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The Quadricuspid Aortic Valve as Visualized at Cardiac Catheterization, Transesophageal Echocardiography, and at Surgery
Iosif Gulkarov, Anastasia Plaunova, Anthony Tortolani,
Berhane Worku.
New York Methodist Hospital, Brooklyn, NY, USA.

OBJECTIVE The quadricuspid aortic valve is a very rare congenital anomaly usually found only at surgery or autopsy, as traditional evaluation by transthoracic echocardiography can miss this anatomy. Here we show excellent visualization of the quadricuspid valve at catheterization, on transesophageal echocardiography, and at surgery. METHODS: We describe a case of severe aortic regurgitation secondary to a quadricuspid aortic valve treated with valve replacement. Preoperative and intraoperative imaging is demonstrated. RESULTS: A 38-year-old female presented to her primary care physician with worsening dyspnea and chest pain at rest for several months. She had a positive stress test, followed by cardiac catheterization revealing normal coronaries, severe aortic regurgitation, and suggesting a quadricuspid aortic valve (Fig.1: A). Transthoracic echocardiogram demonstrated severe aortic regurgitation with a probable trileaflet valve. Transesophageal echocardiography demonstrated a quadricuspid aortic valve (Fig.1: B, C), which was confirmed intraoperatively (Fig.1: D, E). This is a class A valve on the Hurwitz and Roberts nomenclature with four equal leaflets. The patient underwent valve replacement with a pericardial bioprosthesis and was discharged home uneventfully. She is currently asymptomatic and doing well four months postoperatively. CONCLUSIONS: Aortic regurgitation may be secondary to a congenitally quadricuspid aortic valve. This finding may be missed preoperatively and is usually discovered during intraoperative inspection. We demonstrate preoperative findings of a quadricuspid aortic valve and intraoperative correlation. Aortic valve replacement is usually successful at treating symptoms and preventing progressive heart failure.

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