Left ventricular rotational abnormalities in hemophilia—insights from the three-dimensional speckle-tracking echocardiographic MAGYAR-Path Study

Quantitative Imaging in Medicine and Surgery

article

Attila Nemes¹  Árpád Kormányos¹  Péter Domsik¹  Nóra Ambrus¹  Nándor Gyenes¹  Klára Vezendi²  Imelda Marton²  Zita Borbényi³ 

[1] Department of Medicine, Faculty of Medicine, Albert Szent-Györgyi Clinical Center , University of Szeged;Department of Transfusiology, Faculty of Medicine, Albert Szent-Györgyi Clinical Center , University of Szeged;Division of Haematology, Department of Medicine, Faculty of Medicine, Albert Szent-Györgyi Clinical Center , University of Szeged

关键词: Three-dimensional (3D);    speckle-tracking;    echocardiography;    hemophilia;    left ventricular (LV);    rotation;    twist;   

DOI  :  10.21037/qims-21-30

学科分类：外科医学

来源: AME Publications

【 摘 要 】

Background: Hemophilia is an X-linked inherited disorder primarily affecting males, its major types are type A (deficiency in factor VIII) and B (deficiency in factor IX), and is considered to be the most common severe congenital coagulation factor deficiency. The present study was designed to test whether any differences in left ventricular (LV) rotational mechanics could be demonstrated between male patients with hemophilia and healthy controls using three-dimensional speckle-tracking echocardiography (3DSTE)-derived virtual LV models. Methods: The present study consisted of 17 patients with hemophilia, however, 3 patients were excluded due to insufficient image quality. In the remaining patient population, 12 patients had hemophilia A and 2 patients had hemophilia B (mean age: 42.2±18.9 years, all males). The control group comprised 16 age-matched healthy subjects (46.0±5.9 years, all males). Results: None of the routine two-dimensional echocardiographic data differ between patients with hemophilia and controls. None of the patients and controls showed ≥ grade 1 valvular regurgitations and had valvular stenoses. In one subject, the near absence of LV twist called as LV “rigid body rotation” could be detected, data of which were managed separately. While 3DSTE-derived apical LV rotation was 3.65 degrees, basal LV rotation proved to be 3.57 degrees leading to 0.08-degree LV apico-basal gradient suggesting counterclockwise LV “rigid body rotation”. In the remaining patients, both LV apical rotation (7.25±6.20 vs. 10.39±4.16 degrees, P<0.02) and LV twist (10.24±5.60 vs. 14.38±3.93 degrees, P<0.003) showed significant impairment in patients with hemophilia. Conclusions: LV rotational abnormalities are present in hemophilia with reduced LV apical rotation and twist.

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