Hsi David H, Ryan Gerald F, Hellems Steven O, Cheeran David C, Sheils Lucy A
Department of Medicine, Park Ridge Hospital, Rochester, NY, USA.
Mayo Clin Proc. 2003 Jun;78(6):774-6. doi: 10.4065/78.6.774.
We describe a 50-year-old man with a history of hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu disease) who presented with chest pain, atrial fibrillation, and congestive heart failure. Echocardiography revealed a large ascending aortic aneurysm accompanied by severe aortic regurgitation and giant coronary artery aneurysms involving the right, left main, left anterior descending, and circumflex coronary arteries. Coronary angiography clearly defined multiple aneurysms involving the aorta and coronary arteries. The patient underwent complex and successful surgical repair of the aneurysms. To our knowledge, this is the first reported case of extensive cardiac involvement in a patient with this uncommon genetic disorder.
我们描述了一名50岁男性,有遗传性出血性毛细血管扩张症(奥斯勒-韦伯-伦杜病)病史,出现胸痛、心房颤动和充血性心力衰竭。超声心动图显示一个巨大的升主动脉瘤,伴有严重主动脉瓣反流以及累及右冠状动脉、左主干、左前降支和回旋支冠状动脉的巨大冠状动脉瘤。冠状动脉造影清晰地显示了累及主动脉和冠状动脉的多个动脉瘤。该患者接受了复杂且成功的动脉瘤手术修复。据我们所知,这是首例报道的患有这种罕见遗传疾病的患者出现广泛心脏受累的病例。