Extreme shortening of non-coronary cusp of the aortic valve detected by transesophageal echocardiography: a case report

Excerpt

The etiology of aortic regurgitation (AR) includes aortic root dilation, congenital bicuspid valve, and calcification. Acute severe AR can be caused by aortic dissection and infective endocarditis [1, 2]. A 66-year-old male patient presented with a 2-month history of progressively worsening dyspnea on exertion. There was no history of rheumatic fever or other diseases. Vital signs were as follows: blood pressure 149/54 mmHg, pulse rate 80 bpm, percutaneous oxygen saturation 100% on room air, and body temperature 36.6℃. Physical examination revealed a diastolic murmur in the third intercostal space along the left sternal border, while breathing sounds were clear, and there was no extremity edema. Additionally, there were no suggestive of infective endocarditis (IE) and rheumatoid arthritis (RA). Serum N-terminal pro-brain natriuretic peptide concentration was 522 pg/mL. Chest X-ray and computed tomography scan showed bilateral pleural effusions, leading to admission for further examinations. Transthoracic echocardiography demonstrated severe AR. The eccentric AR jet along the non-coronary cusp, suggesting prolapse of the right coronary cusp as an etiology of regurgitation (Fig. 1a–d). Left ventricular end-diastolic dimension was slightly increased (56 mm), and left ventricular ejection fraction was preserved (61%). Transesophageal echocardiography revealed severe shortening of the non-coronary cusp (NCC) and mild shortening of the left coronary cusp. Calcification was observed at each commissure but with no fusion. Long-axis view showed thickening and severe shortening of the NCC with regurgitation (Fig. 1e–h). The aortic root was not enlarged. Furthermore, the three-dimensional color Doppler echocardiography and the technique multiplanar reconstruction showed that the large vena contracta area existed at the site of NCC during all diastole (Fig. 1i). After diagnosing severe AR resulting from extreme shortening of the NCC, the patient underwent aortic valve replacement using Avalus 23 mm valve on the 13th day after admission. The operative findings confirmed the extremely shortened and almost disappeared NCC, leaving only a thickened valve annulus (Fig. 1j, k). However, the pathological finding of the valve leaflet could not confirm as IE because NCC could not be removed, and others valve leaflets were no inflammatory histologic features. In addition to general mechanisms of AR [1, 2], there was possibility of valve leaflet shortening due to IE [3] or destruction due to RA [4]. However, in our case, even pathological finding did not prove the inflammatory features. As a result, the actual differential diagnosis was challenging. Nevertheless, we report this rare case of extreme shortening of NCC.

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