心血管系统受累是相当普遍的,也是威廉姆斯综合征(WS)患者发病和死亡的主要原因。大多数人需要手术。本研究旨在对单中心经验给予的WS患者的外科手术特征和结局进行详细评估。此外,还要对Türkiye进行详细的审查。
对1992年至2021年间诊断为WS的35名儿童进行了回顾性评估,包括心血管数据。手术治疗特点,和结果。总共评估了来自Türkiye的六篇文章。
共有35例Williams综合征患者(24例男性),心脏病诊断的中位年龄为6个月(范围,2天-6年)进行了评估。WS患者的心脏缺陷被发现为瓣上主动脉瓣狭窄(SVAS)(n=30,85%)和周围性肺动脉瓣狭窄(PPS)(n=21,65%)。在71%的患者中发现了额外的心脏异常。所有WS患者的SVAS和PPS手术率为77.1%。患者的中位手术年龄为2.5岁(范围,7个月-15.5年)。没有患者因手术死亡。但是一名患者在血管造影开始时由于麻醉而死于室性心动过速。从Türkiye发表的文章中评估了总共138名(63%为男性)WS患者。在138名患者中,64.4%有SVAS,52.1%有PPS,39.8%有额外的心脏异常。中位随访期为17个月至18年,6例(4.3%)患者在术后早期死亡。
心血管系统受累非常普遍,是WS患者发病和死亡的主要原因。通常需要手术干预。正如我们的研究中所看到的,包括35例WS患者和来自Türkiye的出版物,WS患者的SVAS通常需要手术,尤其是在生命的第一年。PPS,另一方面,需要的手术频率低于SVAS,肺动脉狭窄似乎随着时间的推移而减少。
Cardiovascular system involvement is quite common and the leading cause of morbidity and mortality in patients with Williams syndrome (WS), most of whom need surgery. The present study aimed to provide a detailed evaluation of the features of surgical procedures and outcomes of patients with WS given as single-center experience, and additionally to make a detailed
review from Türkiye.
Thirty-five children with WS diagnosed between the years 1992 and 2021 were evaluated retrospectively including cardiovascular data, surgical treatment features, and outcomes. A total of six articles from Türkiye were evaluated.
A total of 35 patients with Williams Syndrome (24 male) with a median age of cardiologic diagnosis of 6 months (range, 2 days-6 years) were evaluated. The cardiac defects of the patients with WS were found as supravalvular aortic stenosis (SVAS) (n=30, 85%) and peripheral pulmonary stenosis (PPS) (n=21, 65%). Additional cardiac anomalies were seen in 71% patients. The rate of SVAS and PPS surgery in all patients with WS was 77.1%. The median surgical age of the patients was 2.5 years (range, 7 months-15.5 years). No patients died due to surgery. But one patient died because of ventricular tachycardia due to anesthesia at the beginning of angiography. A total of 138 (63% male) patients with WS were evaluated from the articles published in Türkiye. Of 138 patients, 64.4% had SVAS, 52.1% had PPS, and 39.8% had additional cardiac anomaly. The median follow-up period ranged from 17 months to 18 years, and six (4.3%) patients died in the early postoperative period.
Cardiovascular system involvement is extremely common and is the leading cause of morbidity and mortality in patients with WS, often requiring surgical intervention. As seen in our study including 35 patients with WS and in publications from Türkiye, SVAS in patients with WS generally requires surgery, especially in the first year of life. PPS, on the other hand, requires surgery less frequently than SVAS, and pulmonary stenosis appears to decrease over time.