OVERVIEW
Pulmonary arteriosynostosis (PAA) is a relatively rare congenital malformation that includes several types, such as absence of the main pulmonary artery, absence of the proximal right or left pulmonary artery, anomalous origin of the left pulmonary artery, and narrowing of the pulmonary artery, and is often associated with other cardiovascular malformations such as tetralogy of Fallot.
Etiology
Absence of the main pulmonary artery is characterized by atresia or residual fibrous cords of the pulmonary artery segments, whereas the right and left pulmonary arteries are in a normal position and are connected to the aorta. Proximal absence of the right or left pulmonary artery is characterized by atresia of the vessel lumen, with the blood supply to the lung tissue originating from the bronchial artery or an abnormal branch of the aorta, with ipsilateral pulmonary hypoplasia. Anomalous origin of the left pulmonary artery from the right side often forms a sling around the trachea or right main bronchus, causing compression and obstruction. Narrowing of the pulmonary arteries occurs as single or multiple stenoses of varying degrees of severity, often accompanied by vasodilatation distal to the stenosis and other cardiovascular malformations.
Symptoms
The absence of the main pulmonary arteries is usually associated with stillbirth or death in infancy from pulmonary hypertension. Hemoptysis and dyspnea are common symptoms of absence of the proximal part of the right or left pulmonary artery. Anomalous origin of the left pulmonary artery is characterized by tracheal and esophageal obstruction, such as stridor, dyspnea, vomiting, dysphagia, and recurrent respiratory infections.
Examination
1. Chest X-ray
Chest X-ray may show increased translucency of the affected lung field and small size of the pulmonary hilum.
2. Lung Ventilation Perfusion Scan
Ventilation without perfusion may be detected on the affected side.
3. Cardiac catheterization
A cardiac catheterization may reveal dysplastic pulmonary arteries by angiography.
4. Echocardiography
It can detect pulmonary hypertension and other malformations of the heart.
5. Enhanced CT and MRI examination
It can detect abnormalities in the morphology of pulmonary blood vessels and help to clarify the diagnosis.
Diagnosis
Diagnosis can be made on the basis of medical history and the results of ancillary tests.
Differential diagnosis
Clinically, it should be differentiated from pulmonary heart disease and congenital heart disease.
Treatment
Surgical treatment is chosen according to the condition of the patient.