Absent pulmonary valve syndrome with associated anomalies of the pulmonary blood supply

Patrizia Presbitero, Ettore Pedretti, Fulvio Orzan, Demetrio Malara, Massimo Villani, Paolo Ferrazzi, GianCarlo Crupi, Lucio Parenzan

Research output: Contribution to journalArticlepeer-review


We report four cases of so-called absent pulmonary valve syndrome associated with absence or anomalous origin of the left pulmonary artery. The fate of the patients with this condition appears to be affected mainly by the occurrence of pulmonary complications (three of our patients died of pulmonary causes, two of them after surgery). The proper timing of corrective surgery is still uncertain. Preoperative investigations should strive to obtain a clear-cut identification of the pulmonary arteries, particularly the left one or, in its absence, of the anomalous vascular supply to the lung. Right ventriculography, as employed by us, is insufficient. Pulmonary angiography, aortography and pulmonary vein "wedge" angiography may be needed.

Original languageEnglish
Pages (from-to)587-596
Number of pages10
JournalInternational Journal of Cardiology
Issue number5
Publication statusPublished - 1984


  • absent pulmonary artery
  • angiocardiography
  • pulmonary branch stenosis

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine


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