Late Complication of Quadricuspid Aortic Valve: Early Moderate to Severe Aortic Regurgitation

Quadricuspid aortic valve (QAV) is a rare congenital cardiac anomaly. A normal aortic valve has three cusps, but cases of unicuspid, bicuspid, and quadricuspid aortic valves have been reported. Although QAV usually appears as an isolated congenital anomaly, it may also be associated with other heart conditions. In comparison to the bicuspid aortic valve (BAV) that results in aortic stenosis by the early 50s due to age-related early calcification, this case series suggests that patients with QAV are likely to develop moderate to severe aortic regurgitation in their late 40s or early 50s. Most patients with QAV require tricuspidalization, which is the preferred method for QAV surgical repair, especially in patients with associated aortic regurgitation. The condition was previously diagnosed intraoperatively or postpartum. Today, with imaging modalities like transthoracic echocardiography (TTE), transesophageal echocardiography (TEE), and cardiac magnetic resonance imaging, more cases of QAV have been diagnosed in asymptomatic individuals. We present a case series of a previously healthy 49-year-old male and a 47-year-old female who had similar presentations of acute congestive heart failure (CHF). An echocardiogram confirmed that both patients had heart failure with reduced ejection fraction, dilated cardiomyopathy, QAV, and moderate to severe aortic valve regurgitation on echocardiogram. The male patient had an ejection fraction (EF) of 30-35% and a QAV with partial fusion of the leaflets, resulting in a functionally bicuspid aortic valve, while the female patient had an EF of 25-30% with what appears to be a type III QAV according to Nakamura et al. classification. The purpose of this case series is to highlight another potential late complication of congenital QAV.