Diffuse bilateral pulmonary arteriovenous fistula (PAVF) developed in a 19-month-old girl with polysplenia, single atrium, single right ventricle, left superior vena cava and interrupted inferior vena cava (IVC) after total cavopulmonary shunt (TCPS) operation. In addition, left pulmonary vein obstruction (PVO) by compression between the dilated hemiazygos vein and the atrium was identified. We performed total right heart bypass operation with anterior translocation of the hemiazygos vein for release of PVO and direct anastomosis of the hemiazygos vein to the hepatic vein to divert hepatic venous flow to the bilateral pulmonary circulation. Follow-up cardiac catheterization 9 months later showed disappearance of PAVF and no stenotic region. The merits of this operation are: 1) to provide balanced distribution of hepatic venous flow through the hemiazygos vein to the pulmonary circulation, 2) to release the PVO caused by the dilated hemiazygos vein, and 3) to allow for potential growth. This operation can be performed to Fontan candidates with interrupted IVC.

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 successful case of aortic valve-sparing root replacement for dilated aortic root after a Ross procedure. A 29-year-old male underwent a Ross procedure when he was 11 years old for congenital aortic bicuspid valve. The right ventricular outflow tract was reconstructed using an autologous pericardium as a single leaflet valve. Aortic root dilatation and moderate aortic valve regurgitation were noted. Further investigation with enhanced computed tomography and ultrasonic cardiography revealed good quality of leaflets and sufficient geometric height, and aortic valve-sparing root replacement was performed. In addition, we performed pulmonary valve replacement with a biological valve. The post-operative course was uneventful and the patient was discharged after 8 days with a completely controlled aortic valve regurgitation. No recurrence of aortic valve regurgitation was observed 1 year later. Because surgical outcomes of congenital heart diseases have improved and more patients have an increasing life expectancy, several other problems were revealed, such as the occurrence of aortic root dilatation after a Ross procedure. Aortic roots may dilate due to arterial pressure; however, valve-sparing procedures may be performed if the volume of the leaflets is sufficient.