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Primary and Secondary Pulmonary Hypertension in Childhood: A Clinicopathological Reappraisal

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Pulmonary Diseases

Part of the book series: Current Topics in Pathology ((CT PATHOLOGY,volume 73))

Abstract

In adults and older children with pulmonary hypertension the sequence of structural changes occurring in the pulmonary circulation and the relation between structural and haemodynamic change is well recognised, following the classical studies of Heath and Edwards (1958). However, in infants and young children this is not so, and the relation between structural and functional change in the immature lung is different from that seen in adult life. Recently, improved understanding of pulmonary hypertension in childhood has resulted from (a) studies on the normal development of the pulmonary circulation; (b) the application of quantitative morphometric techniques to the study of pulmonary vascular structure, an approach which is essential in a growing organ in health or disease; (c) improved clinical care in the perinatal period, bringing to light the problem of failure of the pulmonary circulation to adapt normally to extra-uterine life when the heart is normal; and (d) improved techniques of angiographic and haemo-dynamic evaluation, which permit a more accurate correlation between pulmonary vascular structural and haemodynamic change in children with congenital heart disease.

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Haworth, S.G. (1983). Primary and Secondary Pulmonary Hypertension in Childhood: A Clinicopathological Reappraisal. In: Müller, KM. (eds) Pulmonary Diseases. Current Topics in Pathology, vol 73. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-69134-8_3

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