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.

A 52-year old woman was referred to our hospital because of abdominal pain and restlessness. A chest contrast-enhanced CT showed huge pericardial effusion and intraluminal defects in the main pulmonary artery. We could not make a diagnosis based on the cytology of the pericardial effusion and histopathology of the mass with a sample taken by a catheter. Therefore, we undertook biopsies of the mass by median sternotomy, which led to the diagnosis of pulmonary intimal sarcoma. The tumor resection was performed to release the right ventricular outflow stenosis. We tried to resect the tumor as much as possible, and reconstructed the pulmonary artery and aortic root. She was discharged to home and survived 5 months after surgery.