Timely Surgical Intervention for Absent Pulmonary Valve Syndrome with an Intact Ventricular Septum in a Patient with a Long-Term Survival: A Case Report

Absent pulmonary valve syndrome with an intact ventricular septum (APVS with IVS) is a rare congenital heart defect that is associated with a high perinatal mortality. Most fetuses can hard to survive without inotropic support or heart transplantation due to the severe respiratory dysfunction, heart failure and serious infections resulting from tracheobronchial compression and right ventricular dilation. Almost one-third of the patients opted for a termination of pregnancy. The etiology of APVS patients is still unknown. Previous research only noted an enhancement in the long-term survival of APVS with tetralogy of Fallot patients over the past decade. However, the timeliness of surgical intervention and survival status in APVS with IVS patients remains unclear. In our case, we described a 27-year-old patient with a rare combination of the rudimentary pulmonary valve, intact ventricular septum and absent superior vena cava who successfully developed biventricular physiology without any medical intervene. And he received a timely pulmonary valve implantation in our centre to prevent further right ventricular dysfunction. Thus, we highlighted that biventricular circulation can be successfully achieved in this rare subtype of APVS with IVS patients. However, more multicentre studies and lifelong follow-up are required to determine the timeliness for pulmonary valve surgery to avoid over-dilatation of the right ventricle in APVS with IVS patients.