B50-34 Four’s a Crowd: Incidental Quadricuspid Aortic Valve in a Critically Ill Patient

Abstract Introduction A quadricuspid aortic valve (QAV) is a rare congenital anomaly associated with a high incidence of aortic regurgitation (AR) that may progress to requiring surgical intervention. We present an incidental finding of QAV identified during evaluation of a critically ill patient. Case Presentation A 48-year-old man with severe nonischemic cardiomyopathy (last ejection fraction 12%) presented with exertional dyspnea, chest, and abdominal pain. He was hypotensive to 82/47 mmHg. Labs showed normal troponin but marked elevations in creatinine, bilirubin, and transaminases. CT chest revealed a dense right upper lobe infiltrate with diffuse tree-in-bud and micronodular opacities.He was started on broad-spectrum antibiotics and dobutamine and admitted to the ICU for shock and multiorgan failure. Transthoracic echocardiography on inotropic support showed moderate LV dilation with preserved biventricular function. During central line placement, he developed supraventricular tachycardia requiring emergent intubation. Four electrical cardioversions and amiodarone restored sinus rhythm. A resuscitative transesophageal echocardiogram (TEE) demonstrated globally reduced biventricular function, severe diastolic dysfunction, and a Hurwitz-Roberts type E QAV (see image). Despite vasopressors, bicarbonate infusion, and continuous renal replacement therapy, he developed refractory shock and died the next day. Discussion QAV is a rare congenital cardiac anomaly with an estimated prevalence of 0.005–0.006% in echocardiographic series. It is typically detected incidentally on echocardiography, CT, or MRI. Despite numerous prior transthoracic echocardiograms, this patient’s anomaly had not been reported previously.QAV is clinically significant due to its association with AR and aortic dilation. The Hurwitz-Roberts classification delineates eight different subtypes (Type A-H) by the relative size of cusps. Valve morphology influences outcomes: Hurwitz-Roberts type B (two larger and two smaller cusps) is most associated with severe AR. Other morphologies such as our patient’s type E (three equal cusps and one larger cusp), may remain functionally normal. Management centers on surveillance for AR progression and surgical repair or replacement in severe cases. Conclusion While the type E QAV in this case was unlikely to be the patient’s primary cause of shock, its recognition is clinically important. With growing use of resuscitative TEE in critical care and its superior resolution compared with transthoracic imaging, intensivists are increasingly likely to identify such congenital variants. Awareness of QAV can prevent misinterpretation during emergent imaging and ensure appropriate follow-up when aortic insufficiency contributes to hemodynamic instability. This abstract is funded by: None