LAUSR

clinical presentation involving cyanosis and clubbing jugular vein distension, loud S2P2, and parasternal lift was concordant with Eisenmenger’s syndrome. Electrocardiography indicated the right axis deviation, right ventricular strain pattern and anterolateral ischemia was suspected (Fig. 1a). Echocardiography revealed a dilated PA and right chambers with a leftward septal shift (Videos 1 and 2), and the estimated PA systolic pressure was 110 mm Hg. The LMCA compression was suspected. Computed tomography showed a complete LMCA occlusion due to external compression by the PA aneurysm (Fig. 1b and 1c). Cardiac catheterization revealed an advanced PAH (mean PA pressure, 88 mm Hg; pulmonary vascular resistance, 22.2 woods unit; cardiac index, 1.6 L/min/m2). Aortagraphy and selective coronary angiography confirmed the LMCA occlusion (Fig. 1d and 1e, Videos 3 and 4). Coronary angiography also proved that the circumflex coronary artery originated from the right sinus of Valsalva, and the patient survived because of an efficient retrograde flow from the circumflex and right coronary arteries (Fig. 1f and 1g; Videos 5 and 6). Coronary by-pass surgery and PA aneurysm repair was offered. However, the patient refused surgery, and it was decided to switch the inhaled iloprost to parenteral prostanoid therapy.