Transesophageal Echocardiography of the Quadricuspid Aortic Valve

A 26-yr-old woman was referred to our institution for evaluation and management of aortic insufficiency (AI) secondary to a known quadricuspid aortic valve (AV). The congenital valvular disease, diagnosed in infancy secondary to a murmur, was followed closely with regular echocardiograms. Her exercise tolerance had been unremarkable until the past 3–4 mo when she noted increasing dyspnea on mild exertion. Transthoracic echocardiography demonstrated a normal left ventricular (LV) size and function, severe AI, and a mild aortic stenosis. No other pathology was noted. A cardiac magnetic resonance scan, performed to better define her aortic root and valvular anatomy, showed a quadricuspid AV with preserved valve opening motion pattern and severe central AI. The quadricuspid AV consisted of three cusps of equal size and one smaller accessory cusp. The tubular ascending aorta was mildly aneurismal but the aortic arch vessels and the descending thoracic aorta were normal. The patient was scheduled for AV replacement surgery. Intraoperative transesophageal echocardiogram showed a quadricuspid AV (Fig. 1; also please see video clip available at www.anesthesia-analgesia.org). In addition to a severe AI with a central jet width >65% of LV outflow tract, pressure half-time <200 ms, and holodiastolic aortic flow reversal in descending aorta, there was a mild aortic stenosis. The cusps showed a mild degree of calcification, but well-preserved motion. Further transesophageal echocardiogram examination showed normal LV function, mild LV hypertrophy, a patent foramen ovale, and mild aneurysmal dilation of the ascending aorta. The patient underwent a minimally invasive quadricuspid AV replacement with a pericardial tissue valve via a right thoracotomy. Histologically, the quadricuspid AV showed fibromyxoid degenerative changes. Her remaining hospital course was unremarkable.Figure 1.: The midesophageal aortic valve short axis view in regular mode (A) and zoom mode (B) shows a quadricuspid aortic valve. The four cusps demonstrated a mild degree of calcification, but a well-preserved motion. There was a central jet on Color Doppler examination consistent with severe aortic insufficiency.Quadricuspid AV is a rare congenital anomaly with an incidence between 0.008% at autopsy and 1% in patients presenting for AV surgery (1). The clinical significance of quadricuspid AV arises from its frequently abnormal function, as approximately 50% of patients with quadricuspid AV have AI. (2) In addition, quadricuspid AV can be associated with other congenital cardiac defects, including coronary ostium displacement or obstruction, altered coronary artery anatomy, ventricular septal defect, patent ductus arteriosus, subaortic fibromuscular stenosis, and malformations of the mitral valve (2). AV surgery is indicated in symptomatic patients, and recommended in asymptomatic patients with evidence of LV systolic dysfunction (LV ejection fraction <50%) or LV enlargement (LV end-systolic dimension >55 mm, or LV end-diastolic dimension >75 mm) who have not yet progressed to the decompensated stage.