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Left Atrial Stenosis Induced Pulmonary Venous Arterialization and Group 2 Pulmonary Hypertension in Rat
Published on: November 18, 2018
Absent pulmonary valve syndrome with left hemitruncus
Ashraf M Aly1, Deborah A Reid, Amyn K Jiwani
1Division of Pediatric Cardiology, Department of Pediatrics, University of Texas Medical Branch-Galveston, Galveston, Texas.
AJP Reports
|August 16, 2013
Summary
This study reports a rare case of absent pulmonary valve syndrome (APVS) in a neonate with tetralogy of Fallot (TOF). The condition involved an abnormal left pulmonary artery origin, successfully treated with surgery.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Fetal Echocardiography
Background:
- Absent pulmonary valve syndrome (APVS) is a rare congenital heart defect.
- It is often associated with tetralogy of Fallot (TOF).
- Prenatal diagnosis can be challenging.
Observation:
- A pregnant woman presented with fetal ultrasound findings suggestive of TOF and a cystic mass.
- Fetal echocardiography confirmed TOF with APVS and a dilated main pulmonary artery.
- The precise origin of the left pulmonary artery (LPA) was unclear prenatally.
Findings:
- Postnatal echocardiography revealed an anomalous origin of the LPA from the ascending aorta (hemitruncus).
- This represents a novel association of TOF/APVS with hemitruncus.
- Surgical repair was performed with a favorable outcome.
Implications:
- Highlights the importance of detailed fetal echocardiography for diagnosing complex congenital heart defects.
- Demonstrates the feasibility of surgical correction for this rare combination.
- Contributes to understanding the spectrum of vascular anomalies in TOF/APVS.
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