American Journal of Perinatology Reports (Nov 2012)

Absent Pulmonary Valve Syndrome with Left Hemitruncus

  • Ashraf M. Aly,
  • Deborah A. Reid,
  • Amyn K. Jiwani,
  • Pooja H. Desai

DOI
https://doi.org/10.1055/s-0032-1316464
Journal volume & issue
Vol. 02, no. 01
pp. 051 – 054

Abstract

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Absent pulmonary valve syndrome (APVS) is a rare congenital heart defect, usually associated with tetralogy of Fallot (TOF), although other associations have been described. A pregnant woman was referred to fetal echocardiography clinic from the Maternal Fetal Medicine department due to abnormal findings on routine antenatal ultrasound, showing a pulsatile cystic mass above the left atrium and a suspected TOF. A fetal echocardiogram confirmed the presence of TOF/APVS. The pulsatile cystic mass was the aneurysmally dilated main pulmonary artery. The exact origin of the left pulmonary artery (LPA) was not clearly established prenatally. A postnatal echocardiogram of the neonate showed an abnormal origin of the LPA from the ascending aorta (hemitruncus). The neonate subsequently underwent surgical repair with a good outcome. We present a novel case of a TOF/APVS associated with an abnormal origin of the LPA from the ascending aorta.

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