Tetralogy of Fallot and absent pulmonary valve syndrome

Absent pulmonary valve syndrome is a relatively rare condition, representing a subset of about 3% of cases of tetralogy of Fallot in both autopsy and clinical reviews (1). The syndrome is characterized by a ringlike and usually stenotic malformation rather than by the absence of the pulmonary valve, with failure of development of the valve cusps. Another important feature of absent pulmonary valve syndrome is the marked aneurysmal dilatation of the proximal pulmonary arteries, which causes extrinsic compression of the tracheobronchial tree, leading to respiratory symptoms of variable severity (2). The surgical goals are to correct any intracardiac anomaly, prevent right-sided heart failure, and alleviate or prevent bronchial compression and peripheral lung damage. In this video tutorial, we present the straightforward correction of tetralogy of Fallot with absent pulmonary valve with a transannular patch combined with size reduction arterioplasty of bilateral aneurysmal pulmonary arteries. This patient also had a subaortic membrane that was completely removed concomitantly. Our technique shows right ventricular outflow tract reconstruction without using a valved conduit or creating a monocusp patch in order to reduce the incidence of a right ventricular outflow track reoperation. Nonapplication of the right ventricle-pulmonary artery conduit did not affect the early postoperative course or the immediate postoperative outcome.