Sudden Intrapartum Fetal Death in Fetuses with Absent Pulmonary Valve Syndrome: Report of Two Cases

Abstract

Objective To describe potential intrapartum complications for fetuses affected by absent pulmonary valve syndrome.

Study Design Two cases of intrapartum fetal death at full term were collected from our institution's labor and delivery unit records.

Results In both cases of intrapartum fetal death, the fetuses had been diagnosed with absent pulmonary valve syndrome and had likely experienced acute cardiac events during labor. Both were delivered as stillbirths despite emergency cesarean delivery.

Conclusion Patients should be counseled prior to labor about potential intrapartum complications for a fetus with absent pulmonary valve syndrome. Plans for fetal monitoring and the extent of aggressive intervention should be in place before labor in case sudden complications occur.

Keywords

absent pulmonary valve syndrome - intrapartum fetal demise - high-risk pregnancy

• References

• 1 Emmanoulides GC, Thanopoulos B, Siassi B, Fishbein M. “Agenesis” of ductus arteriosus associated with the syndrome of tetralogy of Fallot and absent pulmonary valve. Am J Cardiol 1976; 37: 403-409
• 2 Galindo A, Gutiérrez-Larraya F, Martínez JM , et al. Prenatal diagnosis and outcome for fetuses with congenital absence of the pulmonary valve. Ultrasound Obstet Gynecol 2006; 28: 32-39
• 3 Becker R, Schmitz L, Guschmann M, Wegner RD, Stiemer B, Entezami M. Prenatal diagnosis of familial absent pulmonary valve syndrome: case report and review of the literature. Ultrasound Obstet Gynecol 2001; 17: 263-267
• 4 Berg C, Thomsen Y, Geipel A, Germer U, Gembruch U. Reversed end-diastolic flow in the umbilical artery at 10–14 weeks of gestation is associated with absent pulmonary valve syndrome. Ultrasound Obstet Gynecol 2007; 30: 254-258
• 5 Abu-Rustum RS, Daou L, Abu-Rustum SE. Role of ultrasonography in early gestation in the diagnosis of congenital heart defects. J Ultrasound Med 2010; 29: 817-821
• 6 Joshi AN, Rane HS, Kamble RC, Mestry PJ, Maniar H, Shah Y. Prenatal diagnosis of absent pulmonary valve syndrome: report of 2 cases, most common and most rare presentations. J Ultrasound Med 2010; 29: 823-829
• 7 Moon-Grady AJ, Tacy TA, Brook MM, Hanley FL, Silverman NH. Value of clinical and echocardiographic features in predicting outcome in the fetus, infant, and child with tetralogy of Fallot with absent pulmonary valve complex. Am J Cardiol 2002; 89: 1280-1285
• 8 Zucker N, Rozin I, Levitas A, Zalzstein E. Clinical presentation, natural history, and outcome of patients with the absent pulmonary valve syndrome. Cardiol Young 2004; 14: 402-408
• 9 Litovsky S, Choy M, Park J , et al. Absent pulmonary valve with tricuspid atresia or severe tricuspid stenosis: report of three cases and review of the literature. Pediatr Dev Pathol 2000; 3: 353-366
• 10 Hartge D, Hoffmann U, Schröer A, Weichert J. Three- and four-dimensional ultrasound in the diagnosis of fetal tetralogy of fallot with absent pulmonary valve and microdeletion 22q11. Pediatr Cardiol 2010; 31: 1100-1103