Aortic tubular hypoplasia/preductal coarctation with large patent ductus arteriosus

Case contributed by Razan Hamed Alayed
Diagnosis probable

Presentation

The newborn presented with oxygen desaturation and hemodynamic instability after delivery.

Patient Data

Age: 1 day
Gender: Female

Hypoplasia of the aortic arch involving the distal arch and isthmus with remaining small communication with descending aorta.

Significant dilatation of pulmonary trunk with large PDA communicating with the descending aorta which appears normally located on the left side.  The pulmonary veins are seen and appear patent but attenuated.

Reflux of contrast in IVC and hepatic veins associated with the large right ventricle,  suggesting right-sided heart failure.

Case Discussion

A newborn presented with oxygen desaturation and hemodynamic instability soon after delivery.

The oxygen saturation was reduced in the lower limb than the upper limb while the blood pressure in both upper and lower limbs was almost equal.

An echocardiogram was ordered showing a large PDA and the diagnosis of interrupted aortic arch vs coarctation of the aorta was suspected.

Subsequently, CT angiography was undertaken which showed a hypoplastic aortic arch communication with the descending aorta and a large PDA .

The diagnosis of an interrupted aortic arch was excluded due to the presence of communication between the ascending and descending aorta.

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