40例心脏移植术后受体心脏病理诊断与术前临床诊断的对比分析

目的通过比较40例心脏移植前、后诊断的异同来评价终末期心力衰竭患者术前诊断的准确性。方法40例患者术前全部经过询问病史,体格检查,心电图、超声心动图检查。凡有心绞痛症状,超声心动图证实存在节段性室壁运动异常或存在明确的危险因素等条件之一,且病情允许的患者在术前作冠状动脉造影。若≥1支冠状动脉主支的横断面面积减少75％以上被认为是有意义的冠状动脉病变。术前诊断为冠心病的患者全部行双核素检查,评价存活心肌。所有心脏移植术取下的心脏均被称重,测量室壁厚度,并经大体检查和组织学检查。结果心脏移植术前诊断为原发性扩张型心肌病（IDC）占45．0％,酒精性心肌病17．5％,冠心病占17．5％,肥厚型心肌病7．5％。高血压病、瓣膜病、致心律失常性右室心肌病、克山病和心肌致密化不全各1例,各占2．5％。术前诊断为冠心病者,受体心脏均有严重冠状动脉病变。术前诊断为IDC的18例和酒精性心肌病的7例患者,受体心脏病理检查共有8例（占32％）可见严重的冠状动脉病变。术前诊断为高血压心脏病和主动脉瓣换瓣术后心力衰竭的患者各1例,也可见冠状动脉严重病变。术前诊断为IDC和克山病者,6例受体心脏病理诊断为致心律失常性右室心肌病。术前诊断为IDC者1例,在移植取下心脏后被病理诊断为巨细胞心肌炎。结论早期正确诊断心力衰竭的病因,可决定患者不同的治疗方法（血管重建而不是心脏移植）,且能判断移植患者的预后。本研究的结果提示：无论临床表现如何,所有怀疑IDC的患者均应尽早作冠状动脉造影,并尽可能查明其病因。

Comparison between pre- and post-transplant diagnosis of end-stage dilated cardiomyopathy

OBJECTIVE: To evaluate the discrepancy between pre- and post-transplant diagnosis of end-stage dilated cardiomyopathy, a pre-transplantation diagnosis was compared with the diagnosis made after macroscopic and microscopic examination of the explanted hearts in 40 cardiac transplant recipients who had undergone cardiac transplantation at our institute. METHODS: Pre-operation echocardiograms were obtained in all patients and coronary angiogram was obtained in 9 patients who had significant risk factors for coronary heart disease (CHD). CHD was considered present when there was a 75% reduction in cross-sectional luminal area of >or= 1 major coronary artery. Idiopathic dilated cardiomyopathy (IDC) was diagnosed when ventricular dilation and global reduction in ventricular systolic function were present in the absence of any identifiable cause. IDC patients with an alcohol consumption of > 100 g/day during the last 12 months before the onset of congestive heart failure were classified as having alcoholic cardiomyopathy. The pathological diagnosis of arrhythmogenic right ventricular cardiomyopathy was formulated in the presence of gross/or histological evidence of regional or diffuse transmural fatty or fibrofatty infiltration of the right ventricular free wall. RESULTS: Before transplantation, 45.0%, 17.5%, 17.5% and 7.5% of patients were classified as IDC, CHD, alcoholic cardiomyopathy and hypertrophic cardiomyopathy. Post-transplant CHD diagnosis was made in all patients with a pre-transplant diagnosis of CHD. Post-transplant CHD diagnosis was also established in 4 patients with a pre-transplant diagnosis of IDC, in 4 patients with presumptive alcoholic cardiomyopathy, in 1 patient with hypertensive cardiomyopathy and in 1 patient with a pre-transplant diagnosis of aortic valve disease. Post-transplant arrhythmogenic right ventricular cardiomyopathy diagnosis was made in 6 patients with a pre-transplant diagnosis of IDC or KaShan disease. Post-transplant giant cell myocarditis diagnosis was made in 1 patient with a pre-transplant diagnosis of IDC. CONCLUSION: Post-transplant CHD diagnosis is significantly higher than that of pre-transplant (42.5% vs. 17.5%, P < 0.05). Part of these patients might benefit from bypass surgery or PCI. Therefore, "in-depth" search for a heart failure cause, especially the coronary angiography examination, should be conducted in all heart transplantation candidates due to heart failure, regardless of their clinical presentation.