Absent pulmonary valve syndrome with left hemitruncus.
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.
Division of Pediatric Cardiology, Department of Pediatrics, University of Texas Medical Branch-Galveston, Galveston, Texas.
Ashraf M
AM
Aly
Deborah A
DA
Reid
Amyn K
AK
Jiwani
Pooja H
PH
Desai
absent pulmonary valve; fetal echocardiography; left hemitruncus; tetralogy of Fallot