Pulmonary atresia overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: Muhammad Waqas, M.D [2]
Overview
Pulmonary atresia is a congenital malformation of the pulmonary valve in which the outflow of the blood from the right side of the heart to the pulmonary artery is obstructed due to the valve orifice fails to develop. In most instances, the condition is almost always fatal without any intervention. Atresia means "no opening". In a normal healthy functioning heart, the valve consists of three flaps that open on ventricular systole leading to emptying of blood from the right ventricle to the pulmonary artery and ultimately to the lungs for proper oxygenation.
This condition often is accompanied by a shunt/foreman Pulmonary atresia with Ventricular septum defect' that transports the Oxygen-poor blood directly from the right ventricle to the left side of the heart through a shunt, often referred to as 'Tetralogy of Fallot with VSD'. This oxygen-poor blood is then pumped through the aorta to the rest of the body, making fingers, toes, and lips appear blue or cyanotic. In Pulmonary atresia with intact Ventricular ventricular septum, another subtype of Pulmonary Atresia, the blood can not flow from the right side to the left side via shunt and due to this reason, the size of the right ventricle is comparatively smaller than the former one. Early intervention is required in these cases and with the immediate intervention, the 5-year survival is 80 percent.
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. [1]
References
- ↑ Dweck MR, Boon NA, Newby DE (2012). "Calcific aortic stenosis: a disease of the valve and the myocardium". J Am Coll Cardiol. 60 (19): 1854–63. doi:10.1016/j.jacc.2012.02.093. PMID 23062541.