Mechanical valve at pulmonary site in adult TOF & absent pulmonary valve

Abstract

Absent pulmonary valve syndrome (APVS) is a rare congenital heart disease. Tetralogy of Fallot (TOF) with APVS is a rare variation of TOF. These patients are commonly cyanotic at birth. Respiratory complaints predominate due to airway compression by dilated pulmonary arteries. Commonest age of presentation is infancy with anecdotal adult case-reports. Surgical treatment requires establishing unobstructed competent right ventricular outflow tract (RVOT) often with monocusp or conduits. We present a novel technique of rendering RVOT competent by implanting a tilting disc mechanical prosthesis in a rare adult TOF with APVS.