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Quadricuspid aortic valve: a rare but important abnormality
  1. Yu-Hsiang Juan1,2,
  2. Hui Liu2,3,
  3. Alfonso H Waller2,
  4. Amil M Shah2,
  5. Ron Blankstein2
  1. 1Department of Medical Imaging and Intervention, Chang Gung Memorial Hospital, Linkou and Chang Gung University, Taoyuan, Taiwan
  2. 2Non-Invasive Cardiovascular Imaging Program, Department of Medicine (Cardiovascular Division) and Department of Radiology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA
  3. 3Department of Radiology, Guangdong General Hospital, Guangdong Academy of Medical Sciences, Guangdong, Guangzhou, China
  1. Correspondence to Dr Hui Liu, Department of Radiology, Guangdong General Hospital, Guangdong Academy of Medical Sciences. No.106, Zhongshan 2 Rd, Guangzhou 510080, People's Republic of China; liuhuijiujiu{at}gmail.com

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Introduction

Since first reported in 1862, quadricuspid aortic valve (QAV) remains rare with a reported incidence of 0.008–0.033% on autopsy; however, the actual incidence of QAV is underestimated.1 ,2 QAV is characterised by the presence of four aortic cusps of varying sizes. Clear delineation of the anatomy of the QAV is difficult but of great clinical importance as these patients may present with progressive aortic regurgitation (AR) and other cardiovascular abnormalities.1 ,2 We present a case of QAV in an asymptomatic patient who presented with a murmur on routine examination.

Case report

An asymptomatic patient presented with a 2/6 grade holosystolic apical cardiac murmur on routine physical examination. Transthoracic echocardiography (TTE) demonstrated QAV with cusps of equal size (figure 1A, upper and middle images), mild AR and a small restrictive muscular ventricular septal defect (VSD) (figure 1A, lower image). Cardiac MRI …

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