A 72-year-old man had undergone aorto-bifemoral bypass for Leriche syndrome at age 67, but acute limb ischemia developed three times after the first operation, in January 2008, April 2008, and April 2009. There were no abnormal heart rhythms or thrombotic factors, and he had received anticoagulant therapy with warfarin (target prothrombin time-international normalized ratio : 1.7 to 3.0) since January 2008. Nevertheless, he came to our hospital because of sudden onset of severe pain in the right lower limb in April 2010. Since CT showed occlusions in the right leg involving the aortobifemoral bypass and femoropopliteal bypass graft, emergency thrombectomy and femoropopliteal bypass (below knee), was performed. CT on admission showed enlargement of lymph nodes around the stomach, and gastric cancer was diagnosed by esophagogastroduodenoscopy. Since we considered the hypercoagulability in this patient with cancer to have resulted in repeated acute arterial thrombosis, these episodes were broadly diagnosed as Trousseau's syndrome.

Percutaneous transcatheter closure of ostium secundum atrial septal defect (ASD) has become an alternative to conventional open surgical repair. Cardiac perforation is a rare complication after transcatheter closure of ASD by an Amplatzer Septal Occluder (ASO). We present a patient with hemodynamic collapse secondary to cardiac perforation occurring 5 months after placement of the ASO and discuss the complications of this device. A 14-year-old girl underwent transcatheter closure of ASD by the ASO in our institution. Transesophageal echocardiography showed ASD sized 17.4×15.0mm, with no aortic rim. The placement of the ASO was performed without complications, but 5 months after the procedure she started to complain of chest pain and subsequent syncope. She was brought to a local emergency department. Transthoracic echocardiography showed an important cardiac effusion with signs of cardiac tamponade. Emergency pericardial drainage was performed under echocardiographic control from the subxiphoidal region. Once she was hemodynamically stabilized, the patient was transferred to our institution immediately for the necessary emergency surgical procedure. The operation was performed through a median sternotomy and the bleeding source was identified. The left-side of the ASO disc had cut through the roof of the left atrium between the superior vena cava and the aortic root, creating a 5-mm perforation. There was another perforation at the aortic root in the region of the non-coronary sinus of Valsalva, approximately 5 mm. The metallic rim of the ASO could be easily seen protruding through the roof of the left atrium. Cardiopulmonary bypass was established and cardiac arrest induced. After opening the right atrium we found the ASO, which was positioned well. The ASO was removed and the perforations of the aortic root and the left atrium were closed with 5-0 polypropylene directly. Then the ASD was closed using an autopericardial patch. The patient was weaned off bypass without difficulty. The postoperative course of the patient was uneventful and free of neurologic events. Finally, we conclude that patients with an aortic rim defect may be at higher risk for device perforation. Such a patient should be carefully followed up by echocardiography.

An 81-year-old man underwent aortic valve replacement with a 21-mm Medtronic Mosaic porcine bioprosthesis for the treatment of bicuspid aortic valve stenosis. In addition to the appearance of chest discomfort on effort and a new diastolic murmur, echocardiography performed 2 years and 3 months after the surgery showed a high pressure gradient across the bioprosthetic valve and a reduction in the valve orifice area. Prosthetic valve dysfunction was diagnosed. During a repeat operation, 2 large tears on the left cusp and a subvalvular overgrown abundant pannus were observed, and the bioprosthetic valve was replaced with a 19-mm On-X mechanical heart valve. On analysis of the explant bioprosthesis, the right non-coronary stent post was bent outwards by approximately 9°, it compressed the left cusp by pulling the left right and left non-coronary stent posts closer together, thus altering the leaflet geometry and function. We speculated that pannus formation had resulted from turbulent blood flow caused by impaired or altered leaflet function. The 2 large tears appeared to be the result of contact with the bias cloth secondary to the stent distortion.

Shprintzen-Goldberg syndrome (SGS) is a rare disorder with many characteristics of generalized connective tissue dysplasia. SGS is characterized by Marfanoid habitus with craniosynostosis and mental retardation. Patients with SGS have cardiovascular disorders similar to Marfan syndrome (MFS) and those disorders seem to play an important role in the prognosis of SGS. To our knowledge, only 19 patients with SGS have been reported, and 7 of them had cardiovascular disorders. The major cardiovascular disorders of SGS are aortic root dilatation and mitral valve prolapse. We reported the first case of SGS successfully treated surgically for cardiovascular disorders. Since then, we performed another operation in a patient with SGS. In this paper, we report our surgical results in patients with SGS.

There are rare reports of families with multiple members with aortic dissection in the absence of Marfan syndrome. We encountered four cases of aortic dissection in two families. The aortic dissection occurred in the mother and child of the first family and in sisters of the second family. All cases had systemic hypertension preoperatively and presented Stanford type A aortic dissection. All of them were operated successfully. None of them showed the characteristics of connective tissue disease affecting the skeletal, ocular, and cardiovascular system. However, many members of the two families had systemic hypertension and histopathological examination of the aorta showed cystic medial necrosis in all of the four cases. The present study suggests that the familial aortic dissection may be caused by weakness of the aortic wall related to heredity and systemic hypertension.

The patient was an 89-year-old male who underwent transcatheter edge-to-edge repair to the mitral valve using MitraClip for severe degenerative mitral regurgitation (MR) one year earlier. Although two clips were implanted, grade III/IV MR still remained. As his heart failure progressed, he was referred to us for surgery. The patient also had aortic stenosis. He underwent mitral valve repair and aortic valve replacement. The postoperative course was uneventful. He was transferred to the referring hospital on postoperative day 14. When performing MitraClip for degenerative MR, it is important to consider carefully not only the operative risk for open surgery but also the anatomical adequacy of MitraClip. When MitraClip fails to control MR, early surgical intervention should be considered.

A 77-year-old man presenting with uremic acidosis was referred to our department for a misplaced vascular access catheter. Computed tomography revealed the catheter was passing through the subclavian artery and terminating in the ascending aorta. Under angio-fluoroscopic monitoring, a VIABAHN stent graft was deployed immediately after removing the catheter. The patient had no hemorrhagic complication although continuous hemodiafiltration was started just after surgery. His postoperative course was uneventful.