The patient was a 69-year-old man brought to the emergency room with severe chest pain. A contrast chest CT scan revealed a hematoma around the ascending aorta and a notch in the aortic wall, suggesting an intimal tear. An emergency operation was performed via standard median sternotomy under deep hypothermic circulatory arrest. Upon operation, there was a 2.0 cm intimal tear just above the right coronary leaflet, which was extended near the right coronary artery orifice. There was no specific evidence of aortic aneurysm or dissection, therefore a spontaneous rupture of the ascending aorta was diagnosed. The pathological finding was cystic medial necrosis Grade 2. His postoperative course was unremarkable and he was discharged 12 days after surgery.

We report on a rare case of a left subclavian artery aneurysm concomitant with Loeys-Dietz Syndrome (LDS) which required us to operate in childhood. A 12-year-old female, who had been diagnosed with LDS by genetic examination one year eariler, was revealed to be suffering from a left subclavian artery aneurysm (25 mm in diameter) by follow up chest MRI scan. Hybrid surgery consisting of coil embolization of aneurysm and surgical revascularization of the left subclavian artery and left vertebral artery was performed successfully. The patient was discharged on postoperative day 8.