[Association between dysplastic development of ventricular wall and primary cardiomyopathy: pathomorphological observations on 92 hearts from transplant recipients.].

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.

[Pathologic features of arrhythmogenic right ventricular cardiomyopathy with severe heart failure].

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.

[Clinicopathologic analysis of dilated heart in cardiac transplant recipients].

OBJECTIVE: To study the pathologic features of dilated heart in cardiac transplant recipients, with clinicoradiologic correlation. METHODS: Sixty recipient hearts from cardiac transplantation performed in Fuwai Hospital were analyzed by gross examination, histologic observation and electron microscopy. Clinicoradiologic correlation was available in 40 cases. RESULTS: Amongst the 40 cases of dilated heart, 52.5% (21/40) were due to dilated cardiomyopathy, 22.5% (9/40) due to arrhythmogenic right ventricular cardiomyopathy, 15.0% (6/40) due to ischemic cardiomyopathy, and the remaining 10.0% (4/40) due to miscellaneous causes, including local noncompaction of ventricular myocardium, giant cell myocarditis, alcoholic cardiomyopathy and hypertensive cardiomyopathy. The discrepancy rate between clinical and pathologic diagnosis was 37.5% (15/40). The erroneous categories included arrhythmogenic right ventricular cardiomyopathy (7 cases), ischemic cardiomyopathy (5 cases), and giant cell myocarditis (1 case), which were all mistaken clinically as dilated cardiomyopathy. While ischemic cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy, noncompaction of ventricular myocardium and giant cell myocarditis have distinctive pathologic features, the diagnosis of alcoholic and hypertensive cardiomyopathies required clinicopathologic correlation. Dilated cardiomyopathy due to viral myocarditis was not identified in the cases studied. CONCLUSION: Pathologic examination is essential in analysis of transplant recipient heart and helps to rectify clinical diagnostic discrepancy.

[Clinicopathologic studies of 11 cases of primary cardiac valve tumors].

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.

[The clinicopathological endomyocardial biopsy analysis in cardiac transplant recipients due to end-stage heart failure].

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.