Interrupted aortic arch overview

Associate Editor-In-Chief:, Keri Shafer, M.D. [mailto:kshafer@bidmc.harvard.edu], Priyamvada Singh, MBBS mailto:psingh@perfuse.org

Assistant Editor-In-Chief: Kristin Feeney, B.S. mailto:kfeeney@perfuse.org

Overview
Interrupted aortic arch is a very rare heart defect in which the aorta is not completely developed. There is a gap between the ascending and descending thoracic aorta. Almost all patients also have other cardiac anomalies. It can be diagnosed with an echocardiogram. Treatment consists of open heart surgery soon after birth. Awaiting surgery, prostaglandin can be administered to keep the ductus arteriosus open, thereby allowing blood flow to the lower body.


 * Interrupted aortic arch (IAA) is a separation between the ascending and descending aortas. There are 3 types of this abnormality:
 * Type A: Interruption is distal to the subclavian artery
 * Type B: Interruption occurs between the second carotid and the ipsilateral subclavian arteries
 * Type C: Interruption occurs between two carotid arteries.


 * Each type is divided into 3 subtypes:
 * Subtype 1: Normal subclavian artery
 * Subtype 2: Aberrant subclavian artery
 * Subtype 3: Isolated subclavian artery that arises from the ductus arteriosus.


 * In addition to the type of IAA, the following information is important for surgical planning
 * Evaluation of the distance between the proximal and distal segments,
 * Size of a patent ductus arteriosus
 * Narrowest dimension of the left ventricular outflow tract
 * Other cardiac structural abnormalities are important for surgical planning.


 * A right-sided descending aorta with aortic interruption is almost always associated with DiGeorge syndrome.