Hybrid Procedure For Pulmonary Atresia With Intact Ventricular Septum

Background/Objective:Pulmonary atresia with intact ventricular septum(PAIVS) is a rare form of complex congenital heart defect.It consists of pulmonary valve (PV) atresia,combined with varying degrees of right ventricular (RV) and tricuspid valve (TV) hypoplasia and with or without coronary artery abnormalities.This condition is associated with a duct-dependent pulmonary circulation,with severe hypoxemia and acidosis,50%of infants will death within two weeks if they are not treated.This condition is typically difficult to manage because of its wide spectrum of RV morphologic features.The optimal treatment pathway depends on several factors:Type of atresia, RV anatomy, TV size and function, and right ventricle-to-coronary artery fistulas all play roles in the decision toward a biventricular or a univentricular pathway.The purpose of this paper is to analyze the effect of the intervention and surgical Hybrid procedure and explore the suitable treatment for PA/IVS through the review of the cases.Materials/Methods:Between2009and2014,11PA/IVS patients with severe RV and TV hypoplasia from Department of Cardiac surgery, Children□s Hospital of Zhejiang University School of Medicine were enrolled in the study.All patients were diagnosed by echocardiography and angiocardiography before the surgical procedures.The cases of type Ⅱ PA/IVS were8and type III PA/IVS were2and type Ⅰ PA/IVS were1.The management was divided into three parts:stage Ⅰ arterial duct stenting,stage Ⅱ surgical treatment,stage Ⅲ interventional block atrial septal defect.Patients were followed up at1,3and6months post stage Ⅰ and1,3,6months and twice a year post stage Ⅱ.Analyze the effect of the intervention and surgical Hybrid procedure through review the echocardiography,ECG and chest X-ray.Results:No death was occurred in the study.7patients have completed surgical treatment,5of them have done the one-and-a-half ventricular repair,2of them have done the biventricular repair.One patient received stent balloon dilatation due to pulse oxygen saturation decrease at3months after DS.One patient did the one-and-a-half ventricular repair due to oxygen saturation sharp decrease and arterial duct embolism in the echocardiography at4months after DS.One patient occured pulmonary hypertensive crisis and low cardiac output syndrome after the one-and-a-half ventricular repair.3patients emerged pleural effusion after the one-and-a-half ventricular repair,and one of them had severe pneumonia.All patients were successfully discharged after active treatment.Follow-up for3months to4years, their symptoms were improved significantly, RV cavity and function and TV were well-developed than before, regurgitation of tricuspid valve was mild.Conclusion:Hybrid procedure is less invasive and have fewer complication and demostrates a higher survival rate,meanwhile,it is a safety therapeutic method. It is a promising and attractive alternative management of the PA/IVS with severe RV and TV hypoplasia. However, a clear need for further follow-up of long term efficacy of the technology.