MRI assessment of bronchial compression in absent pulmonary valve syndrome and review of the syndrome
- 205 Downloads
Absent pulmonary valve syndrome (APVS) is a rare cardiac malformation with massive pulmonary insufficiency that presents with short-term and long-term respiratory problems secondary to severe bronchial compression from enlarged central and hilar pulmonary arteries. Association with chromosome 22.Q11 deletions and DiGeorge syndrome is common. This historical review illustrates the airway disease with emphasis on assessment of the bronchial compression in patients with persistent respiratory difficulties post-valvular repair. Cases that had MRI for cardiac assessment are used to illustrate the pattern of airway disease.
KeywordsAbsent pulmonary valve syndrome Airway abnormalities MRI
- 4.Hraska V (2005) Repair of tetralogy of Fallot with absent pulmonary valve using a new approach. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 132–134Google Scholar
- 5.Chevers NA (1847) Collection of facts illustrative of the morbid conditions of the pulmonary artery, as bearing upon the treatment of cardiac and pulmonary diseases. Lond Med Gaz 38:828–835Google Scholar
- 15.Fink AM, Edis B, Massie J (2005) The CT appearances of delayed amniotic fluid clearance from the lungs in an infant with absent pulmonary valve and congenital lobar emphysema. Pediatr Radiol Apr 12 (epub)Google Scholar