Pulmonary circulation in alpha1-antitrypsin deficiency investigated by cinedensigraphy

The series consisted of eight cases of severe serum alpha1-antitrypsin deficiency (Piz homozygosity) and eight lung patients with a heterozygous level of the compound. The regional blood pulsation pattern in the lungs in the recumbent position was compared with the pattern in a group of normal controls using a cinedensigraphic device specially constructed for lung circulation studies. In the homozygous group, pulsation in the lower part of the lungs was significantly weakened to the benefit of the apical part. Individual variations occurred. The disturbance observed was not altered with oxygen breathing. This phenomenon is analogous to the redistribution of the blood flow demonstrated by scintigraphic methods. The heterozygous group did not differ significantly from the normal controls with regard to regional pulsation pattern.