Congenital Vascular Rings : An Exploratory Study in Adult Patients

Lodeweges, J. (2015) Congenital Vascular Rings : An Exploratory Study in Adult Patients. thesis, Medicine.

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Abstract

Introduction. Vascular rings are rare congenital vascular anomalies, surrounding the trachea and/or esophagus, causing variable degrees of compression and giving rise to a wide range of symptoms that vary in severity and age at presentation. Due to the aspecific complaints and the presence of associated cardiac anomalies, the diagnosis is challenging. The aim of this study was to describe the different types of vascular rings according to their anatomical heterogeneity, presenting symptoms, used diagnostic strategies and (long-term) surgical outcome, in order to raise clinical awareness of this complex congenital anomaly and its diagnostic and treatment strategies. Methods. A retrospective observational database study was conducted, reviewing the electronic and paper medical charts of patients from seven academic centers, identified from the CONCOR database. The type of vascular ring was classified according to the categories by Backer and Mavroudis. Descriptive statistics were applied. Results. 69 patients were included with a median age at presentation of 8.5 years (range 0 – 53 years). 21 patients (30.4%) had a double aortic arch, 16 patients (23.2%) a right aortic arch with aberrant left subclavian artery, 30 patients (43.5%) a left aortic arch with aberrant right subclavian artery, 1 patient (1.4%) an innominate artery compression and 1 patient (1.4%) a right cervical aortic arch. Associated cardiac anomalies were found in 63.8% of patients. Main symptomatology were respiratory symptoms (82.9%), with dyspnea being most frequent (86.2%). Dysphagia was the most common gastro-intestinal symptom (69.2%). Chest radiography was the most frequent obtained diagnostic strategy (85.5%). Barium esophagography, chest CT- and MRI scan were diagnostic in most cases (90%, 100% and 88.9%, respectively). Spirometry showed a variable intrathoracic obstruction, suggestive for a vascular ring. Surgery was performed in 42% of patients with a median age of 17 years. A left thoracotomy was the most performed type of surgery (55.2%). The median length of followup after surgery was 5 years. 93% of patients showed (some) improvement or complete relief of symptoms shortly after surgery. Conclusion. With this retrospective observational database study, recognition of several useful clinical points concerning vascular rings in the CONCOR database population was obtained, increasing clinical awareness of this condition and its diagnostic and treatment strategies.

Item Type:	Thesis (Thesis)
Supervisor name:	Faculty Supervisor: and Dikkers, F.G. MD and Daily Supervisor: and Melle, J.P. van MD and University Medical Centre Groningen, department of Cardiolog
Faculty:	Medical Sciences
Date Deposited:	25 Jun 2020 10:54
Last Modified:	25 Jun 2020 10:54
URI:	https://umcg.studenttheses.ub.rug.nl/id/eprint/1526

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