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.