刘蕾
阜外华中心血管病医院 大血管外科
OBJECTIVES:To study the pathologic features of arrhythmogenic right ventricular cardiomyopathy (ARVC) in the phase of heart failure.METHODS:Eight cases underwent heart transplantation in Fuwai Hospital during the period from May, 2004 to July, 2007 with pathologic diagnosis of ARVC were studied. The age of patients ranged from 15 to 54 years. They had history of palpitation and syncope for 1 to 22 years. Severe heart failure was diagnosed according to the New York Heart Association Classification System. The recipient hearts were examined and the following parameters were evaluated: weight of heart, presence of cardiac dilatation, myocardial hypertrophy, fatty infiltration, fibrosis, parietal thrombosis and myocarditis. The degree of left ventricular involvement was also analyzed.RESULTS:Of the 8 cases studied, 7 cases with prominent right ventricular lesion (fibrofatty replacement) were classified as classic type. One case with prominent left ventricle lesion and mild right ventricle involvement was classified as left predominant type. No biventricular type and no pure fatty infiltration were found. The cases of classic type showed moderate to severe dilatation of right ventricle, sometimes with aneurysm formation. Left ventricle was involved in 6 cases, which showed diffuse interstitial fibrosis, patchy fibrous replacement and subepicardial fatty infiltration. Mild to moderate dilatation of left ventricle, myocardial hypertrophy and vacuolation were also observed in these cases. The case of left predominant type had severe hypertrophy and dilatation of left ventricle, with prominent diffuse interstitial fibrosis and transmural fatty infiltration. Besides, 3 cases showed left ventricular hypertrophy and parietal thrombosis in both ventricles. Focal lymphocytic myocarditis was noted in 1 case.CONCLUSIONS:Left ventricular involvement is common in the heart failure phase of ARVC. Extensive interstitial fibrosis, marked hypertrophy and degeneration of myocardial fibers, as well as severe cardiac dilatation with organized thrombi, represent the major pathologic changes which resembles dilated cardiomyopathy.
Zhonghua bing li xue za zhi = Chinese journal of pathology 2008
OBJECTIVE:To investigate the morphological characteristics and types of ventricular wall with dysplastic development and their associations to primary cardiomyopathy.METHODS:Ninety-two hearts from heart transplant patients were studied soon after explanation from 2004 to 2007. Gross examination/measurement, histopathology and photography were performed.RESULTS:Dysplastic development of ventricular wall could be evidenced in patients with various heart diseases but more often in patients with primary cardiomyopathy, though the extension and distribution of dysplastic development of ventricular wall varied between patients with or without primary cardiomyopathy. Severe dysplastic development of ventricular wall is associated with clinical dysplastic cardiomyopathy. The range of extension and degree of dysplasia in the ventricular wall correlated positively to heart dilation/failure and time point of heart failure development. The incidence of severe ventricular wall dysplasia was 27.17% in all transplanted hearts and was 43.1% (25/58) in hearts diagnosed as primary cardiomyopathy (P < 0.05). The main pathological changes of dysplastic hearts were: (1) extensive proliferative hypertrophy of the heart wall, (2) fibrous/fat or fat/fibrous tissue replacement of normal myocardium, (3) disarrangement of myocardial fibers, (4) dysplastic change in the medium-sized intramural arteries. Dysplastic cardiomyopathy was presented mainly as a combination of several forms of dysplasia. The same clinical manifestations of dysplastic cardiomyopathy patients did not always show the same pathologic changes. Fibrous-fat tissue replacement was commonly found in dilated cardiomyopathy and arrhythmogenic right ventricular cardiomyopathy. Disarrangement of myocardium was often accompanied by hypertrophic cardiomyopathy. Dysplasia of intramural arteries could result in heart dilatation due to myocardial ischemia.CONCLUSION:Dysplasia of ventricular wall is a common variation of heart structure. Only severe or diffuse types of dysplasia is associated with cardiomyopathy, especially primary cardiomyopathy.
Zhonghua xin xue guan bing za zhi 2008
OBJECTIVE:(99)Tc(m) labeled C2A domain of synaptotagmin I ((99)Tc(m)-Syt I-C2A) is used for noninvasive detection of vulnerable atherosclerotic plaque.METHODS:Recombinant C2A domain of synaptotagmin I, overexpressed in E. Coli, was thiolated with 2-iminothiolane (2-IT) and labeled with (99)Tc(m). Atherosclerotic plaques were produced in 5 rabbits by deendothelialization of the abdominal aorta and the rabbits were fed with cholesterol diet for 3 months. Three rabbits not manipulated served as normal controls. All animals were injected with (99)Tc(m)-Syt I-C2A and underwent in vivo imaging thereafter. Aortas were then explanted for ex vivo imaging and histological characterization.RESULTS:In deendothelialized animals, intense radio-uptake in abdominal aorta, showed by gamma camera at 2 h after injection, was visualized and T/B was 3.25 +/- 0.51 by ROI measurement, quantitative uptake ratio of abdominal aortas with atherosclerotic lesions to thoracic aortas was 8.39 +/- 1.74 in ex vivo imaging. The mean uptake in specimens of abdominal aortas with lesions was 12.6-fold higher than in control abdominal aortas, and 10.2-fold higher than in thoracic aortas of deendothelialized animals by gamma-counter.CONCLUSION:(99)Tc(m)-Syt I-C2A has a high affinity for vulnerable atherosclerotic plaque and is a suitable a gent for the noninvasive detection of vulnerable atherosclerotic plaque.
Zhonghua xin xue guan bing za zhi 2007
OBJECTIVE:It is difficult to differentiate the causes of dilated cardiomyopathy only by clinical evaluation and image analysis. Pathomorphologic examinations on diseased hearts may help to improve the diagnosis accuracy.METHODS:Fifty-six extransplanted hearts from June, 2004 to June, 2006 were examined. Gross and histopathological findings were recorded, photographed and final pathological diagnosis was compared to clinical diagnosis.RESULTS:Dilations were caused by sole myocardial wall damage in 38 (67.9%) of the 56 patients, including 19 primary dilated cardiomyopathy, 9 arrhythmogenic right ventricular cardiomyopathy, 1 non-compaction cardiomyopathy, 6 ischemic cardiomyopathy, 1 alcoholic cardiomyopathy, 1 hypertensive cardiomyopathy and 1 giant cell myocarditis. The clinical and pathological diagnoses were different in 15 cases (39.5%). The most discrepancies were arrhythmogenic right ventricular cardiomyopathy (77.8%), ischemic cardiomyopathy (83.3%), and giant cell myocarditis (100%).CONCLUSIONS:This pathological study of recipient hearts showed a high portion of patients with arrhythmogenic right ventricular cardiomyopathy and ischemic cardiomyopathy were misdiagnosed as primary cardiomyopathy. Correct diagnosis of primary cardiomyopathy needs to rule out possible secondary causes of myocardial dilation.
Zhonghua xin xue guan bing za zhi 2007
OBJECTIVE:To evaluate the diagnostic value of magnetic resonance imaging (MRI) for arrhythmogenic right ventricular cardiomyopathy (ARVC).METHODS:MRI was performed in 27 (male 21, mean age: 37.4 y, ranging from 15 - 67 y) clinically diagnosed ARVC patients according to the 1994 ARVC diagnosis criteria of WHO from Oct. 2004 to Jun. 2006. Heart chamber size, fat infiltration, local or global ventricular function, myocardium perfusion of contrast first pass and late enhancement were examined.RESULTS:Fat infiltration was found in 24 (88.89%), trabecular disarray in 17 (62.96%), significant dilated right ventricle outlet (RVOT) in 18 (66.67%), dilated RV apex in 14 (51.85%), dilated RV free wall and posterior wall in 18 (66.67%) and right atrium enlargement in 11 (40.74%) patients. Local RV dysfunction was found in 18.52% (5/27), global RV dysfunction in 70.37% (19/27) of patients with mean RV ejection fraction (EF) of 35%. Left ventricle was affected in 40.74% (11/27) of patients. Perfusion defects were found in only 10.52% (2/19) of patients. Positive late enhancement of myocardium were found in 36.84% (7/19) of patients and affecting mainly the wall of RVOT and the free wall associated with lateral wall enhancement of LV. Five patients received heart transplantation and histology on transplanted hearts confirmed the MRI findings.CONCLUSION:"One-stop-shop" MRI scanning can be used for the diagnosis of ARVC. While for some ARVC cases with dominant abnormality in LV, it is difficult for MRI to differentiate ARVC from dilated cardiomyopathy or coronary heart disease. We found fibrosis of lateral wall of LV can be a characteristic sign of ARVC.
Zhonghua xin xue guan bing za zhi 2006
OBJECTIVE:To study the clinicopathologic features of primary cardiac valve tumors.METHODS:Eleven cases of primary valve tumors collected from Fuwai Hospital during the period from 1983 to 2005 were enrolled into the study. The tumors were stained with hematoxylin and eosin and Weigert-Van Gieson stain. Immunohistochemistry was also carried out in selected examples.RESULTS:Primary cardiac valve tumors were uncommon and accounted for only 3% (11/426) of all primary cardiac tumors. Most of them (10/11) were benign and malignancy was rarely encountered (1/11). The tumor subtypes included papillary fibroelastoma (4/11), cavernous hemangioma (4/11), glomus tumor (1/11), angiosarcoma (1/11) and hamartoma (1/11). Of the 11 tumors studied, 4 involved the tricuspid valve, 4 involved the mitral valve, 2 involved the pulmonary valve and 1 involved the aortic valve. The diagnosis was established by preoperative echocardiography in 7 patients. The remaining 4 cases were either misdiagnosed or undiagnosed.CONCLUSIONS:Preoperative diagnosis of primary cardiac valve tumors can be difficult due to lack of detailed information related to this group of lesions. Although benign cardiac valve tumors carry a good prognosis, the clinical outcome may be disastrous as a result of hemodynamic disturbances. Intraoperative frozen section examination is advisable for guiding proper surgical management.
Zhonghua bing li xue za zhi = Chinese journal of pathology 2006
OBJECTIVE:Endomyocardial biopsies from 42 (35 males and 7 females, aged 43.3 years) heart transplant recipients due to end-stage heart failure between June 2004 and January 2006 in our institute were obtained for pathological studies.METHODS:Sixteen patients underwent 1 endomyocardial biopsy (right ventricular septum) between 13 days to 5 months, 13 patients underwent second biopsy between 1.5 to 8 months and 10 patients underwent third biopsy between 3 to 8.5 months post transplantation. Specimen were stained by hematoxylin-eosin (HE) and Phosphotungstic Acid Hematoxylin (PTAH) and observed under light microscope and cardiac allograft rejection were evaluated according to the Revision of the 1990 working formulation for the standardization of nomenclature in the diagnosis of heart rejection in 2004.RESULTS:The rejection grades were as follows: Grade 0 R in 31 biopsies; Grade 1 R (mild rejection 1990 grade 1A, 1B and 2.) in 30 biopsies; Grade 2 R (moderate rejection, 1990 grade 3A) in 3 biopsies; Grade 1 R cellular rejection companies with humoral rejection in 1 biopsy. Cellular rejection with Quilty effect was found in 2 biopsies. Ischemic myocardial injury presented in 4 biopsies. Quilty effect was observed in 1 biopsy. Cytomegalovirus or toxoplasmic myocarditis was not observed.CONCLUSIONS:Endomyocardial biopsy (EMB) is a valuable diagnostic procedure for rejection surveillance in heart allograft recipients. The observed low rejection incidence and mild rejection from specimens of our heart recipients were comparable to the results of developed countries.
Zhonghua xin xue guan bing za zhi 2006
