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.

Quadricuspid aortic valve: Case series and review of literature

“…Type A—Four equal cusps; Type B—Three equal and one smaller cusp; Type C—Two equal larger and two equal smaller cusps; Type D—One large, two intermediate, one small cusp, Type E—Three equal and one larger cusp; Type F—Two equal larger and two unequal smaller cusps; Type G—Four unequal cusps. Type B being the most common . Both patients shown in Figures and have the type I Nakamura classification with the supernumerary cusp between the left and right coronary cusps and Type B by Hurwitz and Roberts classification.…”

“…Of these, the most frequent abnormalities are malformation and misplacement of the coronary ostia noted in 10% of cases . Preoperative diagnosis of a QAV is essential to recognize these associated anomalies before intervention . Echocardiography, in particular transesophageal echocardiography, along with computerized tomography or cardiac magnetic resonance imaging suffice as diagnostic imaging modalities in the majority of patients .…”

Quadricuspid aortic valve: A rare intraoperative diagnosis by transesophageal echocardiography

“…It is frequently associated with coronary ostial anomalies[ 1 ] and may present in adulthood. First reported by Balinton in 1862,[ 2 ] QAV was previously detected only during autopsy. With technological advances in the form of transesophageal echocardiography, cardiac magnetic resonance imaging, there has been increased diagnosis of this congenital anomaly even in asymptomatic individuals.…”

Quadricuspid aortic valve infective endocarditis

“…QAV is a rare congenital anomaly with an incidence of less than 0.05% . The first case was reported by Balinton in 1862 . To date, over 200 cases have been reported due to increased rate of detection using advanced imaging techniques such as TEE and MRI.…”

“…2 The first case was reported by Balinton in 1862. 3 To date, over 200 cases have been reported due to increased rate of detection using advanced imaging techniques such as TEE and MRI. QAVs result from aberrant fusion of the aorticopulmonary septum or abnormal proliferation in the cardiac trunk.…”