Is surgery necessary for adults with isolated interrupted aortic arch?: Case series with literature review

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. It is rare for patients with isolated IAA to survive to adulthood without operation. Here we present three rare cases with isolated IAA together with a review of the literature of IAA. Besides, we reviewed reported adult cases with isolated IAA in the last 20 years and summarized the relevant data of the isolated type.

Methods: We retrospectively searched the hospital databases for adult patients with isolated IAA diagnosed at the Wuhan Union Hospital over the past 10 years. Cases related to adult isolated IAA published in last 20 years were identified by searching Pubmed.

Results: Three adult patients with isolated IAA were identified. Two were referred to us for hypertension management and were diagnosed with IAA. They declined surgical treatments and took antihypertensive medications. One patient was referred to our hospital for further treatment options after diagnosed with IAA at another hospital. He received an extra-anatomic bypass surgery. But his hypertension did not well resolve after surgery, and was subsequently managed by anti-hypertensives medications. 25 published adult patients with isolated IAA were identified in Pubmed and relevant details were summarized.

Conclusions: Adult patients with isolated IAA usually have extensive collateral vessels joining the descending aorta. Anti-hypertensives medical management with long-term follow-up appears to be a reasonable treatment option for these patients, although surgical intervention is a good choice.