Abdelrahman Eman Gamal, Ezzat Ahmed Mahmoud, Mohammed Lina Abdelhady, Fakher Reem Zaki Mohamed, Elbahy Samar Mahmoud
Pediatric Department, Faculty of Medicine, Benha University, Benha, Egypt.
Biochemistry Department, Faculty of Medicine, Benha University, Benha, Egypt.
Eur J Pediatr. 2025 Jun 16;184(7):420. doi: 10.1007/s00431-025-06250-1.
Vitamin D deficiency is a recognized complication of β-thalassemia major (β-TM), impacting cardiac function. Speckle tracking echocardiography (STE) offers a sensitive assessment of myocardial deformation, particularly global longitudinal strain (GLS). This study aimed to assess the association between vitamin D levels and LV function in β-TM children and to evaluate the impact of vitamin D supplementation on cardiac parameters. Seventy-five β-TM children underwent vitamin D level assessment, conventional echocardiography, and STE. Patients with vitamin D deficiency/insufficiency (25-OHD3 < 30 ng/ml) received vitamin D supplementation (4000-5000 IU/day). Follow-up assessments were conducted after vitamin D normalization. Vitamin D insufficiency (81.3%) and deficiency (18.7%) were prevalent. Vitamin D levels were inversely correlated with age and disease duration (p < 0.001) and positively correlated with transfusion/chelation therapy onset. Conventional echocardiography showed an inverse correlation between vitamin D level and left ventricular end diastolic diameter (p < 0.001) and deceleration time (p = 0.003). STE revealed a positive correlation between vitamin D and GLS (p < 0.001). Vitamin D supplementation significantly increased median vitamin D levels (from 16.0 to 39.0 ng/ml, p < 0.001) and improved STE parameters, including AP4L, AP3L, AP2L, and GLS (p < 0.001), indicating enhanced myocardial function.
Vitamin D deficiency in β-TM children was correlated with impaired cardiac function. Vitamin D supplementation significantly improved cardiac function. Regular monitoring and maintenance of adequate vitamin D levels are crucial for preventing adverse cardiac effects.
• β-Thalassemia major is frequently complicated by cardiac dysfunction, a major contributor to mortality. • Cardiac iron overload is a primary driver of cardiac dysfunction in β-TM.
• Vitamin D deficiency leads to impaired cardiac function, beyond iron overload, in β-TM children. • Vitamin D supplementation could improve cardiac function in β-thalassemia major patients.
维生素D缺乏是重型β地中海贫血（β-TM）公认的并发症，会影响心脏功能。斑点追踪超声心动图（STE）能对心肌变形进行敏感评估，尤其是整体纵向应变（GLS）。本研究旨在评估β-TM患儿维生素D水平与左心室功能之间的关联，并评估补充维生素D对心脏参数的影响。75例β-TM患儿接受了维生素D水平评估、传统超声心动图检查和STE检查。维生素D缺乏/不足（25-OHD3<30 ng/ml）的患者接受了维生素D补充治疗（4000-5000 IU/天）。在维生素D水平恢复正常后进行了随访评估。维生素D不足（81.3%）和缺乏（18.7%）很常见。维生素D水平与年龄和病程呈负相关（p<0.001），与输血/螯合治疗开始时间呈正相关。传统超声心动图显示维生素D水平与左心室舒张末期内径呈负相关（p<0.001），与减速时间呈负相关（p=0.003）。STE显示维生素D与GLS呈正相关（p<0.001）。补充维生素D显著提高了维生素D水平中位数（从16.0 ng/ml提高到39.0 ng/ml，p<0.001），并改善了STE参数，包括AP4L、AP3L、AP2L和GLS（p<0.001），表明心肌功能增强。
β-TM患儿维生素D缺乏与心脏功能受损相关。补充维生素D可显著改善心脏功能。定期监测和维持充足的维生素D水平对于预防不良心脏影响至关重要。
•重型β地中海贫血常并发心脏功能障碍，是死亡的主要原因。•心脏铁过载是β-TM心脏功能障碍的主要驱动因素。
•维生素D缺乏除了导致铁过载外，还会导致β-TM患儿心脏功能受损。•补充维生素D可改善重型β地中海贫血患者的心脏功能。
