Vol.47 - Número 3, Julio/Septiembre 2018 Imprimir sólo la columna central

Current underdiagnosis of unicuspid aortic valve by different imaging tests

Hospital de Tortosa Verge de la Cinta. IISPV.
(43500) Tortosa, Tarragona, España.
Recibido 06-MAYO-2018 – ACEPTADO despues de revisión el 27-MAYO-2018
There are no conflicts of interest to disclose.


Congenital anomalies of the aortic valve amount to 3-6% of congenital heart diseases in adults, with bicuspid aortic valve being the most frequent (2%); followed by the unicuspid (UAV) and the quadricuspid ones. The prevalence of UAV in echocardiographic series is 0.02%; and in series of patients operated by aortic stenosis, with or without insufficiency, 4-5% [1-2]. However, in spite of the new imaging techniques (computed tomography and magnetic resonance) allowing to assess accurately the cardiac anatomy; and current echocardiographs and particularly transesophageal echo, which equally enable a thorough study, UAV is still underdiagnosed.

We report the experience on this field, of an echocardiography laboratory where, from an average of 1500 echocardiograms/year, 3 cases were detected in 15 years. The characteristics of this congenital heart disease and the main findings in these patients in general [1-5], as well as those detected in the 3 patients of the series, in particular, are shown and compared in Table 1.

constitutes a rare congenital anomaly to be considered in the differential diagnosis of young patients with aortic valve disease, particularly stenosis. Figure 1 displays UAV in diastole and systole, along with the associated coronary artery anomaly with independent ostia of the anterior descending and circumflex coronary arteries.

From the review of the 3 mentioned cases (Table 1), one of whom corresponds to the images in Figure 1, the following diagnostic conclusions are gleaned: 1) that its detection is only possible after a high index of suspicion; and 2) that in spite of having several imaging techniques available, echocardiography is still essential (particularly transesophageal), as the absence of separation of the aortic cusps reveals its characteristic eccentric “teardrop” opening during cardiac systole (Figure 1).


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  4. Novaro GM, Mishra M, Griffin BP. Incidence and echocardiographic features of congenital unicuspid aortic valve in an adult population. J Heart Valve Dis 2003; 12: 674-8.
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Publication: September 2018


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