INTRODUCTIONAbsent pulmonary valve syndrome (APVS) with an intact ventricular septum (IVS) or small ventricular septum is a rare type of APVS associated with high perinatal mortality in fetuses. Although APVS/IVS constitutes only 7.5% to 25% of the APVS cohort, most fetuses (80%) will not survive to birth without inotropic support or heart transplantation due to fetal heart failure, hydrops fetalis, extracardiac and chromosomal abnormities, and pregnancy termination[1-4]. A ductus arteriosus(DA) is usually present in APVS/IVS patients, and a large DA could result in bronchial compression leading to severe respiratory symptoms and signs of congestive heart failure early after birth[5]. With the improvement of postnatal care and intervention, the survival of subjects rises from 20% to 80%[2]. However, long-term surviving analysis remains unknown in patients with APVS/IVS.Symptoms varies according to individual in these patients. Here, we report a rare subtype of APVS/IVS patient with the combination of the absent pulmonary valve leaflets, intact ventricular septum and superior vena cava who develop a surprisingly long-term survival and underwent timely pulmonary valve implantation surgery.

In this case, we reported a 24-day-old infant with a rare combination of the anomalous origin of coronary artery and the type A interrupted aortic arch, who presented with significant shortness of breath. Besides these anomalies, further cardiac examination including transthoracic echocardiography and CT angiogram revealed ventricular septum defect, patent foramen ovale and patent ductus arteriosus at the same time. Surgical procedures including repair of ventricular septum defect, ligation of patent ductus arteriosus, unroofing of right coronary artery and end-to-side aortic anastomosis were performed to cure the infant. This patient stayed in ICU for few days without any major complications and discharged from hospital at the tenth day after surgery.