Pistoia Laura, Meloni Antonella, Positano Vincenzo, Quota Alessandra, Corigliano Elisabetta, Messina Giuseppe, Renne Stefania, Zerbini Michela, Romani Simona, Sinagra Gianfranco, Ait Ali Lamia, Mavrogeni Sophie, Lupi Amalia, Cademartiri Filippo, Pepe Alessia
U.O.C. Ricerca Clinica, Fondazione G. Monasterio CNR-Regione Toscana, 56124 Pisa, Italy.
Department of Radiology, Fondazione G. Monasterio CNR-Regione Toscana, 56124 Pisa, Italy.
Diagnostics (Basel). 2024 Dec 14;14(24):2816. doi: 10.3390/diagnostics14242816.
BACKGROUND/OBJECTIVES: We aimed to establish biatrial and biventricular reference ranges using cardiac magnetic resonance (CMR) parameters in SCD patients without heart damage.
This study compared CMR parameters, quantified by cine SSFP sequences, in 48 adult SCD patients without apparent cardiac involvement (defined by the absence of known risk factors, normal electrocardiogram, and no macroscopic myocardial fibrosis or significant cardiac iron on T2* CMR) to matched cohorts of 96 healthy controls and 96 thalassemia major (TM) patients without cardiac damage. Nine paediatric SCD patients were also analysed and compared to age- and gender-matched groups of nine TM patients and nine healthy subjects.
In all groups, studied males displayed higher biventricular volumes and mass indexes than females. Male SCD patients showed significantly higher left ventricular (LV) end-diastolic volume index (EDVI), LV end-systolic volume index (ESVI), LV stroke volume index (SVI), cardiac index, LV and right ventricular (RV) mass index, and atrial areas than healthy subjects. Females with SCD exhibited increased LV EDVI, LV SVI, RV mass index, and left atrial area index compared to healthy controls. SCD and TM patients showed comparable biatrial areas and biventricular volumes and function. When compared to TM, SCD males exhibited a larger mass index, while SCD females showed an increased RV mass index. CMR parameters were similar across all paediatric groups.
By establishing the biatrial and biventricular reference ranges through CMR for adult male and female SCD patients, we aimed to prevent possible misdiagnosis of cardiomyopathy in this population by taking into account cardiac adaptation due to anaemia.
背景/目的：我们旨在利用心脏磁共振成像（CMR）参数，为无心脏损伤的镰状细胞病（SCD）患者建立双心房和双心室的参考范围。
本研究比较了48例无明显心脏受累的成年SCD患者（定义为无已知危险因素、心电图正常、T2* CMR无宏观心肌纤维化或显著心脏铁沉积）通过电影稳态自由进动序列量化的CMR参数，与96例健康对照者和96例无心脏损伤的重型地中海贫血（TM）患者的匹配队列。还分析了9例儿科SCD患者，并与9例TM患者和9例健康受试者的年龄和性别匹配组进行比较。
在所有组中，研究显示男性的双心室容积和质量指数高于女性。男性SCD患者的左心室舒张末期容积指数（EDVI）、左心室收缩末期容积指数（ESVI）、左心室每搏输出量指数（SVI）、心脏指数、左心室和右心室（RV）质量指数以及心房面积均显著高于健康受试者。与健康对照相比，患有SCD的女性左心室EDVI、左心室SVI、右心室质量指数和左心房面积指数增加。SCD和TM患者的双心房面积、双心室容积和功能相当。与TM相比，SCD男性的质量指数更大，而SCD女性的右心室质量指数增加。所有儿科组的CMR参数相似。
通过为成年男性和女性SCD患者通过CMR建立双心房和双心室参考范围，我们旨在通过考虑贫血导致的心脏适应性变化，预防该人群中心肌病的可能误诊。
