Several patients with severe pulmonary hypertension associated with patent ductus arteriosus have been described in the past few years by Johnson et al. (1950), Campbell and Hudson (1951), Bothwell et al. (1952), Dammann and Sell (1952), Cosh (1953), Hultgren et al. (1953), and Smith (1954), and these reports suggest that pulmonary hypertension may be a primary abnormality and not secondary to an increased pulmonary blood flow from a shunt through the patent ductus arteriosus. The view, that severe pulmonary hypertension is not a direct complication of patent ductus arteriosus, receives some support from Brown's (1950) observation that cyanosis does not usually occur in the natural history of the lesion, while experience of patients with patent ductus arteriosus associated with severe pulmonary hypertension suggests that it is justifiable to regard this combination as a specific entity, which must be considered in the differential diagnosis of any patient presenting signs of pulmonary hypertension, irrespective of other auscultatory signs or the presence or absence of cyanosis. The features of eight patients with severe pulmonary hypertension and patent ductus arteriosus are described below and an account of the pathological findings is given in two who died. The clinical manifestations of this combination are defined and its diagnosis by cardiac catheterization and angiocardiography is discussed.
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