Mancuso Luigi, Vitrano Angela, Mancuso Andrea, Sacco Massimiliano, Ledda Antonietta, Maggio Aurelio
a Franco and Piera Cutino Campus di Ematologia , Ospedali Riuniti Villa Sofia-V. Cervello , Palermo Italia.
b Medicina Interna, Azienda di Rilievo Nazionale ad Alta Specializzazione (ARNAS) Civico , Palermo , Italia.
Hemoglobin. 2018 Jan;42(1):68-71. doi: 10.1080/03630269.2018.1451341.
We studied the clinical, electrocardiographic, echocardiographic, Doppler and T2* cardiac magnetic resonance (CMR) data of all adult β-thalassemia major (β-TM) patients with heart failure (HF) consecutively observed at our referral center of the Sicilian region between 2008 and 2016. There were 16 patients enrolled in the study. Echocardiographic examination showed that only one patient had HF with systolic dysfunction of the left ventricle (HFrEF), whereas the others had HF with preserved systolic function of the left ventricle (HFpEF). Systolic dysfunction of the right ventricle (RV) was observed in 13 cases. Furthermore, 30.0% of the patients presented T2* CMR values consistent with intermediate risk of systolic dysfunction of the left ventricle (LV) due to iron overload, whereas 70.0% had normal values. Typical electrocardiographic abnormalities (wide T wave inversion and low voltages) were observed in 11 out of 16 patients. In conclusion, in the adult β-TM patients with HF recently observed at our center, the predominant form was that with diastolic dysfunction of the LV, and with systolic dysfunction of the RV. Only 30.0% had low values of T2* CMR. Typical electrocardiographic abnormalities were found in 69.0%.
我们研究了2008年至2016年间在西西里地区我们的转诊中心连续观察到的所有成年重度β地中海贫血（β-TM）合并心力衰竭（HF）患者的临床、心电图、超声心动图、多普勒及T2心脏磁共振（CMR）数据。共有16例患者纳入研究。超声心动图检查显示，只有1例患者为左心室收缩功能障碍的心力衰竭（HFrEF），而其他患者为左心室收缩功能保留的心力衰竭（HFpEF）。13例患者观察到右心室（RV）收缩功能障碍。此外，30.0%的患者T2 CMR值与因铁过载导致左心室（LV）收缩功能障碍的中度风险一致，而70.0%的患者值正常。16例患者中有11例观察到典型的心电图异常（T波深倒置和低电压）。总之，在我们中心近期观察到的成年β-TM合并HF患者中，主要形式是左心室舒张功能障碍和右心室收缩功能障碍。只有30.0%的患者T2* CMR值较低。69.0%的患者发现典型的心电图异常。
