Gupta Abhinav, Kapoor Aditya, Phadke Shubha, Sinha Archana, Kashyap Shridhar, Khanna Roopali, Kumar Sudeep, Garg Naveen, Tewari Satyendra, Goel Pravin
Department of Cardiology, Sanjay Gandhi PGIMS, Lucknow, Uttar Pradesh, India.
Department of Genetics, Sanjay Gandhi PGIMS, Lucknow, Uttar Pradesh, India.
Ann Pediatr Cardiol. 2017 May-Aug;10(2):158-166. doi: 10.4103/apc.APC_132_16.
Global ventricular function often remains normal in patients with beta-thalassemia major (β-TM) until late. Tissue Doppler and strain imaging may be useful to assess regional myocardial function abnormalities in these patients.
Systolic (Sm), early diastolic (Em), and late diastolic (Am) (Em/Am) myocardial velocities at basal lateral and septal left ventricular (LV) segments, strain (S), and strain rate (SR) in basal and mid LV, right ventricular (RV) and septum were measured in 30 patients (β-TM, 12.4 ± 5.2 years, serum ferritin 2603.1 μg/L) and twenty controls (12.5 ± 5.2 years). Flow-mediated dilatation (FMD) vasodilatation as a measure of endothelial function was also assessed.
Patients had significantly higher LV mass index (169.45 ± 61.14 vs. 104.66 ± 24.42; = 0.009) while global LV Sm and diastolic function was similar to controls. Patients had significantly lower lateral Em velocity, Em (10.12 ± 1.16 vs. 17.9 ± 2.11; = 0.002), Em/Am ratio (0.811 ± 0.192 vs. 2.06 ± 0.62; = 0.001) at the basal lateral LV, lower strain values at the basal lateral LV (19.5 ± 4.17 vs. 24.196 ± 1.81; = 0.002), mid lateral LV (19.07 ± 3.98 vs. 25.56 ± 2.62; = 0.042), basal septum (17.04 ± 3.44 vs. 25.43 ± 2.53; = 0.001), and mid septum (20.49 ± 5.34 vs. 24.45 ± 2.20; = 0.001) as compared to controls. SR at the basal and mid segment of the lateral LV wall and at the basal and mid septum was also significantly lower in patients. SR in basal and mid RV although lower was not significantly different from controls. Patients also had significantly lower FMD (7.57 ± 3.16 vs. 18.08 ± 1.9, = 0.018) implying endothelial dysfunction.
Tissue Doppler, strain and SR imaging are useful to quantify regional myocardial function in asymptomatic β-TM patients with preserved global Sm and diastolic function.
重型β地中海贫血(β-TM)患者的整体心室功能通常直到晚期仍保持正常。组织多普勒和应变成像可能有助于评估这些患者的局部心肌功能异常。
对30例患者(β-TM,12.4±5.2岁,血清铁蛋白2603.1μg/L)和20例对照者(12.5±5.2岁)测量左心室(LV)基底侧壁和间隔的收缩期(Sm)、舒张早期(Em)和舒张晚期(Am)(Em/Am)心肌速度、LV基底和中间段、右心室(RV)及间隔的应变(S)和应变率(SR)。还评估了作为内皮功能指标的血流介导的血管扩张(FMD)。
患者的LV质量指数显著更高(169.45±61.14对104.66±24.42;P = 0.009),而LV整体Sm和舒张功能与对照者相似。与对照者相比,患者LV基底侧壁的Em速度、Em(10.12±1.16对17.9±2.11;P = 0.002)、Em/Am比值(0.811±0.192对2.06±0.62;P = 0.001)显著更低,LV基底侧壁(19.5±4.17对24.196±1.81;P = 0.002)、中间侧壁(19.07±3.98对25.56±2.62;P = 0.042)、基底间隔(17.04±3.44对25.43±2.53;P = 0.001)和中间间隔(20.49±5.34对24.45±2.20;P = 0.001)的应变值也更低。患者LV外侧壁基底和中间段以及基底和中间间隔的SR也显著更低。RV基底和中间段的SR虽然更低,但与对照者无显著差异。患者的FMD也显著更低(7.57±3.16对18.08±1.9,P = 0.018),提示内皮功能障碍。
组织多普勒、应变和SR成像有助于量化整体Sm和舒张功能保留的无症状β-TM患者的局部心肌功能。