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创新心血管铸型技术突出展示了浆果综合征的复杂畸形特征。

Innovative cardiovascular casting technique features the complex malformation of berry syndrome.

机构信息

Hubei Provinical Clinical Research Center for Accurate Fetus Malformation Diagnosis, Department of gynaecology and obstetrics, Xiangyang No. 1 People's Hospital, Hubei University of Medicine, No 15, Jiefang Avenue, Xiangyang, 441000, China.

Depatment of Ultrasound, Hubei University of Medicine, Xiangyang No. 1 People's Hospital, Xiangyang, 441000, China.

出版信息

BMC Pregnancy Childbirth. 2024 Mar 12;24(1):194. doi: 10.1186/s12884-024-06340-2.

Abstract

BACKGROUND

Prenatal diagnosis of Berry syndrome, a rare combination of cardiac anomalies including aortopulmonary window (APW), aortic origin of the right pulmonary artery (RPA), interrupted aortic arch (IAA), hypoplastic aortic arch, or coarctation of the aorta (COA), poses a significant challenge. Due to the rarity of the disease, and the limited case reports available to features the complex malformation of Berry syndrome postpartum, this article introduces an innovative approach to visually showcase this unusual disease. The proposed method provides a comprehensive display of the structural deformities, offering valuable insights for clinical practitioners seeking to comprehend this condition.

CASE PRESENTATION

In this report, we present a case where fetal echocardiography aided in diagnosing Berry syndrome, which was later confirmed through postpartum cardiovascular casting. Our experience highlights the importance of using the three-vessel view to diagnose APW and aortic origin of the right pulmonary artery. Additionally, obtaining true cross-sectional and sagittal views by continuously scanning from the three-vessel-trachea view to the long-axis view of the aortic arch is necessary to image IAA or coarctation of the aortic arch.

CONCLUSIONS

Early and accurate prenatal diagnosis of Berry syndrome is feasible and our cardiovascular cast can perfectly display the microvascular morphology of the fetal heart, which may have great application prospects for postpartum diagnosis and teaching of complex cardiac abnormalities.

摘要

背景

Berry 综合征是一种罕见的心脏畸形综合征,包括主肺动脉窗(APW)、右肺动脉起源于主动脉(RPA)、主动脉缩窄(IAA)、主动脉弓发育不良或主动脉弓缩窄(COA)等。产前诊断 Berry 综合征具有很大的挑战性。由于该病罕见,且现有的产后病例报告有限,无法全面描述 Berry 综合征的复杂畸形,本文提出了一种创新的方法,以可视化的方式展示这种不常见的疾病。所提出的方法提供了结构畸形的全面显示,为临床医生提供了有价值的信息,有助于理解这种情况。

病例介绍

在本报告中,我们介绍了一个通过胎儿超声心动图诊断 Berry 综合征的病例,该病例随后通过产后心血管铸型得到了证实。我们的经验强调了使用三血管切面诊断 APW 和 RPA 的重要性。此外,通过从三血管-气管切面连续扫描到主动脉弓长轴切面,获得真正的横断面和矢状面视图,对于成像 IAA 或 COA 是必要的。

结论

Berry 综合征的早期和准确的产前诊断是可行的,我们的心血管铸型可以完美地显示胎儿心脏的微血管形态,这对于产后诊断和复杂心脏异常的教学可能具有广阔的应用前景。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1d57/10935913/25a957d5d40e/12884_2024_6340_Fig1_HTML.jpg

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