Nineteen cases who had pulmonary with MAPCA, diagnosed at Seoul National University Children's Hospital from October 1987 to May 1989, were studied to lool at the relationship between MAPCA, central pulmonary artery and segmental pulmonary arteries. Also was observed the clinical course and operative management.

The average number of MACPA in each patient was 4, two toward right lung and the other two toward left lung. MACPA arose most frequently from descending aorta and innominate artery contralateral to the side of aortic arch. Central pulmonary artery was identified in 86.7%. The number of bronchopulmonary segment connected to central pulmonary artery directly or indrectly was very variable so was the number of bronchopulmonary segment connected to MAPCA only.

Three had a total correction and 14 had a various kinds of palliative operation once or twice. Seven had a called "unifocalization"(connection of MAPCA to central pulmonary artery) to correct arborization abnormality but the result was not satisfactory for technical reason.