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A 28-year-old man admitted our outpatient cardiology clinic with exertional dyspnea. He has been diagnosed with bicuspid aortic valve for five years ago, however, he did not go routine follow-up.  Cardiac examination revealed regular pulse (90 beats/min) with normal blood pressure and a 3/6 systolic crescendo-decrescendo murmur loudest at the right upper sternal border. Electrocardiography revealed normal sinus rhythm with left ventricular hypertrophy. Transthoracic echocardiography showed bicuspid aorta with aortic aneurysm and severe aortic regurgitation. Interestingly, severe calcification of the valve was extending to subvalvular area and causing severe left ventricular outflow tract obstruction (subaortic stenosis)(Figure 1, Video 1). Transesophageal echocardiography and computed tomography revealed that calcification was starting from coronary cusp and extending to the interventricular septum and caused severe septal hypertrophy (Figure 2, Video 2). The patient underwent aortic valve replacement with mechanical prosthesis and thoracic aorta replacement with a Dacron graft after heart team discussion. The patient was well after surgery and discharged without any problem (Figure 3, Video 3).
BAV is the most common congenital cause for the development of aortic valve calcification 1 . Calcification of BAV has different genetic tendency in contrast to tricuspid valves2. BAV mostly undergo strain, and wall shear stress on their edges of the leaflets during opening 3, however, in this case calcification started from annularly and extending to LVOT and basal septum. Besides the valve calcifications and stenosis, subaortic stenosis caused patient’s symptoms.
Funding : None
Acknowledgement: Informed consent was obtained from the patient to use of images for academic purposes.
Conflict of interest : None
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