Interrupted aortic arch

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Interrupted aortic arch (IAA) is an uncommon congenital cardiovascular anomaly where there is a separation between the ascending and descending aorta¹. It can either be complete or connected by the a remnant fibrous band². An accompanying large ventricular septal defect (VSD) and/or patent ductus arteriosus (PDA) is frequently present.

Epidemiology

It may account for ~1.5% of congenital cardiac anomalies.

Pathology

Faulty embryological development of the aortic arch (thought to occur during the 5^th to 7^th week of intra uterine life).

Classification

It can be classified to three types according to location of occurrence:

type a: second commonest, interruption occurs distal to the left subclavian arterial origin
type b: commonest (>50%), interruption occurs between left common carotid arterial and left subclavian origins
type c: rare, interruption occurs proximal to left common carotid arterial origin

Each type is divided into 3 subtypes ⁷:

sub-type 1: normal subclavian artery
sub-type 2: aberrant subclavian artery
sub-type 3: isolated subclavian artery that arises from the ductus arteriosus.

Associations

DiGeorge syndrome ¹
- found commonly in those with a type B interruption
- almost always associated if there is a right sided descending aorta.
truncus arteriosus ⁸

Radiographic features

Plain film: chest radiograph

Plain film features are often non specific³:

the aortic knuckle may be absent
may show cardiomegaly

Antenatal ultrasound

The right ventricle may appear a lot larger than the left, although this is a non specific finding. The ascending aorta may also appear more vertical than usual.

MRI/MRA

non visualisation of portion of interruption
great vessels may show a "V" configuration on coronal imaging ²

Treatment and prognosis

If un-corrected, it carries a very poor prognosis with extra uterine survival being as less as a few days. Prostaglandin E₁may be given as initial management to keep the ductus open.

Differential diagnosis

General differential considerations include:

short segment severe aortic coarctation

-<p><strong>Interrupted aortic arch (IAA)</strong> is an uncommon <a href="/articles/congenital-cardiovascular-anomalies">congenital cardiovascular anomaly</a> where there is a separation between the ascending and descending aorta <sup>1</sup>. It can either be complete or connected by the a remnant fibrous band <sup>2</sup>. An accompanying large <a href="/articles/ventricular-septal-defect-1">ventricular septal defect (VSD)</a> and/or <a href="/articles/patent-ductus-arteriosus">patent ductus arteriosus (PDA)</a> is frequently present.</p><h4>Epidemiology</h4><p>It may account for ~1.5% of congenital cardiac anomalies. </p><h4>Pathology</h4><p>Faulty embryological development of the aortic arch (thought to occur during the 5<sup>th</sup> to 7<sup>th</sup> week of intra uterine life).</p><h5>Classification</h5><p>It can be classified to three types according to location of occurrence:</p><ul>
+<p><strong>Interrupted aortic arch (IAA)</strong> is an uncommon <a href="/articles/congenital-cardiovascular-anomalies">congenital cardiovascular anomaly</a> where there is a separation between the ascending and descending aorta. It can either be complete or connected by the a remnant fibrous band. An accompanying large <a href="/articles/ventricular-septal-defect-1">ventricular septal defect (VSD)</a> and/or <a href="/articles/patent-ductus-arteriosus">patent ductus arteriosus (PDA)</a> is frequently present.</p><h4>Epidemiology</h4><p>It may account for ~1.5% of congenital cardiac anomalies. </p><h4>Pathology</h4><p>Faulty embryological development of the aortic arch (thought to occur during the 5<sup>th</sup> to 7<sup>th</sup> week of intra uterine life).</p><h5>Classification</h5><p>It can be classified to three types according to location of occurrence:</p><ul>

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