Isolated congenital pulmonary regurgitation with right ventricular outflow tract aneurysm--a rare variant of Uhl's anomaly.
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.
Vaidyanathan
Karthik
K
Department of Pediatric Cardiac Surgery, Dr KM Cherian Heart Foundation, Chennai, India. rkvdoc@rediffmail.com
Agarwal
Ravi
R
Johari
Raghav
R
Subramanian
Raghavan
R
Cherian
Kotturathu Mammen
KM
D002675:Child, Preschool; D006322:Heart Aneurysm / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D006352:Heart Ventricles / Q000002:abnormalities / Q000473:pathology* / Q000601:surgery; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D011664:Pulmonary Valve / Q000002:abnormalities / Q000000981:diagnostic imaging / Q000473:pathology; D011665:Pulmonary Valve Insufficiency / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D011666:Pulmonary Valve Stenosis / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D013577:Syndrome; D014463:Ultrasonography; D014694:Ventricular Outflow Obstruction / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery