Retroesophageal Right Aortic Arch Associated with an Aortic Aneurysm and a Ventricular Septal Defect

Abstract

This chapter presents the case of a patient admitted due to complaints of shortness of breath and chest pain upon exertion radiating to the back and episodes of tachycardia, with a perimembranous VSD on transthoracic echocardiography and a retroesophageal right aortic arch with anomalous left subclavian artery and a compressive saccular aneurysm on CT scan. Surgical correction was made with an interposition of a Dacron graft creating an aortic bypass from the ascending aorta to the retroesophageal portion of the aorta and a ventricular septoplasty. After surgery, the patient was admitted to the ICU henodinamically stable under 7 mcg/kg/min of dobutamine and 0.4 mcg/kg/min of nitroprusside, which were totally weaned after 24 h. After 29 days, the patient was discharged and followed up until complete remission of chest pain and improvement of respiratory symptoms were noted. This case brings the discussion about vascular rings and how to manage them. From their embryonic formation to associated anatomical anomalies, we dissect their anatomical features and their clinical presentation, which may depend upon their type, severity, and associated anomalies. Followed by its propedeutics, we present how clinical investigation should proceed, when to best indicate an imaging study depending upon clinical presentation, and when to surgically intervene.