Congenital Absence versus Acquired Attenuation of One Pulmonary Artery

Abstract

tion of a pulmonary artery was the unequivo- cal evidence of its occlusion or atresia not- withstanding Maier's emphasis in 1954 on how closely acquired attenuation of one pul- monary artery could masquerade as true atresia.8The purpose of this presentation is to point out the frequent errors of diagnosis based on this premise and to suggest certain technical refinements aimed at improving diagnostic accuracy.The two cases of actual and one case of apparent absence of one pulmonary artery presented here were chosen from over 35 cases of congenital and acquired unilateral pulmonary artery involvement and serve to illustrate the essential points of distinction. Case ReportsCase 1 This 23-year-old mentally retarded Negro girl, product of a normal pregnancy and delivery, was noted to have a slow physical development when she was 22 years old.A heart murmur was dis- covered at this time.Cyanosis and clubbing of all extremities were noted when she was 8 years of age, but squatting and fainting were never observed.-She was committed to an institution for retarded children at the age of 9 years.There was no history of cough, chesit colds, or hemop- tysis.The striking findings were marked, univer- sally distributed cyanosis and clubbing, small stature, mentality and interests of a 5-year-old girl, and short, stubby fingers.The only audible murmur was a grade III/VI late ejection mur- mur, loudest in the third left interspace para- sternally.The electrocardiogram showed marked right atrial and ventricular hypertrophy.Breath sounds were of normal vesicular quality throughout but were weaker over the right lung.Rales were absent.Chest roentgenogram (fig. 1) showed a cen- trally located heart of normal size and a small right lung with conspicuously absent right main pulmonary artery.There was a lace-like vascular pattern in the parahilar area of the right lung.