動脈管開存症，末梢性肺動脈狭窄症，大動脈弁狭窄症など多彩な先天異常を合併した1症例

A rare case of complicated congenital heart disease:PDA, AS and peripheral PS congenital mental retardation and others

　今回我々は心血管系をはじめとする多彩な先天異常を合併したきわめて稀な1症例を経験したので，文献的考察を加えて報告する。

A twenty-year-old woman who was diagnosedas congenital heart disease at birth, was admittedbecause of palpitation and shortness of breath.The blood pressure was 118/60 mmHg, the pulse92/m.(irregular) and the respiration 32/m.Agrade 4 systolic murmur was present along theupPer left sternal border and at the aortic area,that was radiated bilaterally to the neck.Another grade 2 cotinuous murmur was presentat the inner portion of the left scapula withoutradiation. Examination of the lungs disclosedno rales. Edema and cyanosis were not present.She had "elfin face", dental abnormalities, higharched palate and scoliosis of lumbar spine.Mental retardation was present.

Electrocardiogram and vectorcardiogram show-ed bi-ventricular hypertrophy, left atrial overloadand frequent premature ventricular contractions.X-ray films of the chest demonstrated lungcogestion and cardiac enlargement;cardiothoracicratio was 62%. Lung perfusion scintigramsrevealed marked hypoperfusion of the left lung(especially upperfield). Right heart catheter-ization demonstrated high degree of PulmonaryHypertension, PA pressure 95/60 mmHg, andPDA with left to right shunt of 76%.