Pediatric Hematology/Oncology and ImmunopathologyPediatric Hematology/Oncology and ImmunopathologyВопросы гематологии/онкологии и иммунопатологии в педиатрии1726-17082414-9314Fund Doctors, Innovations, Science for Children16610.24287/1726-1708-2016-15-3-23-28HEMATOLOGYГЕМАТОЛОГИЯEchocardiographic assessment of the state of the heart in children with ß-thalassemiaЭхокардиографическая оценка состояния сердца у детей с ß-талассемиейNikolaevaGalina N.НиколаеваГалина Николаевна
Russian Federation
galina-2020@yandex.ruSugakAnna B.СугакАнна Борисовна
Russian Federation
sugak08@mail.ruFeoktistovaElena V.ФеоктистоваЕлена Владимировна
Russian Federation
9433672@mail.ruKurovIl'ya O.КуровИлья Олегович
Russian Federation
kurov@phystech.eduDmitry Rogachev Federal Research Centre of Paediatric Haematology, Oncology and Immunology, Ministry of Health of the Russian FederationФедеральный научно-клинический центр детской гематологии, онкологии и иммунологии им. Дмитрия Рогачева Минздрава РоссииMoscow Institute of Physics and Technology (State University)Московский физико-технический институт (государственный университет)19092016153232819092018Copyright ©; 2016, «D. Rogachev NMRCPHOI»Copyright ©; 2016, ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава России2016«D. Rogachev NMRCPHOI»ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава Россииhttps://creativecommons.org/licenses/by/4.0https://hemoncim.com/jour/article/view/166

The main causes of the development of thalassemia-associated cardiomyopathy are volume overload of the heart resulting from adaptation to chronic anaemia and iron toxic effects on the myocardium resulting from replacement blood transfusion therapy. In order to reveal left ventricle (LV) dysfunction echocardiography (Echo-CG) with examination of the mitral valve fibrous ring (MV FR) motion was performed in the tissue Doppler mode in 50 patients with ß-thalassemia and 47 conditionally healthy children aged 2 to 16 years. Patients with ß-thalassemia as compared with the control group had a significantly larger LV diameter, higher LV volume indices, LV myocardial mass index, left atrial diameter, cardiac index, transmitral flow velocity, MV FR motion diastolic velocities and a ratio between LV early diastolic filling velocity and MV FR motion early diastolic velocity, significantly smaller LV ejection fraction (p < 0.5). LV wall thickness, LV myocardial mass, heart rate, LV stroke volume, LV minute volume, ratios between transmitral flow velocities, MV FR motion systolic velocities did not differ among groups. In patients with ß-thalassemia, echo-CG permits to diagnose dilatation, hypertrophy and hyperkinesis of LV associated with cardiac volume overload. The hyperkinetic status might mask early signs of diastolic dysfunction. For diagnosing early signs of systolic dysfunction it is necessary to develop reference values of LV ejection fraction for patients with ß-thalassemia.

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