Yefan Jiang

and 3 more

Background and Aim: Interrupted aortic arch (IAA) is defined as a complete interruption of aortic lumen between the ascending and descending aorta. It is an uncommon and complicated congenital heart disease with high mortality in infants. It is rare for patients with isolated IAA to survive to adulthood without operation unless the extensive collateral vessels joining the descending aorta. Here we present three unique cases with isolated IAA together with a review of the literature. Methods: case presentation: We retrospectively searched the hospital patient databases for patients (>14 years) with IAA diagnosed at the Wuhan Union Hospital over the past 10 years and excluded the patients with other cardiac malformations. Three patients were identified. Two were referred to us for hypertension management and were diagnosed with IAA at our hospital. They both declined surgical treatments and underwent conservative therapy including management of their hypertension. One patient was referred to our hospital for further treatment options after the patient was diagnosed with IAA at another hospital. This patient received an extra-anatomic bypass surgery from ascending aorta to descending aorta. His high blood pressure did not resolve and was subsequently managed by anti-hypertensives medications after the surgery. Discussion and conclusions: Adult patients with isolated IAA usually have extensive collateral vessels joining the descending aorta. Surgical intervention may not be necessary for these patients if the patients have no symptoms except hypertension. Anti-hypertensives medical management with long term follow-up appears to be a reasonable treatment option for these patients.