Interrupted Aortic Arch in the Adult

Abstract

Interrupted aortic arch (IAA) is a rare condition in infants that occurs approximately three times per million births. It is considered incompatible with life after the ductus arteriosus closes if it is not surgically corrected. A review found 37 reported cases not identified until the patient was over the age of 18, and analysis of these cases and of our own case is reported.

Interrupted Aortic Arch in an Adult with Polycystic Kidney Disease

“…Although a diagnosis of infancy is primarily considered, there have been more than 30 cases reported in the adult population [10]. The IAA found in our patient is not an isolated anomaly as it is accompanied by ADPKD.…”

“…In a review published in 2011, 37 interrupted aortic cases diagnosed at an adult age were reported [10]. Seven of them were reported in Turkey.…”

Response to Letter to the Editor: Interrupted Aortic Arch in the Adult

“…We appreciate the insightful comments of Dr. Casati and Dr. Novelli. We recognize this group's contribution with their report on this problem 1 and apologize for our oversight in not including this in our review 2 . We agree that the absence of a standard definition with objective factors differentiating Type A interruption and severe coarctation in the adult does not exist.…”

Type B Interrupted Left Aortic Arch with Isolated Right Subclavian Artery

“…The vast majority of cases are diagnosed in neonates and infants; however, there are case reports of IAA in adult patients, with type A being the most common (79%). Presumptively, the predominance of the type A variety in cases diagnosed in adulthood stems from the propensity for collateralization in type A IAA; similar to postductal coarctation of the aorta 3 . In fact, it is hypothesized that type A IAA presenting in adults represents the most severe form of (or “complete”) postductal coarctation.…”