PAPVC is a rare congenital heart disease first reported by Winslow in 1789, Pathological studies show that this disease incidence 0.7% of the population[4],and research on the incidence of PAPVC has yielded a mixed bag of result, because many patients without clinical symptoms were not captured by the PAPVC rate[5]. Thus the true incidence of PAPVC is slightly higher than expected. Most cases of PAPVC are from the right lung, Only 10–18% cases of PAPVC from the left pulmonary are reported, PAPVC originating from the right pulmonary occurs ten times more commonly than the connecting veins started from the left lung[4].Few studies of anomalous connection of the left pulmonary vein to the coronary venous sinus have been reported[6]. And meanwhile, PAPVC presented with ASD was reported in 80%-90% of cases[1].Isolated PAPVC associated without ASD have been rarely reported. Additionally, to our knowledge, left pulmonary vein draining into the coronary venous sinus with intact atrial septum have not been reported at present .
The appearance of the clinical symptoms usually occurs in PAPVC during adulthood[2]. The pathophysiology of PAPVC is alike to the left-to-right shunt in simple ASD ,Congestive heart failure caused by PAPVC depends on how many pulmonary veins abnormally connect to the right atrium. Imaging examinations to diagnose PAPVC consist of chest plain films, CT and MRI, cardiac catheterization[7]. Doppler echocardiography reveals the trend and distribution of pulmonary veins. therefore, cardiac uhrasonography is becoming the simple and effective method of the PAPVC.
Those with PAPVC without symptoms should have a physical exam and an echocardiogram once a year. Patients with the symptoms of heart failure can be controlled with after load reduction, beta blockers ,diuretics, and cardiac glycosides. The surgical repair was the most effective treatment to PAPVC[6]. The preschool age is the optimal time for intervention. Surgical techniques depend on the number and location of abnormal veins or veins.