A Congenital Quadricuspid Aortic Valve Associated With Aortic and Mitral Regurgitation: Case Report and Literature Review.

Abstract

Quadricuspid aortic valve (QAV) is a rare congenital cardiac anomaly that commonly becomes symptomatic in the fifth or sixth decade of life and can present as an isolated finding or in association with other congenital cardiovascular abnormalities. Previously, QAV was mainly a postmortem or intraoperative diagnosis and data was very limited on its natural history, associated complications, and long-term outcomes. In recent decades, however, there has been an increase in the reported cases of QAV, considering the advances in the diagnostic modalities such as transthoracic echocardiography (TTE), transesophageal echocardiography (TEE), and cardiac magnetic resonance (CMR). In this article, we present a case of a congenital QAV associated with aortic regurgitation as well as briefly review the QAV classification systems, treatment options, and long-term outcomes.A 48-year-old woman with a significant past medical history (PMH) of hypertension and coronary artery disease presented with shortness of breath, chest pain, and orthopnea for two to three weeks. The patient’s TTE showed severe aortic regurgitation with significant compromise in cardiac output that could not be otherwise explained. Subsequently, her TEE revealed QAV as the underlying source of these findings.Although the diagnosis of QAV could be very challenging, it is crucial to be considered when evaluating a patient with inscrutable progressive aortic regurgitation. Today, as a result of technological advancement, QAV is being diagnosed more accurately and promptly. Since there are no universal guidelines defined for this cardiac anomaly, regular follow-up with these patients is imperative to monitor for early signs of valvular compromise and to treat accordingly through medical and surgical interventions.