[Quadricuspid aortic valve as an unusual cause of severe aortic regurgitation]

Abstract

Background: Both, acute and chronic aortic insufficiency, can be caused by various conditions. While the acute form is often associated with an endocarditis, type A aortic dissection and trauma, one possible cause of chronic aortic insufficiency is a congenital malformation of the aortic valve. Among these malformations, the quadricuspid aortic valve is the most rare form. The diagnosis is often made by Doppler echocardiography, during cardiac surgery, or postmortem.

Case report: A 44-year-old female patient presented with a 2-year history of increasing dyspnea on exertion. The physical examination revealed a diastolic heart murmur in the second right intercostal space and a blood pressure of 170/60 mmHg. Transthoracic and transesophageal Doppler echocardiography disclosed a quadricuspid aortic valve with severe aortic insufficiency. After implantation of a mechanical aortic valve, the patient was clinically well.

Conclusion: Quadricuspid aortic valve, often associated with aortic insufficiency, is a rare condition. The diagnosis can be made by Doppler echocardiography. The special treatment should be based on objective and subjective findings. In the absence of severe aortic regurgitation or clinical signs, a medical treatment with close follow-up is justified. In case of severe aortic regurgitation or clinical signs of cardiac decompensation, surgical treatment is recommended.