Pulmonary vascular disease in 57 necropsy cases of total anomalous pulmonary venous connection


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In a semi‐quantitative necropsy study, total anomalous pulmonary venous connection (TAPVC) was an isolated anomaly in 35 cases and was associated with asplenia and either pulmonary stenosis or pulmonary atresia in 22 cases. A comparison of the two groups showed pulmonary venous obstruction (26% vs. 4%), a small interatrial communication (51% vs. 4%), a patent ductus arteriosus or operative shunt (40% vs. 64%), medial hypertrophy of muscular pulmonary arteries (80 vs. 23%), muscularization of arterioles (80% vs. 23%), capillary engorgement (60% vs. 27%) alveolar wall thickening (29% vs. 0%), interlobular septal oedema (34% vs. 14%), dilated lymphatics (80% vs. 45%), arterialization of pulmonary veins (86% vs. 68%), medial hypertrophy of veins (26% vs. 0%), and venous dilatation (74% vs. 50%). Only one patient, a 5‐year‐old girl with isolated TAPVC, had severe plexogenic pulmonary arteriopathy. In the remainder, the pulmonary lesions were those generally associated with reversible pulmonary venous hypertension; they were most striking in patients with pulmonary venous obstruction, and were least prominent in patients with pulmonary stenosis or puimonary atresia.

Original languageEnglish (US)
Pages (from-to)487-496
Number of pages10
Issue number4
StatePublished - Jul 1983


  • lung
  • pulmonary hypertension
  • total anomalous pulmonary venous connection
  • vascular disease

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Histology


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