法洛四联症中的隐藏肺动脉和肺动脉高压患者在接受肺动脉手术时的肺动脉压力。
Hidden pulmonary arteries in tetralogy of Fallot and pulmonary artery pressure in patients operated with a pulmonary artery.
机构信息
Cardiovascular and Neonatology Research Center, Namazi Hospital, Shiraz University of Medical Sciences, Shiraz, Iran.
Department of Pediatrics, School of Medicine, Shiraz University of Medical Sciences, Shiraz, Iran.
出版信息
BMC Cardiovasc Disord. 2021 Jan 28;21(1):56. doi: 10.1186/s12872-021-01877-y.
INTRODUCTION
The absence of a pulmonary artery is a rare congenital anomaly that occurs isolated or with other congenital cardiac disorders, particularly tetralogy of Fallot (TOF); meanwhile, a hidden pulmonary artery might exist and originate from a closed ductus arteriosus (DA), which can be stented to reach the artery.
MATERIAL AND METHODS
This prospective study describes cardiac catheterization of nine TOF patients diagnosed with the absence of the left pulmonary artery before the operation. The patients were stratified into three groups: group one, whose closed DA was found and connected to the hidden pulmonary artery with a stent; group two, whose hidden pulmonary arteries were found via the pulmonary vein angiography; and group three, for whom we could not find the remnant of the DA, or our attempt to stent the DA to the hidden pulmonary artery was not successful. We also evaluated outcomes of six other surgically-corrected TOF patients who were operated with the absent left pulmonary artery.
RESULTS
The first group included the patients aged 1, 24, and 30 months, whose CT angiography 6-9 months after stenting showed acceptable left pulmonary artery diameter for surgical correction, and the pulmonary vein angiography of the second group showed a hidden left pulmonary artery with a suitable diameter for surgical correction. However, we were unable to find or stent the DA of group three patients, aged 12, 38, 60, and 63 months. Earlier Angiography might have increased the chance of access to the hidden vessel. Apart from these three groups, follow-ups of six other patients previously corrected with only the right pulmonary artery revealed pulmonary artery hypertension in all patients.
CONCLUSION
The concealed pulmonary artery might be found, and stenting of the closed DA to it might be performed to improve the diameter of the diminutive pulmonary artery. This procedure may allow TOF total surgical correction with two pulmonary arteries. Besides, pulmonary vein angiography can reveal the hidden pulmonary artery.
介绍
肺动脉缺失是一种罕见的先天性异常，可单独发生或与其他先天性心脏疾病一起发生，特别是法洛四联症（TOF）；同时，可能存在一条隐藏的肺动脉，它可能起源于闭合的动脉导管（DA），可以通过支架置入到达动脉。
材料和方法
本前瞻性研究描述了 9 例在手术前被诊断为左肺动脉缺失的 TOF 患者的心脏导管插入术。患者分为三组：组 1，发现闭合的 DA 并通过支架与隐藏的肺动脉相连；组 2，通过肺动脉造影发现隐藏的肺动脉；组 3，无法找到 DA 的残余部分，或者我们试图将 DA 支架置入隐藏的肺动脉的尝试未成功。我们还评估了另外 6 例接受手术矫正的 TOF 患者的结果，这些患者在手术中缺失左肺动脉。
结果
第 1 组包括年龄为 1、24 和 30 个月的患者，支架置入后 6-9 个月的 CT 血管造影显示可接受的左肺动脉直径，适合手术矫正，第 2 组的肺动脉造影显示了一条隐藏的左肺动脉，其直径适合手术矫正。然而，我们无法找到或支架置入第 3 组患者（年龄分别为 12、38、60 和 63 个月）的 DA。早期的血管造影可能会增加接触隐藏血管的机会。除了这三组之外，另外 6 例之前仅用右肺动脉矫正的患者的随访显示所有患者均患有肺动脉高压。
结论
可以找到隐藏的肺动脉，并对其闭合的 DA 进行支架置入，以改善细小肺动脉的直径。该方法可能允许 TOF 通过两条肺动脉进行完全手术矫正。此外，肺动脉造影可以显示隐藏的肺动脉。
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