Unilateral absence of pulmonary artery: a report on 19 selected clinical cases

Nineteen cases of unilateral absence of the pulmonary artery (UAPA) were found in a group of 2,960 patients investigated consecutively by cardiac catheterization, angiocardiography, and pulmonary perfusion scintigraphy. The incidence of UAPA was 0,6%. In 8 cases (40%) UAPA was found in isolation, and in 11 cases (60%) it was combined with other congenital heart defects (CHD). In 30% of our patients the diagnosis of UAPA was suggested by clinical symptoms and plain chest X-ray. Pulmonary perfusion scan was made in 2,600 children before catheterization. Though it can provide strong evidence of the diagnosis, this should be confirmed by cardiac catheterization and selective angiography. Eight patients were operated upon. Systemic-pulmonary shunt operations were performed in 6, and ligation of PDA in 2 patients. In one of the latter severe hemoptysis occurred following ligation of the PDA. The pathophysiological and diagnostic aspects of UAPA are discussed.