Survival is unlikely without surgical intervention. Mortality is variable and has been reported to be relatively high in previous studies. The major contributor to postoperative mortality is the high frequency of bronchial and tracheal abnormalities in this group of patients. Early, aggressive intervention may minimize the mortality caused by these associated lesions. Morbidity includes frequent episodes of stridor and respiratory distress with or without wheezing and cyanosis.
Recurrent pneumonia can occur.
No known racial predilection exists. Dysphagia is rare, but obstructive apnea may occur. These patients tend to be more symptomatic and to present even earlier than those with a vascular ring due to a double aortic arch. History is predictable and usually not subtle. When tracheal obstruction exists, the infant may have constant retractions and tachypnea.