Department, of Pediatrics (Cardiology), Hassenfeld Children's Hospital at New York University Langone Medical Center, 403 East 34th Street, 4th Floor, Pediatric Cardiology, New York, New York, 10016, USA.
Department of Pediatrics (Cardiology), Maimonides Medical Center, Brooklyn, New York, USA.
Pediatr Cardiol. 2024 Jun;45(5):959-966. doi: 10.1007/s00246-024-03442-8. Epub 2024 Mar 11.
Vascular rings are arterial malformations that lead to the compression of the trachea and/or esophagus. While "tight" rings often produce symptoms and require surgery, "loose" rings rarely produce symptoms. Given advances in fetal echocardiography, this diagnosis is now more often made prenatally. This poses a new conundrum in the management of asymptomatic patients, leading to practice variation and creating a target for clinical system improvement. Hence, we conducted this survey aiming to demonstrate the practice variation existing in current evaluation and management of these patients. An anonymous web-based survey was distributed to several listservs for pediatric cardiologists and pediatric cardiothoracic surgeons. Survey questions targeted respondent practice characteristics, testing obtained, and indications for testing or surgical referral. In total 61 responses were received, predominantly from pediatric cardiologists (95%) in the United States (97%). About 60% of clinicians reported frequently diagnosing patients with vascular rings by fetal echocardiogram, with only about 20% diagnosing them frequently on evaluation of symptoms. Computed tomography angiography and echocardiogram were the most common imaging modalities employed. Most clinicians obtained cross-sectional imaging at the time of diagnosis and referred to surgery once patients had at least occasional symptoms. Respondents demonstrated a low degree of agreement (Krippendorf's alpha 0.48). Few statistically significant patterns were identified between respondents based on their practice characteristics. This study identified significant variation between clinicians regarding the evaluation and management of vascular rings. Further research or expert opinions may help to standardize practice, saving costs and improving the quality of care for affected patients.
血管环是一种动脉畸形，导致气管和/或食管受压。虽然“紧”的血管环通常会产生症状并需要手术治疗，但“松”的血管环很少产生症状。由于胎儿超声心动图的进步，这种诊断现在更常在产前做出。这在无症状患者的管理中提出了一个新的难题，导致实践的差异，并为临床系统的改进创造了一个目标。因此，我们进行了这项调查，旨在展示目前对这些患者进行评估和管理的实践差异。一项匿名的基于网络的调查分发给了几个儿科心脏病学家和儿科心胸外科医生的列表服务器。调查问题针对的是受访者的实践特征、所获得的检查以及进行检查或手术转诊的指征。共收到 61 份回复，主要来自美国（97%）的儿科心脏病学家（95%）。约 60%的临床医生报告经常通过胎儿超声心动图诊断出患有血管环的患者，只有约 20%的临床医生经常根据症状评估来诊断。计算机断层血管造影和超声心动图是最常用的影像学检查方法。大多数临床医生在诊断时会进行横断面成像，并在患者出现至少偶尔症状时将其转介给外科手术。受访者之间的一致性程度较低（Krippendorf's alpha 0.48）。根据他们的实践特征，在受访者之间只确定了一些没有统计学意义的模式。这项研究确定了临床医生在评估和管理血管环方面存在显著差异。进一步的研究或专家意见可能有助于规范实践，节省成本，并提高受影响患者的护理质量。
