In 1868, Franzel reported a case of absence of the right Pulmonary artery, associated
with multiple congenital cardiac anomalies as an incidental autopsy findings, and in 1952
Madoff and associates reported the first clinically diagnosed case, of a 14 year old
female and this case was confirmed by angiocardiography.
In 1956, Emmanuel and Pattison were to count a total of 46 such cases, most of which
had been added becauses of newly refined angiocardiographic techniques.
Previous reviews showed that, in general, absence of the right pulmonary artery
appeared as an isolated defect, but absence of the left pulmonary artery was associated
with several major abnomalities of the heart and great vessels.
A case of isolated congenital absence of the right pulmonary artery, diagnozed clinically
by angiocardiography, is presented with review.
Possible embryogenesis, radiographic and angiocardiographic appearance of this
condition is reviewed.
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