心不全を契機に発見された大動脈肺動脈中隔欠損症の成人例

An Adult Case of Aortopulmonary Window Which Was Found After an Episode of Heart Failure

　症例は46歳の女性で，小児期より心雑音を指摘されていたが放置していた．二度の妊娠歴があるが心不全に至らず，健常児を分娩している．46歳になって，労作時呼吸困難と起座呼吸を自覚し，当科に入院した．心臓超音波検査にて大動脈と肺動脈の間に欠損孔を認め，この欠損孔を通る大動脈から肺動脈へのシャント血流が確認された．心臓カテーテル検査にて著明な肺動脈圧の上昇と，肺動脈主幹部にて酸素飽和度のステップアップを認めた．大動脈造影にて肺動脈主幹部が造影された．以上より大動脈肺動脈中隔欠損症と診断した．本症は先天性心疾患の0.1〜0.2％にすぎない稀な疾患で，通常は大量の左右シャントのため小児期までに発見される．本例は更年期になって症状が出現し，初めて診断に至った点が貴重と考え，若干の文献的考察を加えて報告する．

The patient was a 46-year-old woman with heart murmur which had been discovered in her childhood. She went through two pregnancies and delivered with no manifestation of heart failure. She was admitted to our hospital due to orthopnea. Echocardiography documented an aortopulmonary window with left-right shunt flow. Cardiac catheterization revealed a remark-able elevation of pulmonary artery pressure and a step-up of O₂ saturation between the right ventricule and the pulmonary artery. The aortography showed opacifi-cation of the main pulmonary artery. Thus, she was diagnosed as aortopulmonary window. Case reports of aortopulmonary window over the age of forty such as this present case are very rare.