Pulmonary Atresia is a congenital heart defect characterized by the underdevelopment or complete closure of the pulmonary valve, which is the valve that controls blood flow from the right ventricle of the heart to the lungs. This condition prevents the normal flow of blood from the heart to the lungs to pick up oxygen. There are two main types of pulmonary atresia, classified based on whether the baby also has a ventricular septal defect (VSD):
Pulmonary Atresia with Intact Ventricular Septum (PA/IVS)
In this type, the pulmonary valve is closed or underdeveloped, and there's no VSD. The right ventricle is often underdeveloped as well, and blood cannot flow into it properly. This can lead to inadequate blood flow to the lungs.
Pulmonary Atresia with Ventricular Septal Defect (PA/VSD)
In this form, there's a VSD in addition to the closed pulmonary valve. The VSD allows some blood to flow from the right ventricle to the left ventricle and then to the body, but it still may not receive enough oxygen.
Symptoms
Symptoms of pulmonary atresia are typically evident soon after birth and can include:
- Cyanosis (a bluish tint to the skin, lips, and fingernails)
- Rapid breathing or shortness of breath
- Fatigue
- Poor feeding and growth
Diagnosis
Diagnosis usually occurs shortly after birth, using tests such as:
- Echocardiogram: To visualize the structure and function of the heart
- Electrocardiogram (ECG): To measure the heart’s electrical activity
- Chest X-ray: To view the heart and lungs
- Cardiac catheterization: To assess the heart's structure, function, and blood flow
Treatment
Treatment for pulmonary atresia depends on the type and severity of the condition and usually involves surgery.
- In the early stages, a medication called prostaglandin E1 may be used to keep the ductus arteriosus (a fetal blood vessel) open, allowing some blood to bypass the lungs.
- For PA/IVS: Treatment often involves multiple surgeries. The initial surgery may include procedures to open the pulmonary valve or to place a shunt between the aorta and pulmonary artery to improve blood flow to the lungs.
- For PA/VSD: Surgery usually involves repairing the VSD and reconstructing the route for blood flow to the lungs, which may include valve replacement or repair and creating a pathway for blood flow from the right ventricle to the pulmonary arteries.
Long-term Outlook
Children with pulmonary atresia require lifelong cardiac care. They may need additional surgeries or interventions as they grow. Regular follow-up with a cardiologist is essential to monitor heart function, detect complications, and manage health over time. Advances in surgical techniques and medical management have significantly improved the prognosis for children with this condition.
