A 37-year-old male patient presented with dyspnea, chest pain, and episodic dizziness to the point of near syncope on exertion. The patient reported that he was known to have a murmur since he was 2 years old. On clinical examination, systolic and diastolic murmurs could be auscultated. There was no family history of significant cardiac disease, aneurysm, or premature sudden death. Physical examination revealed a 4/6 systolic ejection murmur that radiated to the right greater than left carotid and a 2/6 diastolic murmur heard at the upper sternal borders accentuated with forced exhalation. The remainder of his clinical examination and his laboratory tests were unremarkable.The workup included a chest X-ray, echocardiogram, computed tomographic angiography, and aneurysmal survey which did not show any evidence of additional aneurysmal disease elsewhere in the body. The echocardiogram revealed severe aortic stenosis with a gradient of 54 mm Hg and the valve velocity of 3.7 m/sec, severe aortic insufficiency, a bicuspid aortic valve, severe left ventricular hypertrophy, left atrial dilatation, and an enlarged ascending aorta. CT imaging showed a bicuspid aortic valve with thickened, fused right and noncoronary cusps in addition to a nodular density immediately distal to the cusps. The ascending aorta was noted to be enlarged to 4.1 cm in diameter. The aortic dilatation continued into the arch. The left subclavian artery was also dilated at the takeoff from the aorta and measured 4.2 cm at its origin (►Figs. 1 and 2). The right brachiocephalic artery was abnormal and measured 2.3 cm in its proximal diameter (►Figs. 3 and 4). The left vertebral artery took off from the dilated left subclavian artery and was smaller in caliber compared with the right vertebral artery. Coronary imaging disclosed an absent left circumflex with a diagonal vessel supplying the lateral wall, with no significant stenosis or calcifications was noted.A two-stage procedure was undertaken. The aortic valve and ascending aorta were addressed first. Intraoperative transesophageal echocardiography confirmed the findings noted in preoperative echocardiography. The ascending aorta was noted to be very small, measuring 20 mm at the level of the sinotubular junction, and exceptionally thin walled. The aorta was opened longitudinally and transected above the coronary arteries allowing visualization of the markedly deformed bicuspid aortic valve and annular distortion. There was a 2 cm nodular atheroma originating in the aortic valve and attached to the aortic wall; the valve was nearly unicuspid on direct inspection. The abnormal leaflets were removed, the annulus debrided, and a St. Four weeks later, the patient was taken back to the operating room to address the left subclavian artery aneurysm. Intraoperative transesophageal echocardiography revealed a competent mechanical aortic valve with no abnormalities. A left thoracotomy was performed and the Keywords ► subclavian artery aneurysm ► subclavian ► aneurysm ► bicuspid aortic valve
AbstractWe report...