Whichever way they are categorized, it is helpful to have a working understanding of normal and fetal circulation, as well as an understanding of the segmental approach to imaging in congenital heart disease. There is more than one way to present the variety of congenital heart diseases.

short segment severe aortic coarctation.

General differential considerations include: Surgical correction (either single- or multistage) is the definitive treatment. Prostaglandin E 1 may be given to initial management to keep the ductus open. (a) Two-dimensional echocardiography showing slight curvature of the ascending aorta (A) after the origin of the innominate artery (I), related to the persistence of the aortic arch segment between the origin of the left carotid (C) and subclavian (S) arteries. If uncorrected, it carries a very poor prognosis with extrauterine survival being as little as a few days. Interrupted aortic arch (IAA) Type A in a 24-week fetus.

great vessels may show a 'V' configuration on coronal imaging 2.

non-visualization of the portion of interruption.

CTĪllows visualization of the interrupted aortic arch and associated anomalies. These modalities may not allow differentiation of IAA from severe aortic coarctation with a hypoplastic arch 9. The ascending aorta may also appear more vertical than usual.

The right ventricle may appear a lot larger than the left, although this is a non-specific finding. Plain film features are often non-specific 3: aortopulmonary septal defect (aortopulmonary window).almost always associated if there is a right-sided descending aorta.found commonly in those with a type B interruption.The Dicer mutant mice exhibited a type B interrupted aortic arch (arrow). (B) Gross morphology of heart and great vessels from E17.5 embryos. From: Cardiovascular Pathology (Fourth Edition), 2016. subtype 3: isolated subclavian artery that arises from the ductus arteriosus Neural-crest-specific ablation of Dicer led to multiple cardiovascular defects (A) The penetrance of cardiovascular defects displayed in Dicer mutant mice. Interruption of the aortic arch is a condition in which communication between the proximal part of the aorta and the descending aorta is interrupted, usually due to lack of tissue between these portions of the aorta, and a PDA feeds the descending aorta.type C: rare, interruption occurs proximal to the left common carotid arterial originĮach type is divided into three subtypes 7:.type B: most common (>50%), the break occurs between the left common carotid and left subclavian arterial origins.

type A: second most common, the interruption occurs distal to the left subclavian arterial origin.ClassificationĪccording to the Celoria-Patton classification, IAA can be classified into three types according to the location of the anomaly: Pathologyįaulty embryological development of the aortic arch (thought to occur during the 5 th to 7 th week of intrauterine life). It may account for ~1.5% of congenital cardiac anomalies.

Interrupted aortic arch neural crest