Absent Pulmonary Valve Associated With Tetralogy of Fallot and Double Chamber Right Ventricle

Tetralogy of Fallot (TOF) in the absent of pulmonary valve is a rare congenital anomaly (3%of TOF patients). We are presenting an 18- year-old male with history of dyspnea on exertion and cyanosis since childhood. Despite the diagnosis of congenital heart disease, no intervention was done due to his poor socioeconomic culture. At age of 18, he referred to our center for further evaluation of heart problem. After physical examination and other diagnostic tests, our final diagnosis was tetralogy of fallot, absent pulmonary valve and double-chamber right ventricle. The absence of mature PV tissue leads to severe pulmonary regurgitation, which is often associated with significant dilatation of the pulmonary arteries. After further evaluation of main pulmonary artery, its branches and other abnormalities of the heart, total correction of tetralogy and pulmonary valve replacement was done. His post operation period was uneventful without any complication and he discharged after 10 days. We believe that the prognosis for those patients in this era is good and complete surgical treatment is tolerated well by them.