Quadricuspid Aortic Valve: A Rare and Incidental Finding

Abstract

A quadricuspid aortic valve (QAV) is an exceedingly rare congenital heart anomaly with around 200 cases reported in the literature since the first case was reported in 1862. To the best of our knowledge, there has not been any case of QAV associated with pregnancy. We report a case of a 29-year-old female with new-onset palpitations, diagnosed with QAV and mild aortic regurgitation during pregnancy. The patient presented with new-onset intermittent palpitations at the 37th week of pregnancy. Electrocardiogram (EKG) showed normal sinus rhythm, and transthoracic echocardiography revealed a quadricuspid aortic valve with three equal-sized cusps and one smaller cusp and mild aortic regurgitation without any additional anomalies. QAV morphology falls under the category type b Hurwitz & Roberts classification. She underwent normal vaginal delivery without any peripartum cardiac complications. In conclusion, QAV is a rare congenital anomaly. It is not uncommon to be associated with aortopathies. The presence of QAV and associated anomalies in pregnancy makes it a high-risk state. Close monitoring, especially during the second and third trimesters, remain of utmost importance. Because of its rarity, the characteristics, natural history, and long-term outcomes of QAV are poorly defined.