Bertrand G, Charlier P, Touche T, Hazan E, Gourgon R
Arch Mal Coeur Vaiss. 1984 Mar;77(3):349-55.
The case of a 20 year old woman followed up since adolescence for a progressive congestive cardiomyopathy with mitral regurgitation is reported. She was reinvestigated after hospital admission for cardiac decompensation and supraventricular arrhythmias; 2D echocardiography showed signs of severe mitral regurgitation with thickened mitral leaflets prolapsing into a dilated left atrium and, above all, abnormal diastolic wall motion related to restriction of left ventricular filling with no apparent systolic dysfunction : pulsed Doppler studies confirmed the diagnosis of mitral regurgitation but also showed retrograde diastolic flow in the aortic arch (without aortic regurgitation) and in the main pulmonary artery. Angiography showed these disturbances to be due to an abnormal origin of the left coronary artery in the main pulmonary artery. Reimplantation of the left coronary in the aorta and mitral annuloplasty were justified by the poor spontaneous prognosis of this congenital abnormality which has rarely been described in adults.
报告了一名自青春期起就因进行性充血性心肌病伴二尖瓣反流而接受随访的20岁女性病例。她因心脏失代偿和室上性心律失常入院后再次接受检查;二维超声心动图显示严重二尖瓣反流的迹象,二尖瓣叶增厚并脱垂至扩张的左心房,最重要的是,舒张期室壁运动异常与左心室充盈受限有关,无明显收缩功能障碍:脉冲多普勒研究证实了二尖瓣反流的诊断,但也显示主动脉弓(无主动脉反流)和主肺动脉内有舒张期逆向血流。血管造影显示这些异常是由于左冠状动脉起源于主肺动脉异常所致。鉴于这种先天性异常的自然预后较差,且在成人中很少见,因此进行左冠状动脉再植入主动脉和二尖瓣环成形术是合理的。