Rusovici Arthur, Ibrahim Samia, Sood Sunita, Maher James, Gerula Christine, Kaluski Edo, Klapholz Marc
Division of Cardiology, Department of Medicine, University of Medicine & Dentistry of New Jersey, New Jersey Medical School, Newark, New Jersey 07103, USA.
Tex Heart Inst J. 2012;39(2):281-3.
During a cardiac evaluation prior to liver transplantation, a 51-year-old man with hepatitis C and cirrhosis was found to have nonischemic cardiomyopathy-a condition that would have made him ineligible for liver transplantation. Right ventricular biopsy revealed extensive cardiac hemosiderosis. Despite the elevated levels of serum ferritin, the patient had no history of multiple red blood cell transfusions; moreover, genetic testing for hereditary hemochromatosis was negative for the HFE mutations C282Y and H63D. Chelation therapy was considered for this patient, to reduce the cardiac iron deposits. However, before a course of treatment was established, the patient's clinical condition worsened, and chelation therapy was no longer feasible. He was referred for combined heart and liver transplantation. Cardiac iron deposition can be diagnosed readily using right ventricular biopsy or T2* magnetic resonance imaging. Early detection may allow time for intensive chelation therapy, which might, in turn, reverse the myopathic process. Improved cardiac function should improve cirrhosis patients' chances to be placed on the liver transplant waiting list and ultimately optimize transplantation outcomes.
在肝移植前的心脏评估中，一名患有丙型肝炎和肝硬化的51岁男性被发现患有非缺血性心肌病——这种情况会使他不符合肝移植条件。右心室活检显示广泛的心脏铁沉积。尽管血清铁蛋白水平升高，但该患者无多次红细胞输血史；此外，遗传性血色素沉着症的基因检测显示HFE突变C282Y和H63D为阴性。考虑对该患者进行螯合疗法，以减少心脏铁沉积。然而，在确定治疗方案之前，患者的临床状况恶化，螯合疗法不再可行。他被转诊进行心脏和肝脏联合移植。使用右心室活检或T2*磁共振成像可以很容易地诊断心脏铁沉积。早期检测可能为强化螯合疗法争取时间，而螯合疗法反过来可能逆转肌病进程。改善心脏功能应能提高肝硬化患者进入肝移植等待名单的机会，并最终优化移植结果。
