When an ASD is present in pulmonary atresia blood will pass from the right atrium to the left atrium with the mixture of the venous and arteria blood moving towards the peripheral circulation. The mixed blood will move toward the left ventricle and the aorta. Then, a portion of the blood will continue along the aorta and a portion will move through the PDA, enter the pulmonary artery and then the lungs. The size of the PDA becomes very important with regards to the clinical manifestations of cyanosis and clubbing, where the increased size will decrease both manifestations. Surgical and Medical Inteventions Medical treatment is geared toward the treatment or prevention of bacterial endocarditis, cyanosis, and ischemia to the brain.
Surgical treatment depends upon the development of the right ventricle.
If the RV is hypoplastic with a normal pulmonary artery the surgical procedure is usually directed towards increasing pulmonary blood flow. If the main pulmonary artery is hypoplastic, the Fontan procedure is an effective method of repair.