De Chiara Benedetta, Crivellaro Wilma, Sara Roberto, Ruffini Livia, Parolini Marina, Fesslovà Vlasta, Carnelli Vittorio, Fiorentini Cesare, Parodi Oberdan
Section of Milan, CNR Clinical Physiology Institute, Niguarda Hospital, Milan, Italy.
Eur J Haematol. 2005 Jun;74(6):517-22. doi: 10.1111/j.1600-0609.2005.00434.x.
Cardiac dysfunction remains the major cause of death in beta-thalassemia. Aim of this study was to assess early myocardial damage in thalassemic patients with no symptoms or echocardiographic evidence of dysfunction at routine monitoring.
Twenty patients (seven females; median 25 yr [first quartile 22,third quartile 28]) with beta-thalassemia underwent radionuclide angiography (RNA) at rest and during low-dose dobutamine infusion (5-10 gamma/kg/min). Right and left ventricular ejection fractions (EF) were determined by first-pass method and gated equilibrium acquisition, respectively. Twenty-four-hour Holter monitoring with time-domain heart rate variability (HRV) assessment and echocardiographic follow-up (21 months [5,27]) were performed.
Eleven patients showed regional wall motion abnormalities at RNA; left ventricular EF, HR and diastolic measurements significantly increased after dobutamine infusion. Patients with abnormal RNA right ventricular EF (n = 8, <0.45) showed lower echocardiographic left ventricular EF at the enrollment (0.54 [0.50,0.61] vs. 0.62 [0.56,0.67], P = 0.02) than those with a normal right ventricular EF. Patients with reduced standard deviation of the averages of RR intervals in all 5-minute periods of entire recording (SDANN) (n = 6, <100 ms), a measure of HRV, had lower echocardiographic left ventricular EF (0.53 [0.49,0.62] vs. 0.62 [0.56,0.66], P = 0.03) and lower fractional shortening (0.28 [0.25,0.32] vs. 0.36 [0.30,0.39], P = 0.003) at the enrollment than those with normal SDANN. No significant association was found between RNA and HRV measurements and follow-up left ventricular function.
Right ventricular dysfunction and abnormal HRV may represent the early features of cardiac disease in thalassemic patients with no evidence of ventricular dysfunction at routine evaluation.
心脏功能障碍仍是β地中海贫血患者的主要死因。本研究的目的是评估在常规监测中无症状或无超声心动图功能障碍证据的地中海贫血患者的早期心肌损伤。
20例β地中海贫血患者（7例女性；年龄中位数25岁[四分位数间距：第一四分位数22岁，第三四分位数28岁]）接受了静息和低剂量多巴酚丁胺输注（5 - 10μg/kg/min）期间的放射性核素血管造影（RNA）检查。右心室和左心室射血分数（EF）分别通过首次通过法和门控平衡采集法测定。进行了24小时动态心电图监测，评估时域心率变异性（HRV），并进行超声心动图随访（21个月[5,27]）。
11例患者在RNA检查时出现节段性室壁运动异常；多巴酚丁胺输注后左心室EF、心率和舒张期测量值显著增加。RNA检查时右心室EF异常（n = 8，<0.45）的患者在入组时的超声心动图左心室EF低于右心室EF正常的患者（0.54[0.50,0.61]对0.62[0.56,0.67]，P = 0.02）。在整个记录的所有5分钟时段中RR间期平均值标准差（SDANN）降低（n = 6，<100 ms）的患者，这是一种HRV测量指标，其入组时的超声心动图左心室EF低于SDANN正常的患者（0.53[0.49,0.62]对0.62[0.56,0.66]，P = 0.03），且缩短分数低于正常患者（0.28[0.25,0.32]对0.36[0.30,0.39]，P = 0.003）。未发现RNA和HRV测量值与随访左心室功能之间存在显著关联。
右心室功能障碍和异常HRV可能是在常规评估中无心室功能障碍证据的地中海贫血患者心脏疾病的早期特征。
