Skip to main content
NCBI home page
British Heart Journal logoLink to British Heart Journal
. 1984 Nov;52(5):557–571. doi: 10.1136/hrt.52.5.557

Pulmonary vascular disease in different types of congenital heart disease. Implications for interpretation of lung biopsy findings in early childhood.

S G Haworth
PMCID: PMC481681  PMID: 6498033

Abstract

Pulmonary vascular structure was studied by analysing serial reconstructions of the arterial pathways and random sections of tissue in the lungs of 16 children who died with different types of congenital heart disease and pulmonary hypertension. Cases of ventricular septal defect showed an appreciable increase in muscularity of both preacinar and intra-acinar (respiratory unit) arteries, and intimal proliferation was infrequent and mild. By contrast, cases of transposition of the great arteries with ventricular septal defect and atrioventricular septal defect showed an increase in preacinar muscularity, a short heavily muscularised arterial segment containing intimal proliferation at the entrance to the acinus, whereas the intra-acinar arteries beyond showed only a moderate increase in muscularity. In these children who were less than 1 year of age an increase in pulmonary vascular resistance was due to strategically placed small areas of intimal proliferation and not to widespread obliterative pulmonary vascular disease. The study demonstrated and explained differences in the appearance of the peripheral pulmonary arteries in different types of congenital heart disease, which help interpret the findings of lung biopsies.

Full text

PDF
557

Images in this article

562Image
on p.562
565Image
on p.565
565Image
on p.565
567Image
on p.567

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Davies G., Reid L. Growth of the alveoli and pulmonary arteries in childhood. Thorax. 1970 Nov;25(6):669–681. doi: 10.1136/thx.25.6.669. [DOI] [PMC free article] [PubMed] [Google Scholar]
  2. FRIEDMAN M., BYERS S. O. Experimental thrombo-atherosclerosis. J Clin Invest. 1961 Jul;40:1139–1152. doi: 10.1172/JCI104343. [DOI] [PMC free article] [PubMed] [Google Scholar]
  3. Fry D. L. Acute vascular endothelial changes associated with increased blood velocity gradients. Circ Res. 1968 Feb;22(2):165–197. doi: 10.1161/01.res.22.2.165. [DOI] [PubMed] [Google Scholar]
  4. HEATH D., BEST P. V. The tunica media of the arteries of the lung in pulmonary hypertension. J Pathol Bacteriol. 1958 Jul;76(1):165–174. doi: 10.1002/path.1700760119. [DOI] [PubMed] [Google Scholar]
  5. HEATH D., EDWARDS J. E. The pathology of hypertensive pulmonary vascular disease; a description of six grades of structural changes in the pulmonary arteries with special reference to congenital cardiac septal defects. Circulation. 1958 Oct;18(4 Pt 1):533–547. doi: 10.1161/01.cir.18.4.533. [DOI] [PubMed] [Google Scholar]
  6. Haworth S. G., Hislop A. A. Adaptation of the pulmonary circulation to extra-uterine life in the pig and its relevance to the human infant. Cardiovasc Res. 1981 Feb;15(2):108–119. doi: 10.1093/cvr/15.2.108. [DOI] [PubMed] [Google Scholar]
  7. Haworth S. G., Hislop A. A. Pulmonary vascular development: normal values of peripheral vascular structure. Am J Cardiol. 1983 Sep 1;52(5):578–583. doi: 10.1016/0002-9149(83)90030-9. [DOI] [PubMed] [Google Scholar]
  8. Haworth S. G. Pulmonary vascular disease in secundum atrial septal defect in childhood. Am J Cardiol. 1983 Jan 15;51(2):265–272. doi: 10.1016/s0002-9149(83)80047-2. [DOI] [PubMed] [Google Scholar]
  9. Haworth S. G., Reid L. A morphometric study of regional variation in lung structure in infants with pulmonary hypertension and congenital cardiac defect. A justification of lung biopsy. Br Heart J. 1978 Aug;40(8):825–831. doi: 10.1136/hrt.40.8.825. [DOI] [PMC free article] [PubMed] [Google Scholar]
  10. Haworth S. G., Sauer U., Bũhlmeyer K., Reid L. Development of the pulmonary circulation in ventricular septal defect: a quantitative structural study. Am J Cardiol. 1977 Nov;40(5):781–788. doi: 10.1016/0002-9149(77)90197-7. [DOI] [PubMed] [Google Scholar]
  11. Hislop A., Reid L. Intra-pulmonary arterial development during fetal life-branching pattern and structure. J Anat. 1972 Oct;113(Pt 1):35–48. [PMC free article] [PubMed] [Google Scholar]
  12. Hislop A., Reid L. New pathological findings in emphysema of childhood. 1. Polyalveolar lobe with emphysema. Thorax. 1970 Nov;25(6):682–690. doi: 10.1136/thx.25.6.682. [DOI] [PMC free article] [PubMed] [Google Scholar]
  13. Hislop A., Reid L. Pulmonary arterial development during childhood: branching pattern and structure. Thorax. 1973 Mar;28(2):129–135. doi: 10.1136/thx.28.2.129. [DOI] [PMC free article] [PubMed] [Google Scholar]
  14. Meyrick B., Reid L. The effect of continued hypoxia on rat pulmonary arterial circulation. An ultrastructural study. Lab Invest. 1978 Feb;38(2):188–200. [PubMed] [Google Scholar]
  15. Rabinovitch M., Haworth S. G., Castaneda A. R., Nadas A. S., Reid L. M. Lung biopsy in congenital heart disease: a morphometric approach to pulmonary vascular disease. Circulation. 1978 Dec;58(6):1107–1122. doi: 10.1161/01.cir.58.6.1107. [DOI] [PubMed] [Google Scholar]
  16. Ross R., Glomset J., Kariya B., Harker L. A platelet-dependent serum factor that stimulates the proliferation of arterial smooth muscle cells in vitro. Proc Natl Acad Sci U S A. 1974 Apr;71(4):1207–1210. doi: 10.1073/pnas.71.4.1207. [DOI] [PMC free article] [PubMed] [Google Scholar]
  17. Schroter R. C., Sudlow M. F. Flow patterns in models of the human bronchial airways. Respir Physiol. 1969 Oct;7(3):341–355. doi: 10.1016/0034-5687(69)90018-8. [DOI] [PubMed] [Google Scholar]
  18. Wagenvoort C. A. Grading of pulmonary vascular lesions--a reappraisal. Histopathology. 1981 Nov;5(6):595–598. doi: 10.1111/j.1365-2559.1981.tb01826.x. [DOI] [PubMed] [Google Scholar]
  19. Yamaki S., Wagenvoort C. A. Plexogenic pulmonary arteriopathy: significance of medial thickness with respect to advanced pulmonary vascular lesions. Am J Pathol. 1981 Oct;105(1):70–75. [PMC free article] [PubMed] [Google Scholar]
  20. Zweifach B. W. Quantitative studies of microcirculatory structure and function. I. Analysis of pressure distribution in the terminal vascular bed in cat mesentery. Circ Res. 1974 Jun;34(6):843–857. [PubMed] [Google Scholar]

Articles from British Heart Journal are provided here courtesy of BMJ Publishing Group

RESOURCES