Double Aortic Arch a Rare and Uncommon Vascular Ring Malformation: A Case Report

Introduction Double aortic arch is the most frequent type of vascular ring malformation. The condition involves surrounded and compressed trachea and/or esophagus. Depending on the tracheal or esophageal compression, patients may experience symptoms like respiratory failure, cyanosis, asthma-like symptoms, dysphagia and other [1]. Case Presentation A 31 year-old female patient sought medical attention due to difficulty breathing during physical exercise. Over the past year she has experienced increased general weakness and reported several episodes of syncope, which she attributed to general weakness and previous pregnancy. A chest CT scan was performed for further evaluation, which revealed significant localized stenosis in the lower third of the trachea due to external compression. A CTA was performed, which revealed a dominant right aortic arch. This vascular anomaly formed a ring causing significant tracheal stenosis. Multidisciplinary Heart Team meeting concluded that surgical intervention was indicated. In the early postoperative period, the patient was presented with a markedly hoarse and weak voice. After otorhinolaryngological evaluation the partial bilateral vocal fold paralysis and laryngeal dysfunction were identified. These findings indicated the need for immediate treatment. After hospitalization period of 15 days no additional complications were observed, the patient was deemed stable and discharged from the hospital. Discussion The exact incidence of double aortic arch is generally unknown, though vascular rings are estimated to have a prevalence of about 1%. Up to 12.6% of cases present with an associated cardiac condition, such as ventricular septal defect, tetralogy of Fallot or other congenital heart defects [2]. Both endovascular and open-heart surgery is available depending on additional defects. The primary aim of the traditional open-heart surgery is the division of the vascular ring by dividing and ligating the non-dominant aortic arch [3,4,5]. Conclusions Double aortic arch is an uncommon congenital vascular malformation, especially in adulthood. It is unique for being frequently misdiagnosed in later years of life. Our case depicts the quintessence of accurate differential diagnosis, proper diagnostic methods, and timely surgical treatment to avoid possible complications.