Isolated congenital pulmonary regurgitation with right ventricular outflow tract aneurysm--a rare variant of Uhl's anomaly.

Abstract

Isolated absent pulmonary valve syndrome is a very rare entity. We report the case of a four-year-old boy who had congenital absent pulmonary valve with a thin and aneurysmal right ventricular infundibulum. The histological picture was suggestive of Uhl's anomaly. This association of partial right ventricular Uhl's anomaly with absent pulmonary valve syndrome has not been described before. We discuss the embryological and clinical significance of this association.

• Authors: Karthik Vaidyanathan, Ravi Agarwal, Raghav Johari, Raghavan Subramanian, Kotturathu Mammen Cherian
• Journal: Journal of cardiac surgery (J Card Surg) Vol. 25 Issue 4 Pg. 415-7 (Jul 2010) ISSN: 1540-8191 [Electronic] United States
• PMID: 20529159 (Publication Type: Case Reports, Journal Article)

Topics

• Child, Preschool
• Heart Aneurysm (diagnosis, pathology, surgery)
• Heart Ventricles (abnormalities, pathology, surgery)
• Humans
• Magnetic Resonance Imaging
• Male
• Pulmonary Valve (abnormalities, diagnostic imaging, pathology)
• Pulmonary Valve Insufficiency (diagnosis, pathology, surgery)
• Pulmonary Valve Stenosis (diagnosis, pathology, surgery)
• Syndrome
• Ultrasonography
• Ventricular Outflow Obstruction (diagnosis, pathology, surgery)