Vascular Ring; Spectrum of Pathologies and Scenarios for Diagnosis and Management

Mostafa Elhelali,Walid Hassan,Mohamed-Adel Elgamal,Hysam Abdelmohty,Ahmed Mostafa

doi:10.11648/j.ijcts.20200606.12

Mostafa Elhelali, Walid Hassan + Show 3 more

Open Access

https://doi.org/10.11648/j.ijcts.20200606.12

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Abstract

Background: Vascular rings represent approximately 1% of all congenital cardiac anomalies, with Edward’s classification being the first to outline them into a complete or partial vascular ring. A Complete ring is a combination of patent vessels, atretic vascular segments or ligamentous structures. Methods: We reviewed patients` records from our Pediatric Cardiac Surgery Unit, over 10 years. We encountered 63 patients from January 2009 to January 2019. Our patients were 57 (90.5%) complete vascular rings, 5 (7.9%) pulmonary artery slings, and 1 (1.6%) patient with innominate artery compression. Results: The age of our Patients ranged from 2 to 57 months (mean 16.25±1.3 m), weight ranges from 3k to 26k (mean 9.87±0.8kg). Males were 38 (60.3%) and male: female ratio was 1.5:1. In our series various types of vascular rings were encountered; Double Aortic Arch was the right dominant arch variant in 25 (53.2%) and left arch dominant in 7 (14.9%) whilethe co-dominant variant was 15 (31.9%). Fifty-seven cases of vascular rings operated through left posterolateral thoracotomy. Fivecases of pulmonary artery sling (7.9%) operated by median sternotomy and one case (1.6%) of innominate artery compression. No reported intraoperative mortality in our study. Conclusion: Vascular ring is a rare anomaly that needs suspicion for diagnosis. The operative strategy relies on extensive dissection and division of the non-dominant arch and division of the ligamentum, freeing all fibrous bands between the arch and esophagus and trachea. For augmented results; we need collaboration from pediatricians and pulmonologists who must be aware of such anomalies.

Highlights

Vascular rings represent approximately 1% of all congenital cardiac anomalies, with Edward’s classification being the first to outline them into a complete or partial vascular ring
Symptoms presented in our series were mostly due to tracheal compression in form of stridor, bronchial asthma, feeding problem, and failure to thrive shown in table 2
Any anomaly associated with the development of the 6 pairs of aortic arches can lead to a vascular ring

Summary

Introduction

Vascular rings represent approximately 1% of all congenital cardiac anomalies, with Edward’s classification being the first to outline them into a complete or partial vascular ring. Under the term vascular rings; a constellation of anomalies related to defective development of the branchial arches They present as an abnormal position, doubling, interrupted anomalous origin of the aortic arch or its branches [1]. They encircle the aero digestive tracks within the mediastinum; presentation is wheezy chest, recurrent pneumonia, stridor, troubled feeding or choking, and failure to thrive. This quite uncommon subcategory of cardiac anomalies accounts for 1% or less with equal incidence in both sexes. They may present alone or are associated with other major anomalies of the heart like Ventricular septal defect, Fallot’s tetralogy, or trans-positioning of great vessels [1]

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