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Pulmonary vascular disease in children with congenital heart defects living at high altitude—a quantitative morphometric lung biopsy study

Published online by Cambridge University Press:  19 August 2008

Seshadri Balaji ,
Alison A. Hislop ,
Solomon E. Levin  and
Sheila G. Haworth
Seshadri Balaji
Affiliation:
From the Vascular Biology & Pharmacology Unit, The Institute of Child Health, London and the University of Witwatersrand, Johannesburg
Alison A. Hislop
Affiliation:
From the Vascular Biology & Pharmacology Unit, The Institute of Child Health, London and the University of Witwatersrand, Johannesburg
Solomon E. Levin
Affiliation:
From the Vascular Biology & Pharmacology Unit, The Institute of Child Health, London and the University of Witwatersrand, Johannesburg
Sheila G. Haworth*
Affiliation:
From the Vascular Biology & Pharmacology Unit, The Institute of Child Health, London and the University of Witwatersrand, Johannesburg
*
Correspondence to Professor Sheila G. Haworth, Vascular Biology & Pharmacology Unit, The Institute of Child Health, 30 Guilford Street, London WC1N 1 EH, United Kingdom. Tel. 071 242 9789 ext. 5902; Fax. 071 831 0488.
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Summary

Lung biopsies were taken from 30 children aged three months to 15 years (median, 11 months) who had pulmonary hypertensive congenital heart disease and were living at an altitude of 1750 meters. They had either a ventricular septal defect and/or patency of the arterial duct, atrioventricular septal defect or complete transposition with a ventricular septal defect. Biopsies were studied using quantitative morphometric light microscopic techniques. All patients with a ventricular septal defect with or without patency of the arterial duct showed a significant increase in mean percentage arterial medial thickness of both pre- and intraacinar pulmonary arteries compared with those of normal children of similar age living at sea level (p<0.001 for both pre- and intraacinar vessels) and with children with a ventricular septal defect living at sea level (p<0.001 for both pre- and intraacinar vessels). Extension of muscle to more peripheral pulmonary arteries was also greater. Intimal proliferation and fibrosis was seen in 10 patients, in three of whom it was severe. Intimal proliferation occurred more frequently than in children with a ventricular septal defect living at sea level. The findings were similar in patients with atrioventricular septal defect and complete transposition with ventricular septal defect. These findings suggest that patients with congenital heart disease who live at a relatively high altitude develop pulmonary vascular disease more rapidly than do those living at sea level.

Keywords

Pulmonary vascular diseasehigh altitudecongenital heart disease
Type
Original Articles
Information
Cardiology in the Young , Volume 3 , Issue 2 , April 1993 , pp. 104 - 110
Copyright
Copyright © Cambridge University Press 1993

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References

1.Penaloza, D, Arias-Stella, J, Sime, F, Recavarren, S, Marticorena, E. The heart and pulmonary circulation in children at high altitudes. Physiological, anatomical and clinical observations. Pediatrics 1964; 34: 568–582.CrossRefGoogle ScholarPubMed
2.Arias-Stella, J, Recavarren, S. Right ventricular hypertrophy in native children living at high altitude. Am J Pathol 1962; 41:55–64.Google ScholarPubMed
3.Sime, F, Banchero, N, Penaloza, D, Gamboa, R, Cruz, J, Marticorena, E. Pulmonary hypertension in children born and living at high altitudes. Am J Cardiol 1963; 11: 143–149.CrossRefGoogle ScholarPubMed
4.Haworth, SG, Hislop, AA. Pulmonary vascular development: Normal values of peripheral vascular structure. Am J Cardiol 1983; 55: 578–583.CrossRefGoogle Scholar
5.Haworth, SG. Pulmonary vascular disease in ventricular septal defect: Structural and functional correlations in lung biopsies from 85 patients, with outcome of intracardiac repair. J Pathol 1987; 152: 157–168.CrossRefGoogle ScholarPubMed
6.Haworth, SG. Pulmonary vascular bed in children with complete atrioventricular septal defect: Relation between structural and hemodynamic abnormalities. Am J Cardiol 1986; 57: 833–839.CrossRefGoogle ScholarPubMed
7.Haworth, SG, Radley-Smith, R, Yacoub, M. Lung biopsy findings in transposition of the great arteries with ventricular septal defect: Potentially reversible pulmonary vascular disease is not always synonymous with operability. J Am Coll Cardiol 1987; 9: 327–333.CrossRefGoogle Scholar
8.Emery, JL, Mithal, A. The number of alveoli in the terminal respiratory unit of man during late intrauterine life and childhood. Arch Dis Child 1960; 35: 544–547.CrossRefGoogle ScholarPubMed
9.Cooney, TP.Thurlbeck, WM. Pulmonary hypoplasia in Down's syndrome. N Eng J Med 1982; 307: 1170–1178.CrossRefGoogle ScholarPubMed
10.Arias-Stella, J, Saldana, M. The terminal portion of the pulmonary arterial tree in people native to high altitudes. Circulation 1963; 28: 915–925.CrossRefGoogle ScholarPubMed
11.Heath, D, Smith, P, Rioz, Dalenz J, Williams, D, Harris, P. Small pulmonary arteries in some natives of La Paz, Bolivia. Thorax 1981; 36: 599–604.CrossRefGoogle ScholarPubMed
12.Meyrick, B, Reid, L. Normal postnatal development of the media of the rat hilar pulmonary artery and its remodelling by chronic hypoxia. Lab Invest 1982; 46: 505–514.Google ScholarPubMed
13.Haworth, SG, Hislop, AA. Effect of hypoxia on adaptation of the pulmonary circulation to extra-uterine life in the pig. Cardiovasc Res 1982; 16: 293–303.CrossRefGoogle ScholarPubMed
14.Wagenvoort, CA, Wagenvoort, N. Pathology of Pulmonary Hypertension. John Wiley & Sons, Inc., New York, 1977, p. 238.Google Scholar
15.Meyrick, B, Reid, L. The effect of continued hypoxia on rat pulmonary arterial circulation. An ultrastructural study. Lab Invest 1978; 38: 188–200.Google ScholarPubMed
16.Ferencz, C. Transposition of the great vessels. Pathophysi-ologic considerations based on a study of the lungs. Circulation 1966; 33: 232–241.CrossRefGoogle ScholarPubMed
17.Neufeld, EA, Paul, MH, Muster, AJ, Idriss, FS. Pulmonary vascular disease in complete transposition of the great arteries: a study of 200 patients. Am J Cardiol 1974; 34: 75–82.CrossRefGoogle Scholar
18.Harris, P, Heath, D. The Human Pulmonary Circulation. Third Edition. Churchill Livingstone, Edinburgh, 1986, pp 504.Google Scholar
19.Hall, SM, Haworth, SG. Onset and evolution of pulmonary vascular disease in young children: abnormal postnatal remodelling studied in lung biopsies. J Pathol 1992; 166: 183–194.CrossRefGoogle ScholarPubMed