Congenital rock and a hard place: unicuspid aortic valve with sinus of valsalva aneurysm

Abstract

Cardiac CT angiography (CTA) is an ideal tool to investigate possible cardiac malformations. In this case, careful planning of the CTA acquisition and reconstruction provided high resolution images of cardiac anatomy revealing 2 extremely rare coexisting congenital defects; a unicuspid aortic valve (UAV) and sinus of Valsalva aneurysm (SVA). Detailed planning of CTA acquisition reconstruction protocols is essential in obtaining necessary information for clinical decision-making strategies and interventions in the patients with suspected cardiac anomalies.

Unicuspid aortic valve presenting with cardiac arrest in an adolescent

“…Only one case, in which UAV in a 34-year-old man was followed with echocardiography, is documented in the literature. In 5 years, the patient's AR and stenosis progressed from ‘mild to moderate’ to ‘severe.’25 Studies to determine both the true incidence in the general population and the course of the condition in the asymptomatic patient are warranted.

Learning points

Unicuspid aortic valve (UAV) is a rare congenital anomaly commonly affecting patients in their third and fourth decades, and presenting with signs of heart failure.

Our case is that of a previously asymptomatic teenage girl with a UAV who presented with cardiac arrest and was treated successfully.

Only two other similar cases have been reported in the literature, both were of slightly older male patients.

Our case highlights the morbidity associated with the anomaly supporting the need for careful assessment of the valve in cases where UAV is suspected.

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