Introduction: A right aortic arch (RAA) with aberrant left subclavian artery (ALSA) is an uncommon vascular congenital anomaly that is associated with Down syndrome(DS). The anatomy of this vascular anomaly consists of the subclavian artery, which arises from the aorta and travels behind the esophagus. We describe a case of hemorrhagic shock from subclavian-esophageal fistula in the setting of prolonged tracheostomy tube and repeated endoscopy. Description: A 36-year-old gentleman with a history of DS and peptic ulcer disease was initially admitted for acute respiratory distress syndrome (ARDS) after elective ventral hernia repair. The patient’s respiratory status had eventually improved, but required the need for tracheostomy and percutaneous gastric tube placement. During the evaluation of ARDS, the patient was found to have a RAA with ALSA. The hospital course was further complicated by multiple bronchoscopic evaluations, laryngoscopic evaluations, and endoscopic evaluations. During routine evaluation one morning, the patient was found to be in hemorrhagic shock with hematemesis. Hemoglobin decreased from 9.6 overnight to 5.4, with blood pressure dropping to 70s/40s. Given the rate of bleeding, massive transfusion protocol and emergent CTA neck and chest/abdomen were ordered. CTA of the chest demonstrated active contrast extravasation within the proximal esophagus consistent with active hemorrhage originating from a fistulous communication between the aberrant left subclavian artery and esophagus. The patient was then emergently taken to the OR with vascular surgery for left subclavian artery stent placement. The congenital heart team was consulted and noted that no surgical intervention was required, with close outpatient follow-up. Afterward, the patient’s blood pressure and hemoglobin levels significantly improved following multiple blood transfusions and subclavian-esophageal stent placement. Discussion: A RAA with an ALSA is a rare vascular congenital anomaly that is associated with DS. Subclavian-esophageal fistulae are also rare occurrences that can occur due to prolonged use of medical devices. Here we present a case of subclavian-esophageal fistula in a patient with an ALSA, which likely stems from prolonged tracheostomy tube and multiple endoscopic procedures.
Patel et al. (Sun,) studied this question.