Alhuzaimi Abdullah N, Alotaibi Najoud M, Alsuhaibani Ghadah I, Alanazi Reem K, Temsah Mohamad-Hani
Pediatric Cardiology, Department of Cardiac Science, College of Medicine, King Saud University Medical City, King Saud University, Riyadh, SAU.
Pediatric Intensive Care Unit, Department of Pediatrics, College of Medicine, King Saud University Medical City, King Saud University, Riyadh, SAU.
Cureus. 2021 Feb 7;13(2):e13212. doi: 10.7759/cureus.13212.
To describe the frequency and spectrum of congenital heart defects (CHD) and pulmonary hypertension among pediatric patients with Down syndrome (DS) in Saudi Arabia.
A cross-sectional, retrospective study of the cardiac anomalies among pediatric patients (0-18 years) with DS had been seen and evaluated in one center from August 2001 to October 2020. The demographic data, the reason for referral, echocardiography data including presence and type of CHD, systolic function, atrioventricular regurgitation, and pulmonary hypertension (PHTN) were analyzed.
Among the 468 pediatric patients with DS, 275 (58.8%) had one or more congenital heart defects (CHD). The most common types of CHD among DS pediatric patients were ventricular septal defect (29.45%), atrial septal defect (ASD) secundum (26.9%) and atrioventricular septal defect (AVSD) (22.9%), and moderate to large patent ductus arteriosus (PDA) (9.1%). Pulmonary hypertension analyzed in children older than two months of age and was present in 21.5% of patients with CHD and 2.2% of patients with no CHD. Multivariate logistic regression showed the presence of AVSD, large PDA, and ASD secundum which all independent predictors of pulmonary hypertension.
Almost 60% of DS patients have CHD with pulmonary hypertension which affect almost one-fifth of patients with CHD. AVSD, hemodynamically significant PDA, and ASD secundum were the most common lesions associated with pulmonary hypertension.
描述沙特阿拉伯唐氏综合征（DS）患儿先天性心脏病（CHD）和肺动脉高压的发生率及谱系。
对2001年8月至2020年10月在一个中心就诊并接受评估的0至18岁DS患儿的心脏异常进行横断面回顾性研究。分析人口统计学数据、转诊原因、超声心动图数据，包括CHD的存在情况和类型、收缩功能、房室反流以及肺动脉高压（PHTN）。
在468例DS患儿中，275例（58.8%）患有一种或多种先天性心脏病（CHD）。DS患儿中最常见的CHD类型为室间隔缺损（29.45%）、继发孔房间隔缺损（ASD）（26.9%）和房室间隔缺损（AVSD）（22.9%），以及中度至大型动脉导管未闭（PDA）（9.1%）。对年龄超过两个月的儿童进行肺动脉高压分析，结果显示CHD患者中有21.5%存在肺动脉高压，无CHD患者中有2.2%存在肺动脉高压。多因素逻辑回归显示，AVSD、大型PDA和继发孔ASD均为肺动脉高压的独立预测因素。
近60%的DS患者患有CHD并伴有肺动脉高压，这影响了近五分之一的CHD患者。AVSD、血流动力学显著的PDA和继发孔ASD是与肺动脉高压相关的最常见病变。
