THE PATTERNS OF PULMONARY CIRCULATION IN PULMONARY ATRESIA

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Abstract
The syndromes associated with pulmonary atresia are determined by the presence or absence of significant ventricular septal defect and by the extent and route of pulmonary blood flow. Patients in whom pulmonary atresia is associated with ventricular septal defect may have a small pulmonary flow precariously dependent on a patent ductus, or a larger, sometimes increased, pulmonary flow through bronchial or anomalous systemic arteries which may anastomose directly with hypoplastic pulmonary arteries. The prognosis in the latter group is usually regarded as good, but the occurrence in infancy of left ventricular failure from excessive pulmonary flow is now described. Patients with isolated pulmonary atresia, in whom pulmonary flow is again precariously dependent on duct patency, have a poor prognosis, with severe hypoxia associated at times with right ventricular failure. These patients may be divided into 2 groups according to the size of the right ventricular cavity. The relation of organic tricuspid regurgitation to right ventricular dilatation is discussed further, and the suggestion is made that impaired ventricular filling due to a small tricuspid orifice may be of importance in patients with small right ventricular cavities.