title: Quadricuspid Aortic Valve: A Rare Congenital Cause of Aortic Insufficiency.
abstract: Quadricuspid aortic valve (QAV) is a rare congenital cardiac anomaly causing aortic regurgitation usually in the fifth to sixth decade of life. Earlier, the diagnosis was mostly during postmortem or intraoperative, but now with the advent of better imaging techniques such as transthoracic echocardiography, transesophageal echocardiography (TEE), and cardiac magnetic resonance imaging, more cases are being diagnosed in asymptomatic patients. We present a case of a 39-year-old male who was found to have QAV, with the help of TEE, while undergoing evaluation for a diastolic murmur. The patient was found to have Type B QAV with moderate aortic regurgitation. We also present a brief review of classification, pathophysiology, and embryological basis of this rare congenital anomaly. The importance of diagnosing QAV lies in the fact that majority of these patients will require surgery for aortic regurgitation and close follow-up so that aortic valve replacement/repair is done before the left ventricular decompensation occurs.
authors.affiliation: Department of Internal Medicine, St. Joseph's Regional Medical Center, 07503 NJ, USA.
authors.forename: Rahul
authors.initials: R
authors.lastname: Vasudev
authors.affiliation: Department of Cardiology, St. Joseph's Regional Medical Center, 07503 NJ, USA.
authors.forename: Priyank
authors.initials: P
authors.lastname: Shah
authors.affiliation: Department of Cardiology, St. Joseph's Regional Medical Center, 07503 NJ, USA.
authors.forename: Mahesh
authors.initials: M
authors.lastname: Bikkina
authors.affiliation: Department of Cardiology, St. Joseph's Regional Medical Center, 07503 NJ, USA.
authors.forename: Fayez
authors.initials: F
authors.lastname: Shamoon
keywords: Aortic regurgitation; congenital valvular diseases; quadricuspid aortic valve; transesophageal echocardiography quadricuspid valves