1952年uhl氏首次报告了一例右室壁肌层发育不良,右室腔显著扩张的先天性心脏畸形,此后类似病例屡有发现。后来将这种,右室肌层完全或部份缺如,三尖瓣和肺动脉瓣正常的先天性心脏病称uhL氏畸形,又称羊皮纸心。本病极易误诊为Ebscein畸形或其它先天性心脏病,国内文献尚无详 1952 uhl’s first report of a case of dysplasia of the right wall muscle layer, the right ventricular cavity was significantly enlarged congenital heart disease, since then found similar cases. Later this kind of, right ventricular muscle completely or partially missing, tricuspid and pulmonary valve normal congenital heart disease called uhL’s deformity, also known as parchment paper heart. The disease can easily be misdiagnosed as Ebscein deformity or other congenital heart disease, the domestic literature is still not detailed