Absent pulmonary valve

Synonyms and Related Keywords: Absent pulmonary valve syndrome, APVS, Congenital absence of the pulmonary valve, Pulmonary valve agenesis

Absent pulmonary valve is a rare congenital heart defect. This anomaly is a combination of poorly formed pulmonary valve tissue and significant pulmonary valve insufficiency. This may cause pulmonary artery dilatation. Massive dilatation of pulmonary arteries may lead to extrinsic compression of bronchial airway.

Possible associated abnormalities include;


 * Abnormal tricuspid valve
 * Atrial septal defect
 * Double outlet right ventricle
 * Ductus arteriosis
 * Endocardial cushion defect
 * Marfan's syndrome
 * Tricuspid atresia
 * Ventricular septal defect

Diagnosis
Symptoms can vary depending on associated defects, and may include:


 * Cough
 * Cyanosis
 * Respiratory infections
 * Tachypnea
 * Wheezing

Diagnostic Tests

 * Electrocardiogram
 * Chest x-ray
 * Echocardiogram
 * Computed tomography of the heart
 * Magnetic resonance imaging (MRI) of the heart

Treatment
Infants who have respiratory symptoms should have surgery as soon as possible. Others without symptoms should have surgery within the first 3-6 months of life.

Complications

 * Atelectasis
 * Brain abscess
 * Pneumonia
 * Right-sided heart failure
 * Stroke