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Quadricuspid aortic valve (QAV) is a rare congenital anomaly that can present as aortic insufficiency later in life. We report a case of aortic regurgitation associated with a QAV, treated by aortic valve replacement. The patient presented with breathlessness, lethargy and peripheral oedema. Echocardiography and cardiac magnetic resonance revealed abnormal aortic valve morphology and coronary angiography was normal. The presence of a quadricuspid aortic valve was confirmed intra-operatively. This was excised and replaced with a bioprosthetic valve and the patient recovered well postoperatively. Importantly, the literature indicates that specific QAV morphology and associated structural abnormalities can lead to complications. Hence, early detection and diagnosis of QAV allows effective treatment. Aortic valve surgery is the definitive treatment strategy in patients with aortic valve regurgitation secondary to QAV. However, the long-term effects and complications of treatment of this condition remain largely unknown.


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