Respiratory distress. Absent pulmonary valve syndrome is a rare form of congenital heart disease. There are some features that are similar to tetralogy of fallot. Absent pulmonary valve syndrome is different than pulmonary valve atresia. Corrective surgery is required. Typically, the dominant feature early is respiratory distress due to compression of the trachea and bronchi by enlarged pulmonary arteries.
Cyanosis. This is an extreme type of tetralogy of fallot (tof with pulmonary valve atresia). It results in intense cyanosis and possibly respiratory distress, usually within the first 1-2 days of life when the ductus arteriosus closes. These babies require special medicine after birth (pge1) and surgical intervention directed at providing a reliable source of pulmonary blood flow.
Rare defect. Absent pulmonary valve syndrome is a rare congenital heart defect. Some of the features are similar to tetralogy of fallot. An important difference in most babies with absent pulmonary valve syndrome is that the pulmonary arteries are very large, and often associated with problems with the airways. Surgery is required to repair the problems in the heart and repair the pulmonary arteries. Read more...