Isolated congenital absence of a single pulmonary valve cusp associated with pulmonary aneurysm
█ Case report
Hartyánszky István1, Rácz Katalin3, Katona Márta3, Varga Sándor1, Babik Barna2, Sélei Ágnes4, Bogáts Gábor1
Szegedi Tudományegyetem, Szent-Györgyi Albert Klinikai Központ, 1Szívsebészeti Osztály, 2Aneszteziológiai és Intenzivterápiás Osztály, 3Gyermekgyógyászati Klinika, 4Diagnosztika, Szeged
Isolated absence of a single pulmonary valve is extremely unusual. The flow dynamics is very special in this condition resulting in slow progression in clinical findings (significant pulmonary regurgitation, right ventricular enlargement, and dilatation of the main pulmonary artery) hence it is recognised mostly in adolescent and adult age. A 14-year old female was diagnosed with dilated main pulmonary artery, pulmonary incompetence and right ventricular dilatation. At the time of surgery the dilated pulmonary artery and the pulmonary valve (which had normal anterior, a dysplastic right cusp and the left cusp completely absent) were resected and reconstruction was performed using homograft. There were no postoperative complications. The child was seen in the follow-up clinic five months later with normal Echo findings.