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.

Objective: Histological verification of epithelioid cell granuloma is important in diagnosing sarcoidosis; tissue sampling is a worldwide requirement. In 2006, to reduce medical expenses and avoid invasive procedures, diagnostic criteria without histological verification were permitted by the Japanese government. In 2015, new diagnostic criteria, allowed clinical diagnoses based on only respiratory, ocular, and cardiac systems with at least a two-system involvement, increasing the need to sample tissue from clinically unevaluable organs in suspected sarcoidosis. This study aimed to compare the characteristics of patients who were diagnosed with sarcoidosis according to the 2006 and 2015 criteria.Materials and Methods: Using the 2015 version, we re-evaluated the characteristics of 264 patients with diagnosed or suspected sarcoidosis according to the 2006 criteria, at Jichi Medical University Hospital between 2004 and 2012 (clinical diagnosis, 84; histological diagnosis, 117; suspected sarcoidosis 63).Results: Thirty-nine patients were diagnosed with suspected sarcoidosis due to the absence of at least a two-system involvement; two patients had insufficient laboratory data suggestive of sarcoidosis. Six patients moved from suspected sarcoidosis to a histological diagnosis because of a greater leniency in the criteria for supportive findings. The 2015 diagnostic criteria excluded patients with organ involvement without a requirement for systemic steroids from the clinical diagnosis group. A case of schwannoma, erroneously placed in the clinical diagnosis group by the 2006 criteria, was reclassified according to the 2015 criteria.Conclusion: The 2015 version is preferable for clinically diagnosing sarcoidosis, even without histological specimens, and provides guidance for indications for systemic treatment.