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Interruption of the Aortic Arch


Introduction

Interruption of the aortic arch (IAA) is an uncommon ductal-dependent congenital cardiovascular malformation. It is the most severe form of aortic coarctation (AoC).

Disease

Definition

IAA is characterized by the lack of continuity between the ascending and descending aorta.

Prevalence and Epidemiology

IAA accounts for 0.1% of all prenatally detected congenital heart diseases (CHDs) and 0.2% to 1% of postnatal CHDs, affecting 1 : 300,000 newborns. IAA has a high prevalence of associated anomalies, including:

  • Extracardiac abnormalities: central nervous system, urinary, and gastrointestinal malformations are the most frequently associated (present in 30% of cases).

  • Chromosomal abnormalities: type B IAA has a high association (60% to 70%) with 22q11 microdeletion.

  • Cardiac anomalies: associated cardiac anomalies are common, especially truncus arteriosus (10% to 20%), left ventricular outflow tract obstruction (10% to 16%), aortic valvular stenosis (10%), aortopulmonary window, double-outlet right ventricle (5%), and transposition of great arteries (4% to 6%).

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