Pulmonary Valve Atresia is a medical condition in which the pulmonary valve, responsible for regulating blood flow between the heart and lungs, is completely blocked. The word "pulmonary" is pronounced /ˈpʊlmənəri/ and refers to the lungs, while "valve" is pronounced /vælv/ and refers to the mechanism responsible for controlling blood flow. "Atresia" is pronounced /əˈtriʒə/ and refers to the obstruction of an opening in the body. Treatment for pulmonary valve atresia often involves surgery to repair or replace the valve.
Pulmonary valve atresia is a congenital heart defect characterized by the complete blockage or absence of the pulmonary valve, which is responsible for regulating blood flow from the right ventricle of the heart to the lungs. In individuals with this condition, the pulmonary valve does not form properly during fetal development, resulting in a tiny, malformed, or closed valve.
The absence of a functional pulmonary valve causes significant obstruction to the blood flow from the right ventricle to the pulmonary artery and subsequently to the lungs. This obstruction prevents oxygen-poor blood from entering the lungs to receive oxygen and be properly oxygenated before being pumped throughout the body. As a consequence, affected individuals have reduced circulation of oxygenated blood, leading to severe cyanosis (bluish discoloration of the skin and mucous membranes).
The management of pulmonary valve atresia varies based on the severity and unique characteristics of each case. Treatment options may include medication to maintain an open ductus arteriosus (a temporary vessel connecting the pulmonary artery and the aorta during fetal development), surgical interventions such as a shunt procedure to improve blood flow to the lungs, or more complex procedures like open-heart surgery to create a new pathway for blood flow.
Long-term management typically involves ongoing monitoring, regular check-ups, and potential additional interventions or surgeries as the individual grows and develops. Many individuals with pulmonary valve atresia may require lifelong medical care and follow-up to manage the condition and its potential complications effectively.