INTRODUCTION
Absent 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.