Mohammadi Hanieh, Mohammadpour Ahranjani Behzad, Aghaei Moghadam Ehsan, Kompani Farzad, Mirbeyk Mona, Rezaei Nima
Resident Research Committee, Children's Medical Center, Pediatrics Center of Excellence, Tehran University of Medical Sciences, Tehran, Iran.
Department of Pediatric Cardiology, Bahrami Children's Hospital, Tehran University of Medical Sciences, Tehran, Iran.
Egypt J Med Hum Genet. 2022;23(1):47. doi: 10.1186/s43042-022-00262-4. Epub 2022 Mar 8.
Congenital heart disease CHD is a significant cause of mortality and morbidity in children worldwide. Patients with congenital heart disease may develop hematological problems, including thrombocytopenia and neutropenia. In addition, several studies indicate the higher frailty of patients with CHDs to infections and malignancies. Nevertheless, the mechanisms of immune system changes in these patients have remained in the shadow of uncertainty. Moreover, very few studies have worked on cytopenia in CHD. This study has assessed the frequency of thrombocytopenia, neutropenia, lymphopenia, and anemia in pediatric patients with acyanotic congenital heart disease ACHD prior to open-heart surgery.
This cross-sectional study was handled in the Pediatric Cardiology Clinic, Tehran University of Medical Sciences, during pre-operation visits from 2014 till 2019. Two hundred forty-eight children and adolescents with acyanotic congenital heart disease before open-heart surgery met the criteria to enter the study.
A total of 191 (76.7%) patients with Ventricular Septal Defects (VSD), 37 (14.85%) patients with Atrial Septal Defects (ASD), and 20 (8.11%) patients with Patent Ductus Arteriosus (PDA) were enrolled in this study. The median age was 23.87 months. Thrombocytopenia and neutropenia were found, respectively, in 3 (1.2) and 23 (9.2%) patients. Hemoglobin level and lymphocyte count were significantly lower in patients with neutropenia than patients with normal neutrophil count ( value = 0.024 and value = 0.000). Significant positive correlations were found between neutropenia and anemia. There were no correlations between neutrophil count and Platelets. Also, anemia was found in 48 patients (19.3%). The study also found a statistically significant correlation between the co-existence of VSD and neutropenia in the patients ( value = 0.000).
Although most were mildly neutropenic, there was a significant correlation between neutropenia and Ventricular Septal Defect compared to PDA and ASD groups. Regarding the importance of neutropenia to affect the prognosis of congenital heart defects in infections, it is important to consider further studies on the status of immune system function in these patients.
先天性心脏病（CHD）是全球儿童死亡和发病的重要原因。先天性心脏病患者可能会出现血液学问题，包括血小板减少和中性粒细胞减少。此外，多项研究表明先天性心脏病患者对感染和恶性肿瘤的脆弱性更高。然而，这些患者免疫系统变化的机制仍不明确。此外，很少有研究关注先天性心脏病中的血细胞减少症。本研究评估了非青紫型先天性心脏病（ACHD）小儿患者在心脏直视手术前血小板减少、中性粒细胞减少、淋巴细胞减少和贫血的发生率。
本横断面研究于2014年至2019年在德黑兰医科大学儿科心脏病诊所进行术前访视时开展。248例心脏直视手术前的非青紫型先天性心脏病儿童和青少年符合纳入研究的标准。
本研究共纳入191例（76.7%）室间隔缺损（VSD）患者、37例（14.85%）房间隔缺损（ASD）患者和20例（8.11%）动脉导管未闭（PDA）患者。中位年龄为23.87个月。分别有3例（1.2%）和23例（9.2%）患者出现血小板减少和中性粒细胞减少。中性粒细胞减少患者的血红蛋白水平和淋巴细胞计数显著低于中性粒细胞计数正常的患者（P值分别为0.024和0.000）。中性粒细胞减少与贫血之间存在显著正相关。中性粒细胞计数与血小板之间无相关性。此外，48例患者（19.3%）出现贫血。研究还发现患者中室间隔缺损与中性粒细胞减少并存之间存在统计学显著相关性（P值 = 0.000）。
尽管大多数患者为轻度中性粒细胞减少，但与动脉导管未闭和房间隔缺损组相比，中性粒细胞减少与室间隔缺损之间存在显著相关性。鉴于中性粒细胞减少对先天性心脏缺陷感染预后的重要性，进一步研究这些患者的免疫系统功能状态很重要。
