We report two cases of coronary artery fistulas (CAF) in children with successful surgical repair. The first case was a 14-year-old boy with a continuous heart murmur. A fistula was diagnosed between the right coronary artery (RCA) and the right atrium by echocardiography. He was asymptomatic, but the pulmonary to systemic flow ratio was shown to be high by cardiac catheterization. He underwent surgical closure of the fistula and coronary artery bypass grafting to the RCA with the right interthoracic artery. The second case was a 5-year-old boy with a continuous heart murmur and cardiomegaly on the chest X-ray film. The CAF from the left coronary artery to the right atrium was revealed by echocardiography. The pulmonary to systemic flow ratio was shown to be high by cardiac catheterization. He was asymptomatic, but he also underwent surgical closure of CAF. Surgical repair was effective and safe in these cases. Echocardiography was very useful in the diagonosis of CAF. Surgical repair in the young is recommended because CAF is much more likely to cause congestive heart failure, angina, and infective endocarditis when growing.

A 34-year-old woman had received a diagnosis of X-linked hypophosphatemic osteomalacia when she was born. As an adult she complained of general fatigue, palpitations dyspnea exertional and leg edema. Transthoracic echocardiography showed aortic stenosis and regurgitation with severe aortic valve calcification. Chest computed tomography revealed her ascending aorta to be circumferentially calcified. Surgery was performed through a median sternotomy via cardiopulmonary bypass, with perfusion from the right subclavian artery and the right femoral artery and drainage from the superior and the inferior venae cavae. A left ventricular venting cannula was inserted from the right superior pulmonary vein. When her bladder temperature had dropped to 28°C by central cooling, we stopped the perfusion from the right femoral artery and performed aortotomy. We examined the lumen and clamped the aorta at the usual site, while flushing with blood by femoral perfusion. Aortic valve calcification was observed to extend from the sinus of valsalva to the outflow tract of the left ventricle on the non-coronary cusp side. He was necessary to repair the aortic annulus due to a defect resulting from careful removal of the calcified valve. We then performed aortic valve replacement using a 19-mm Carpentier-Edwards perimount Magna. Her postoperative course was uneventful without any sign of neurological complications, and she was discharged 13 days after surgery. On pathological examination, localized ossification with calcification was observed in her aortic valve which seemed to be characteristic of X-linked hypophosphatemic osteomalacia.

A 42 year-old woman with Marfan syndrome, who had replacement of the ascending aorta for acute aortic dissection several years ago, was found to have distal aortic arch aneurysm. The aneurysm had small entries at cervical arterial branches and large re-entry at the left external iliac artery. It was necessary to perform two-staged operation Bentall procedure with total arch replacement and abdominal aortic replacement with re-entry closure. It was usually performed with a primary entry closure for chronic aortic dissection, but massive invasion was expected. We performed catheter angiography for entry and re-entry, and decided to perform preceding re-entry closure. First, we underwent replacement of the abdominal aorta, and then successfully performed the Bentall procedure with total aortic arch replacement. The catheter angiography was useful for decision-making for medical treatment.

We report a rare case of acute type A aortic dissection with paraplegia which was reversed using cerebrospinal fluid drainage (CFD). The patient was a 80-year-old man who was admitted with acute back pain and paraplegia. Computed tomographic scans showed an acute type A aortic dissection. Four hours after onset of paraplegia, CFD was initiated by inserting an intrathecal catheter at L3-L4. Cerebrospinal fluid was drained freely by gravity whenever the pressure exceeded 10cmH₂O. After 32h, the neurological deficit was completely resolved. CFD can be considered a useful treatment in patients with paraplegia after acute aortic dissection.

A 20-year-old man suddenly complained of back pain and bilateral lower limb weakness. Computed tomography showed acute type B aortic dissection. The patent false lumen extended from distal arch to the left common iliac artery. The true lumen was severely compressed by the false lumen and both legs were ischemic. He underwent emergency fenestration of the abdominal aorta and stenting of the left iliac artery. Although the lower limbs ischemia was improved, he developed myonephropathic metabolic syndrome and received plasma exchange, continuous hemodialysis and endotoxin absorption therapy. Thirteen days after the operation, intestinal ischemia occurred and he underwent emergency bowel resection with creation of a stoma. Development of dissection to the superior mesenteric artery (SMA) and the malperfusion of SMA by severe compression of the true lumen were thought to cause intestinal ischemia.

A 67-year-old man was given a diagnosis of lung cancer and thoracic aortic aneurysm (TAA). We first performed thoracic endovascular repair (TEVAR), and then right lower lobectomy for lung cancer. TEVAR shortened the operation time and yielded less operative damage. Therefore, TEVAR can be an effective choice for simultaneous surgery of TAA and lung cancer.

An 84-year-old man, who had been given a diagnosis of advanced aortoventricular block 2 years previously, underwent implantation of a pacemaker (PM) through the left subclavian vein. However, 7 months later a skin ulcer developed at the implantation site, but without any evidence of bacterial infection. Therefore, a PTFE-covered PM battery was reimplanted at the same site. Three months later, the skin ulcer recurred and he received a third implantation in the right side. However, another skin ulcer with infection developed in the right side. He was then transferred to our hospital for another PM reimplantation. We covered the battery and the entire lead with PTFE, then placed the PM lead directly into the cardiac muscle, and implanted the PM battery below the rectal muscle under general anesthesia. A patch test 4 months later revealed a positive reaction to nickel and silicon. Finally, we diagnosed pacemaker dermatitis. The patient has remained free of skin ulcers for over 1 year.