Rashidi Farid, Sate Hossein, Mohammadi Ali, Koohi Ata, Nejati Babak, Naybzadeh Ahad
a Tuberclosis and Lung Disease Research Center , Tabriz University of Medical Sciences , Tabriz , Iran.
b Department of Cardiology, Cardiovascular Research Center , Tabriz University of Medical Sciences , Tabriz , Iran.
Pediatr Hematol Oncol. 2018 Aug-Sep;35(5-6):322-330. doi: 10.1080/08880018.2018.1534914. Epub 2018 Nov 23.
Pulmonary hypertension is a common complication associated with thalassemia syndromes and it may play an important role in the pathogenesis of right ventricle failure. The true prevalence of pulmonary hypertension in patients with thalassemia major remains unclear and has been reported to be between 2 and 79%.
In total, 70 patients with thalassemia major were initially examined. Patients with valvular left heart disease, congenital heart diseases such as atrial septal defect (ASD) and ventricular septal defect (VSD), left heart failure, and chronic embolism were excluded. All patients with thalassemia major underwent echocardiography. Based on tricuspid regurgitation velocity (TRV), the patients were divided into the following three groups: low, medium, and high risk of pulmonary hypertension.
The mean age of the subjects was 24 y; 60.6% of the subjects were males and 39.4% of the subjects were females. Overall, three (4.5%) subjects were considered at a high risk of pulmonary hypertension. The mean hemoglobin level in the patients with a high probability of pulmonary hypertension was 8.2 g/dL and that in the patients with a low or medium probability of pulmonary hypertension was 9.1 g/dL. No significant difference was observed between the groups (p = .059).
This study showed that, based on new echocardiography criteria, the prevalence of pulmonary hypertension secondary to β-thalassemia was 4.5% and there was no correlation between TRV and the number of received blood units or disease duration.
肺动脉高压是地中海贫血综合征常见的并发症，可能在右心室衰竭的发病机制中起重要作用。重型地中海贫血患者肺动脉高压的真实患病率尚不清楚，报道范围在2%至79%之间。
最初共检查了70例重型地中海贫血患者。排除患有左心瓣膜病、先天性心脏病如房间隔缺损（ASD）和室间隔缺损（VSD）、左心衰竭及慢性栓塞的患者。所有重型地中海贫血患者均接受了超声心动图检查。根据三尖瓣反流速度（TRV），将患者分为以下三组：肺动脉高压低、中、高风险组。
受试者的平均年龄为24岁；60.6%为男性，39.4%为女性。总体而言，3例（4.5%）受试者被认为有肺动脉高压高风险。肺动脉高压高概率患者的平均血红蛋白水平为8.2g/dL，肺动脉高压低概率或中概率患者的平均血红蛋白水平为9.1g/dL。两组间未观察到显著差异（p = 0.059）。
本研究表明，根据新的超声心动图标准，β地中海贫血继发肺动脉高压的患病率为4.5%，TRV与输血单位数量或病程之间无相关性。
