[Two cases of complete atrioventricular canal defect whose pulmonary vascular resistance were over 10 wood unit.m2 before 6 months of age].

We report two cases with complete form of atrioventricular canal defect (CAVCD) accompanied by Down's syndrome whose pulmonary vascular resistance (Rp) were more than 10 Wood unit.m2 at the age of less than 6 months. One child was 3-month-old boy whose Rp was 12 Wood unit.m2. The open lung biopsy at 3 months old showed histopathological change of Heath-Edwards grade I. He underwent intracardiac repair at the age of 4 months. He is doing well at 30 months of postoperative period. Another child was 5-month-old girl whose Rp was 15.5 Wood unit.m2. Histopathological change of lung at 5 months old demonstrated Heath-Edwards grade III. She underwent intracardiac repair at the age of 7 months, 2 months after lung biopsy. However, she died of oversystemic pulmonary hypertension and low output syndrome 7 days after surgery. The postmortem examination revealed that pulmonary vascular obstructive disease progressed during 2 months interval between the lung biopsy and the operation. In conclusion, cardiac catheterization with estimation of Rp should be performed in the cases of CAVCD, especially in those with Down's syndrome, in early infancy. If Rp is more than 10 Wood unit.m2 and lung biopsy indicates the surgical indication, surgical intervention should be done as soon as possible since the pulmonary vascular obstructive disease may progress in a short period.

[Successful repair of a newborn case with total anomalous pulmonary venous connection presenting hemodynamically common pulmonary vein atresia].

Common pulmonary vein atresia is a very rare from of congenital cardiac anomaly in which the common chamber of pulmonary veins has no direct communication to the heart or systemic venous system. We report here on one-day-old boy with total anomalous pulmonary venous connection having severe stenosis (sized less than one millimeter in diameter) of vertical vein between common pulmonary vein and superior vena cava. His anatomic feature was considered as a spectrum between common pulmonary vein atresia and total anomalous pulmonary venous connection because of almost atretic draining vertical vein. His clinical presentation and hemodynamic characteristics were the same as common pulmonary vein atresia. He underwent an emergent anastomosis of common pulmonary vein to left atrium at 33 hours after birth under cardiopulmonary bypass, cross-sectional echocardiography and Doppler color mapping. He was discharged home two months after the surgery. Cardiac catheterization after six months revealed normal cardiac function without any evidence of pulmonary venous chamber stenosis. To improve the outcome of surgical intervention for this life-threatening cardiac anomaly, immediate the precise diagnosis using echocardiography followed by emergent surgical correction is in dispensable.