A 26-year-old woman at 40 weeks of gestation attended our emergency department due to sudden onset of chest and back pain, although she had experienced no previous abnormalities. The patient was very tall, had spider fingers and scoliosis, all of which indicated Marfan's syndrome. Enhanced computed tomography and cardiac ultrasonography revealed acute aortic dissection and annulo-aortic ectasia with aortic regurgitation. Thereafter, a baby weighing 3,070g was delivered by emergency Caesarean section and then a Bentall-type operation was performed consecutively. An intraoperative injection of heparin resulted in minimal uterine bleeding. Surgery at the 40th week of gestation was successful for both the mother and the neonate.

A 34-year-old female patient, who had undergone several operations relating to pulmonary atresia with ventricular septal defect (PA/VAD), was admitted to our hospital for recurrent massive hemoptysis requiring blood transfusion. She had undergone a Potts' shunt (systemic-pulmonary shunt) at age 3, and undergone intraaortic patch closure for paraanastomotic pseudoaneurysm of occluded Potts' shunt at age 28. Chest CT scan revealed an enhancement of this aneurysm surrounding ground glass like appearance of left lung lobe which indicated pulmonary bleeding. In order to avoid skeltonization of severe adhesion of mediastinal and left thoracic space due to several prior operations, endovascular repair of this pseudoaneurysm with debranching of left subclavian artery was performed. Postoperative course was uneventful and hemoptysis was disappeared. She was discharged at 8 postoperative day.

A 71-year-old man had undergone branched open stent grafting for a distal arch aneurysm in May 2006. He subsequently developed multiple episodes of postoperative endoleak successfully treated by TEVAR in January and November 2009. He visited our hospital complaining of back pain in May 2011. Chest computed tomography showed increasing size of the aneurysm and recurrent endoleak of the distal stent graft, and impending rupture of the aneurysm was diagnosed. Considering the technical difficulty of repair by TEVAR, we performed graft replacement of the aneurysm with removal of the previous stent graft. The postoperative course was unremarkable and he was discharged on postoperative day 11.

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.