2Department of Maternal Fetal Medicine, Tufts Medical Center,

Absent pulmonary valve syndrome (APVS) is a rare congenital condition of uncertain etiology often associated with tetralogy of Fallot (TOF). First described by Cheevers in 1847, malforma-tions include an absent or dysplastic pulmonary valve, annular pulmonary stenosis with regurgitation, and aneurysmatic di-lation of the main pulmonary artery. Historically, the majority of cases have been diagnosed postnatally as a result of dilated pulmonary arteries compressing bronchial airways, with a risk of perinatal death as high as 60%.1,2 Few studies have followed the antenatal course of fetuses affected by APVS, and no study to date has described intrapartum fetal death. Case Reports Our first case was a nulliparous 16-year-old woman with abnormal fetal cardiac findings on an 18-week ultrasound. Fetal echocardiogram at 20 weeks visualized a nubbin-lik