We describe a young adolescent patient with spontaneous abdominal aortic rupture who was treated successfully. A 14-year-old boy was admitted to our hospital with severe abdominal pain and hypovolemic shock, without any episode of trauma. Computed tomography (CT) revealed massive hematoma in the retroperitoneal space and extravasation of copious amounts of contrast medium in front of the terminal aorta. Neither aortic aneurysm nor dissection was observed in this CT. An emergency operation was carried out. At first, left thoracotomy and clamping of the thoracic descending aorta were performed in order to reduce the aortic bleeding. Midline laparotomy revealed an aortic perforation of approximately 8mm at the bifurcation of the abdominal aorta. The aortic wall surrounding the perforation was nearly normal without any aortic aneurysm or dissection. A segment of the terminal aorta (length, 3cm) including the perforated lesion was excised and reconstruction was performed with a woven Dacron tube graft (10mm in diameter). On microscopic examination, the marginal tissue near the perforation showed diminished elastic fibers and minimal dissection of the medial layer of the aortic wall; however, no cystic medial necrosis or inflammation was seen.

In atrioventricular (AV) discordance, a morphologic tricuspid valve functioning as a systemic AV valve often becomes incompetent and needs to be replaced. However, mitral valve replacement concomitant with tricuspid valve replacement is unusual in the disease. Here, we report a case of successful double AV valve replacement long after functional biventricular repair in AV discordance. A 32-year-old man with AV discordance was admitted with orthopnea. He had undergone the Rastelli procedure at age 10 and removal of the deteriorated conduit valve at age 24. Preoperative examinations revealed not only tricuspid but also mitral regurgitation. Both deteriorated valves were replaced with mechanical valves. In AV discordance after Rastelli procedure, a non-valved conduit may accelerate mitral deterioration because pulmonary hypertension from tricuspid regurgitation increases the afterload of the pulmonary ventricle.

A 43-year-old man visited another hospital because of dry cough and dyspnea in a supine position after having experienced chest pain about 1 month prior to his visit. He had undergone open aortic commissurotomy and ligation of the ductus arteriosus due to congenital bicuspid valve aortic stenosis and patent ductus arteriosus at age 13. CT scan showed a dissected giant aortic aneurysm (12.0cm in diameter) of the DeBakey Type II which compressed surrounding organs, such as his trachea, bilateral main bronchus, superior vena cava, and right main pulmonary artery. Echocardiograms revealed severe aortic stenosis and a dissecting ascending aortic aneurysm. The patient was admitted to our hospital and an urgent operation was performed. Under cardiopulmonary bypass with selective cerebral perfusion, a replacement of the aortic root and the ascending-arch aorta with the inclusion technique was performed. Postoperatively, the patient suffered from ventilatory disturbance under mechanical ventilation. CT scan showed a giant aneurysmal sac containing a hematoma in the perigraft space and the false lumen of the aneurysmal wall and remaining tracheobronchial compression. A reoperation was performed for removal of the hematoma and placation of the aneurysmal sac. The subsequent postoperative course was good. The patient was weaned from mechanical ventilation at 12 days and discharged at 67 days after the initial operation. Histologically, the resected aortic wall showed cystic medial necrosis.

Surgery for right atrial isomerism usually has a poor outcome because of complex congenital cardiac malformations. Here we rearn the outcomes of all 71 consecutive patients with right atrial isomerism whom we treated from their initial operation at Shizuoka Children's Hospital between January 1987 and October 2006. We categorized 34 patients treated between 1987 and 1996 as the ‘early’ group, and 37 patients between 1997 and 2006 as the ‘late’ group. The early group was more commonly associated with pulmonary stenosis (p=0.010), and the late group was more commonly associated with neonatal status (p=0.010), body weight less than 3.0kg (p=0.037), and pulmonary atresia (p=0.013). All 71 patients were scheduled for single ventricular repair. Survival in the early group was 52.9% at 1 year, and 32.4% at 5 years, and this poor outcome was related to 2 factors; cardiac dysfunction from volume loading and inappropriate lung perfusion area for Fontan completion. We therefore changed our surgical strategy in the late group as follows: earlier right heart bypass operation and aggressive atrioventricular valvoplasty to prevent volume overloading, and central pulmonary artery (PA) strategy (central PA plasty and blood flow source anastomosed to the central PA) and improvement of anastomosis of total anomalous pulmonary venous connection (TAPVC) to preserve appropriate lung perfusion area. If severe unbalanced pulmonary blood flow occurred, it was treated with a novel surgical approach consisting of ‘intrapulmonary-artery septation’. Survival in the late group was 66.8% at 1 year, and 53.1% at 5 years, which was better than the early group, but not with statistical significance (p=0.102). Univariate analysis identified significant risk factors for mortality as neonatal status (p=0.036), extracardiac TAPVC (p=0.049), and preoperative pulmonary vein obstruction (PVO) (p=0.001) in the early group, and mixed TAPVC (p=0.001) in the late group. Multivariate analysis identified preoperative PVO (p=0.038) in the early group, and mixed TAPVC (p=0.007) in the late group as significant risk factors for mortality. Outcome is improving with our current strategy of preventing volume overloading and preserving an appropriate lung perfusion area, even in the late group with more severe cases, and neonatal status, extracardiac TAPVC, and preoperative PVO are no longer risk factors for mortality, but mixed type TAPVC remains a serious problem and is associated with high mortality.

We report a case of reoperation for proximal and distal pseudoaneurysmal formations of the ascending aorta with aortic regurgitation (AR) after an ascending aorta replacement for acute type A aortic dissection. The patient was a 69-year-old woman who had undergone ascending aorta replacement for acute type A aortic dissection six years previously. Subsequent development of pseudoaneurysms of the ascending aorta and aortic regurgitation were revealed by computed tomography and echocardiography respectively. We chose debranch Thoracic Endovascular Aortic Repair (TEVAR) with a staged approach. First, aortic valve replacement, patch closure of proximal pseudoaneurysmal formation, coronary artery bypass, and ascending aorta-axillary artery bypass were performed. Two weeks later, debranching and TEVAR were performed. Cardiac reoperation for proximal and distal pseudoaneurysmal formations of the ascending aorta with aortic regurgitation after an ascending aorta replacement is known to be high risk. Nevertheless we performed the operation safely in two-stage surgery.

The patient was a 48-year-old woman who had been taking oral steroids for dermatomyositis since age 39. The patient experienced an episode of sudden chest tightness at age 48, and acute myocardial infarction was suspected. Coronary angiography revealed a right coronary artery occlusion ; emboli (thrombi) were collected from the same site. Left cardiac ventriculography revealed the presence of a mobile thrombotic mass in the ascending aorta. Although heparin therapy was initiated considering the possibility of thrombosis, no shrinkage of the mass was observed, and surgery was planned as per treatment guidelines. The mass was a rod-shaped thrombus measuring 20 mm×7 mm×7 mm attached to the aortic wall, approximately 2.5 cm distal from the entrance to the right coronary artery. Since the aortic wall at the site of the tumor attachment was normal, surgery involved only removal of the mass. A histopathological assessment revealed that the mass was a mixed thrombus containing both white and red thrombotic components. The patient was started on postoperative oral antiplatelet and anticoagulant drug therapy to prevent additional thrombosis, and no recurrence has been noted at 1 year postoperative.

Here, we report a patient who underwent surgery for acute aortic regurgitation (AR) due to rupture of an aortic valve commissure. The patient was a 51-year-old man who had undergone ascending aorta replacement for acute type A aortic dissection 6 years previously. He presented with a 2-day-history of headache and insomnia. Echocardiography showed only AR initially. However, 2 days later, a vegetation-like mass was noted at the aortic valve commissure on transesophageal echocardiography. We diagnosed AR associated with infective endocarditis, and decided to perform aortic valve replacement immediately. During surgery, we found that the cause of AR was rupture of the aortic valve commissure without infection. The cause of rupture in this case was suspected to be traumatic or myxomatous degeneration.