Absent pulmonary valve
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
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. [1] [2] [3]
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
Diagnosis
Symptoms can vary depending on associated defects, and may include:
Diagnostic Tests
- Electrocardiogram
- Chest x-ray
- Echocardiogram
- Computed tomography of the heart
- Magnetic resonance imaging (MRI) of the heart
Treatment
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.
References
References
- ↑ Park, MK. Park: Pediatric Cardiology for Practitioners. 5th ed. Philadelphia, Pa: Mosby; 2008.
- ↑ Nlke L, Azakie A, Anagnostopoulos PV, Alphonso N, Karl TR. The Lecompte maneuver for relief of airway compression in absent pulmonary valve syndrome. Ann Thorac Surg, 2006;81:1802-1807.
- ↑ Brown JW, Ruzmetov M, Vijay P, Rodefeld MD, Turrentine MW. Surgical treatment of absent pulmonary valve syndrome associated with bronchial obstruction. Ann Thoracic Surg, 2006; 82: 2221-2226.
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