Atresia literarily means closure or absent of something. Pulmonary atresia, therefore, means closure, absent or underdevelopment of the pulmonary valves. Normally, the pulmonary Artery & Valve carries deoxygenated (oxygen-deprived) blood from the body to the Lungs for oxygenation. In P.A the valve is blocked and cannot send blood for oxygenation in the lungs, therefore deoxygenated blood is in the circulation, and the manifestation of that is a bluish discolouration of the body.
Physiologically, the body in order to get oxygenation finds another route. The Ductus arteriosus which closes immediately after birthday can remain open to allow blood to enter the lungs for oxygenation or another hole VSD exists.
Pulmonary Atresia might result from undeveloped Right Ventricle, the Valve may be totally absent or cannot open.
Pulmonary Atresia with VSD: this is similar to Tetralogy of Fallot
Pulmonary Atresia with intact ventricular Septum
Signs and symptoms
- Fast breathing
- Poor growth
- Bluish discolouration of the body lips, fingers and nail bed,
- Cool calmly skin
- Pulse oximetry
- Chest X-ray
- Cardiac catheterization
Treatment depends on patient presentation and severity which are in stages. There are stages of treatment
- Balloon Valvuloplasty: this is done in the cath lab within the first few hours of life. A catheter is passed through one of the big arteries of the body (usually through the thigh) to the heart. Then a balloon is used to open up the valve.
- BT shunt: the first stage of the corrective surgery. Here, a small pipe is used to create a pathway for blood to go from the arterial circulation to the lungs. This is needed to increase pulmonary blood flow for duct dependent Cyanotic heart disease until they can have a definitive surgery.
Good news: treatment is available in Nigeria.