria es una enfermedad genetica que genera una sobrecarga sistemica de hierro, y en etapas avanzadas, puede afectar el corazon. El compromiso miocardico precoz por esta enfermedad no ha sido suficientemente investigado. Objetivo: Comparar mediante ecocardiografia Doppler convencional y strain 2D un grupo de pacientes con diagnostico reciente de hemocromatosis hereditaria vs. un grupo control. Material y metodos: Se estudiaron pacientes con diagnostico reciente de hemocromatosis hereditaria, no tratados. Se les realizo un ecocardiograma Doppler convencional y con strain bidimensional para evaluar la deformacion longitudinal, radial y circunferencial, y el giro y torsion del ventriculo izquierdo. Resultados: Participaron 23 varones con hemocromatosis hereditaria (46 ± 18 anos) y 20 sujetos control (45 ± 15 anos). No hubo diferencias en los espesores y tamanos ventriculares. Los pacientes con hemocromatosis hereditaria tuvieron una fraccion de eyeccion del ventriculo izquierdo menor (59 ± 4% vs. 62 ± 4 %; p = 0,01). No hubo diferencias en cuanto a ESPAM, TAPSE u onda s´ del ventriculo izquierdo. Se observo una disminucion significativa del strain en los pacientes con HH, con mayor compromiso de la deformacion radial (37 ± 12 % vs. 55 ± 17 %; p = 0,01) y circunferencial (−19,5 ± 2,8 % vs. −22,5 ± 2,8 %; p = 0,001), y, en menor medida, de la longitudinal (−19 ± 1,8 % vs. −21,1 ± 2,5 %; p=0,04). Tambien este grupo tuvo menor rotacion apical, menor giro (17,7 ± 13° vs. 25 ± 7°; p = 0,03) y menor torsion (2,3 ± 1,8 °/cm vs. 3,3 ± 1,1 °/cm; p = 0,03). No hubo correlacion entre los diferentes tipos de deformacion y los parametros bioquimicos del metabolismo ferrico. Conclusiones: La ecocardiografia y especialmente la evaluacion del strain 2D es capaz de detectar de manera temprana ligeras alteraciones de la mecanica ventricular en pacientes asintomaticos, con sobrecarga sistemica de hierro por hemocromatosis hereditaria. EnglishBackground: Hereditary hemochromatosis is a genetic disorder characterized by systemic iron overload which can involve the heart 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 strain echocardiography 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 patients underwent 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 fraction was 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 rotation showed 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 no correlation between the different strain parameters and iron metabolism. Conclusions: Echocardiography, and particularly 2D strain analysis can detect early abnormalities of ventricular mechanics in asymptomatic patients with systemic iron overload due to hereditary hemochromatosis