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Congenital absence of the pulmonary valve

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Abstract

Congenital absence of the pumonary valve (CAPV) is a rare anomaly usually associated with ventricular septal defect or Fallot's tetralogy. CAPV may be rapidly fatal in infancy because of severe disturbance of pulmonary ventilation resulting from bronchial compression by massively dilated pulmonary arteries. Other cases may be relatively benign. Our group of five cases with CAPV ranged from the severe to one case in which there was documented closure of a patent ductus artierosus and a ventricular septal defect, leaving the CAPV as an apparently isolated, well-tolerated lesion. Plain chest radiographs play a major part in the diagnosis of CAPV since they almost invariably give some indication of the aneurysmal dilatation of pulmonary arteries. On angiography, dilated main pulmonary arteries are shown giving rise to branches of normal or even diminished caliber.

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Authors and Affiliations

  1. Department of Pediatric Cardiology, University of Virginia Medical Center, Charlottesyille, Virginia, USA

    Marius M. Hubbell Jr., Karen S. Rheuban, Dorothy G. Tompkins & Martha A. Carpenter

  2. Department of Radiology, University of Virginia Medical Center, Box 170, 22908, Charlottesville, VA, USA

    Paul M. Dee M.D.

Authors
  1. Paul M. Dee M.D.
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  2. Marius M. Hubbell Jr.
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  3. Karen S. Rheuban
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  4. Dorothy G. Tompkins
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  5. Martha A. Carpenter
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Dee, P.M., Hubbell, M.M., Rheuban, K.S. et al. Congenital absence of the pulmonary valve. Cardiovasc Intervent Radiol 4, 158–166 (1981). https://doi.org/10.1007/BF02552417

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