Pulmonary atresia is a congenital malformation of the pulmonary valve in which the valve orifice fails to develop. The valve is completely closed thereby obstructing the outflow of blood from the heart to the lungs. Babies with this type of cyanotic congenital heart disease survive only for the first few days of life while the normal fetal shunts between left and right circulations remain patent. Without an operation in that period to open the pulmonary valve or to make a shunt between the aorta and the pulmonary arteries, the condition is fatal. The type of surgery recommended depends on the size of the right ventricle and the pulmonary artery. If they are normal in size and the right ventricle is able to pump blood, open heart surgery can be performed to make blood flow through the heart in a normal pattern. If the right ventricle is small and unable to act as a pump, doctors may perform an operation called the Fontan procedure. In this procedure, the right atrium is connected directly to the pulmonary artery. Many children with Pulmonary Atresia will go on to lead 'normal' lives. A congenital disorder is a medical condition or defect that is present at or before birth (for example, congenital heart disease). ... Congenital heart disease is heart disease in the newborn, and includes congenital heart defects, congenital arrythmias, and cardiomyopathies. ... The largest artery in the human body, the aorta originates from the left ventricle of the heart and brings oxygenated blood to all parts of the body in the systemic circulation. ... The pulmonary arteries carry blood from the heart to the lungs. ... In the heart, a ventricle is a chamber which collects blood from an atrium (another heart chamber) and pumps it out of the heart. ... The Fontan procedure is a surgical procedure that involves diversion of venous blood from the right atrium to the pulmonary arteries without passing through the morphologic right ventricle. ... This page is about the muscular organ, the Heart. ...
Categories: Congenital heart disease | Medicine stubs
Pulmonaryatresia with intact ventricular septum is a complex cardiac lesion that has been extensively described in the cardiovascular literature, and is characterized by one of two patterns of pathophysiology.
and is characterized by atresia of the pulmonary valve with an intact ventricular septum.
The pulmonary lesion in this disease is that of an atretic pulmonary valve apparatus.
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