■血管环占先天性心血管异常的1%。表型表达从无症状到与食管或气管压迫有关的严重形式不等。虽然产前筛查的完善导致胎儿诊断的增加,目前,无症状新生儿和婴儿的最佳管理仍存在争议。我们报告了我们中心三十年来在血管环管理方面的经验。
■在这项单中心回顾性研究中,数据从患者病历中提取.为了在随访中获得症状/药物的信息,我们回顾了儿科诊所的临床记录.对于在其他机构随访的患者,我们向转诊儿科医生发送了一份基于网络的调查问卷.
■在82名患者中,69例出现症状(84%)。常见症状包括反复呼吸道感染(43%),stridor(32%),胃食管反流(33%),和吞咽困难(26%)。诊断依赖于心脏超声,钡吞咽研究,和胸部计算机断层扫描。79例(96%)患者进行了手术修复(开胸手术91%)。修复时的中位年龄为13[四分位数间距(IQR),4.4-48]个月。没有死亡。14例(18%)患者发生轻微并发症。住院时间中位数为7(IQR,6-9)天。总的来说,24%的患者仍有症状(中位随访54个月)。其中一半与哮喘有关,与环相关的症状几乎90%的自由。
■大多数患者在诊断时都有症状。血管环如肺吊索或气管压迫综合征需要及时处理。尽管做了手术,24%的患者在随访时没有症状。最后,无症状患者的手术发病率较低,支持目前建议的早期手术修复.
■III.
UNASSIGNED: Vascular rings represent 1% of congenital cardiovascular abnormalities. Phenotypic expression varies from asymptomatic to severe forms related to either oesophageal or tracheal compression. While refinement in prenatal screening led to an increase in fetal diagnosis, optimal management in asymptomatic neonates and infants is currently a matter of debate. We report our center experience of vascular ring management over three decades.
UNASSIGNED: In this single-center retrospective study, data were extracted from patient medical records. To obtain information on symptoms/medication at follow-up, clinical records from pediatric clinics were reviewed. For patients followed in other institutions, a web-based questionnaire was sent to referring pediatricians.
UNASSIGNED: Out of 82 patients, 69 were symptomatic (84%). Common symptoms included recurrent respiratory tract infections (43%), stridor (32%), gastro-esophageal reflux (33%), and dysphagia (26%). Diagnosis relied on cardiac ultrasound, barium swallow studies, and chest computerized tomography scan. Surgical repair (thoracotomy 91%) was performed in 79 patients (96%). Median age at repair was 13 [interquartile range (IQR), 4.4-48] months. There was no mortality. Minor complications occurred in 14 patients (18%). Median hospital length of stay was 7 (IQR, 6-9) days. In total, 24% of patients remained symptomatic (median follow-up 54 months). Half of those were asthma-related, with nearly 90% freedom from ring-related symptoms.
UNASSIGNED: Most patients were symptomatic at the time of diagnosis. Vascular rings such as pulmonary slings or tracheal compression syndromes require prompt management. Despite surgery, 24% of patients were not symptom-free at follow-up. Finally, surgery in asymptomatic patients resulted in low morbidity supporting the current recommendation of early surgical repair.
UNASSIGNED: III.