Pulmonary atresia (PA) with ventricluar septal defect(VSD) is a complex congenital heart disease. The lack of knowledge on embryology, anatomy and pathophysiology of this disease with or without heterogenetic major aortopulmonary collateral arteries(MAPCA)leads to chaos of its surgical classification and management as well as poor results of surgical management. The operative technique in this field has been advancing a lot since the innovation of unifocalization. The surgical result is getting better and better, boosted by the updating of the principle and knowledge, but there is still a large room for progress. We reviewed the literatures on classification,surgical strategy, procedures and operationrelated issues and put them together here to delineate its past, present and future.
Objective To summarize clinical outcomes of right ventricle-to-pulmonary artery shunt （Sano shunt）as the first stage palliative operation for patients with pulmonary atresia with ventricular septal defect （PA/VSD）. Methods Between September 2009 and May 2011，17 PA/VSD patients underwent Sano shunt in Fu Wai Hospital. There were 10 male patients and 7 female patients with their median age of 9.7 （2.5-73.8） months and average weight of （8.3±3.4）kg. Preoperative McGoon ratio was 1.04±0.29 and Nakata index was （102.0±56.9） mm2/m2. Five children had severe intrapericardial left pulmonary aretery stenosis，11 patients had patent ductus arteriosus （PDA），and 1 patient had major aorto-pulmonary collateral arteries. Preoperative transcutaneous oxygen saturation （SpO2） was 72.6%±11.6%. Results All the patients received Sano shunt placement successfully. Eleven patients underwent concomitant PDA ligation，and 7 patients underwent concomitant left pulmonary artery plasty. The Sano shunts were constructed with glutaraldehyde-fixed autologous pericardium in 10 patients，fresh autologous pericardium in 1 patient and Gore-Tex graft in 6 patients. All the patients underwent reconstruction of the right ventricular outflow tract via the right ventricular incision. The average cardiopulmonary bypass time was （75.0±30.0） min. Postoperative SpO2 was 89.8%±5.3% and significantly higher than preoperative SpO2 （P＜0.05）. All the patients were discharged alive and followed up for （12.1±6.7） months. During follow-up，8 patients underwent angiography study whose McGoon ratio and Nakata index improved to 2.05±0.37 and （304.8±51.3） mm2/m2 respectively，both of which were significantly higher than preoperative values（P＜0.05）. Four patients successful underwent second stage total correction. Conclusion Sano shunt is a comparatively safe procedure as the first stage palliative operation for PA/VSD patients，and can significant improve their pulmonary artery growth.