Results: Of the 204 patients, 6 (3%) had undergone surgical correction for Tetralogy of Fallot with absent pulmonary valve syndrome. All 6(100%) patients underwent complete repair.
Keywords: Absent pulmonary valve syndrome, Contegra valved conduit, Pulmonary valve replacement, Trans- annular patch.
Absent Pulmonary Valve Syndrome (APVS) is a rare variant and makes up about 3-6% of patients of Tetralogy of Fallot (ToF).1 Apart from the hallmark features of ToF, these patients have an absent/rudimentary pulmonary valve, leading to pulmonary regurgitation during foetal life, causing aneurysmal dilatation of pulmonary vessels, compressing the trachea or the main stem bronchi, and leading to tracheomalacia and bronchomalacia.
PA: Pulmonary Atresia, VSD: Ventricular Septal Defect, MAPCAS: Main Aortopulmonary Collaterals, DORV: Double Oulet Right Ventricle, PS: Pulmonary Stenosis, d TGA: Transposition of Great vessels, TOF- APVS: Tetrology Of Fallot with Absent Pulmonary Valve Syndrome
. RMBT: Right Modified Blallock Tausing Shunt, PDA: Patent Ductus Arteriosus, AS: Aortic Stenosis.