Excerpt

A 47-year-old man with paroxysmal atrial fibrillation (AF) and cerebral embolism was referred to Gunma prefectural cardiovascular center for AF ablation. Physical examination revealed no murmur or obvious ejection systolic click. Chest X-ray did not reveal obvious cardiomegaly but electrocardiography indicated AF. To assess structural heart disease, cardiac function and left atrial thrombus prior to AF ablation, two-dimensional transthoracic echocardiography (2DTTE), and transesophageal echocardiography (TEE) were performed. 2DTTE indicated an atypical aortic valve (AV) form, with trivial aortic regurgitation. AV cusp doming was noted on parasternal long-axis plane. In parasternal short-axis view (Online Resource Movie 1), four sinuses of Valsalva (SOV) were visualized, and the AV closure appeared “four-leaf clover-like” in appearance while in diastole, suggesting a quadricuspid AV (QAV) (Fig. 1a). However, in systole, the AV was formed by two functional leaflets with a central raphe and two complete commissures, with a football-shaped opening (Fig. 1b). A clopped enface view of the AV, using three-dimensional TEE (3DTEE, Online Resource Movie 2), featured simultaneous and comprehensive visualization of AV and aortic root morphology including four SOVs, two raphes, two commissures, vertical valve opening motion, and orifices of right and left coronary artery (RCA and LCA, respectively), compared to that of 2DTTE (Fig. 1c, d). The 3DTEE also confirmed equal size of each SOV and identified each cusp according to the orifice of RCA and LCA (Fig. 1c). According to 2DTTE and 3DTEE findings, we diagnosed the patient as having a type A QAV with four equal leafs-like bicuspid AV (BAV), with two raphes. Prior to AF ablation, multidetector computed tomography (MDCT) was performed for electroanatomical mapping (Fig. 1e, f and Online Resource Movie 3). These images of AV in diastole were not necessarily superior to TEE. Moreover, CT images in systole were not available because of AF rhythm and CT revealed no ascending aortic dilatation.

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