A 53-year-old woman was referred for ventricular fibrillation with resuscitation. A CT-angiography showed signs of a right ventricular enlargement without obvious cause. A cardiac MRI demonstrated a dilated and hypokinetic right ventricle with extensive late gadolinium enhancement. Arrhythmogenic right ventricular dysplasia (ARVD) was suspected according to the "revised ARVD task force criteria". An endomyocardial biopsy was inconclusive. The patient developed purulent pericarditis after epicardial ablation therapy and died of toxic shock syndrome. The post-mortem pathologic examination demonstrated sarcoidosis involving the heart, lungs, and thyroid gland.
Arrhythmogenic Right Ventricular Dysplasia/*diagnosis ; Female ; Heart Ventricles/pathology ; Humans ; Lung/pathology ; Magnetic Resonance Imaging ; Middle Aged ; Myocardium/pathology ; Sarcoidosis/*diagnosis ; Thyroid Gland/pathology ; Ventricular Fibrillation/diagnosis/*etiology

Postmortem diagnosis of early myocardial infarction is still a puzzling problem in forensic pathology practice, especially in sudden cardiac death. This study was undertaken to evaluate the distribution patterns of several immunohistochemical markers in various types of sudden cardiac death; 27 cases of obstructive coronary atherosclerosis (SCD-CAD) including 13 cases of critical coronary atherosclerosis, 6 cases of atherosclerotic heart disease and 8 cases of acute myocardial infarction, 26 cases of sudden cardiac death with obvious other causes (SCD-miscellaneous) including hypertensive heart disease, arrhythmogenic right ventricular cardiomyopathy, etc. and 14 cases of sudden cardiac death without any myocardial changes and/or minimal coronary atherosclerosis(SCDunknown). 10 cases of unnatural death were included in control group. Imnunohistochemical reactivity and the severity of reactivity loss were evaluated. The immunoreactivity against myoglobin and troponin C were excellent, and their reactivity loss were statistically significant in SCD-CAD compared to control group, SCD-unknown and SCD-miscellaneous (p<0.05). There were increasing pattern of loss of immunoreactivity in SCD-unknown and SCD-miscellaneous compared to control group. The pattern of expression and the severity of reactivity loss against vascular endothelial growth factor(VEGF) and basic fibroblast growth factor (bFGF) were with no differences in experimental group and control group. Immunohistochemistry may be partially useful in determination of early myocyte necrosis in sudden cardiac death, and myoglobin and troponin C may be better. But, its practical application in forensic pathology may be still limited.
Arrhythmogenic Right Ventricular Dysplasia ; Coronary Artery Disease ; Death, Sudden, Cardiac* ; Diagnosis ; Fibroblast Growth Factor 2 ; Forensic Pathology ; Heart Diseases ; Immunohistochemistry* ; Muscle Cells ; Myocardial Infarction ; Myoglobin ; Necrosis ; Troponin C

OBJECTIVETo evaluate the diagnostic value of magnetic resonance imaging (MRI) for arrhythmogenic right ventricular cardiomyopathy (ARVC).

METHODSMRI 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.

RESULTSFat 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.

Adolescent ; Adult ; Aged ; Arrhythmogenic Right Ventricular Dysplasia ; diagnosis ; Female ; Fibrosis ; Heart Ventricles ; pathology ; Humans ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Prognosis ; Young Adult

Arrhythmogenic Right Ventricular Dysplasia ; complications ; Autopsy ; Cardiomyopathy, Hypertrophic ; complications ; Cause of Death ; Coronary Disease ; complications ; Coronary Vessel Anomalies ; complications ; Death, Sudden, Cardiac ; etiology ; pathology ; Humans

OBJECTIVESTo study the pathologic features of arrhythmogenic right ventricular cardiomyopathy (ARVC) in the phase of heart failure.

METHODSEight 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.

RESULTSOf 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.

CONCLUSIONSLeft 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.

Adipose Tissue ; pathology ; Adolescent ; Adult ; Arrhythmias, Cardiac ; Arrhythmogenic Right Ventricular Dysplasia ; complications ; pathology ; physiopathology ; Cardiomyopathy, Dilated ; etiology ; Female ; Fibrosis ; etiology ; Heart Failure ; complications ; Humans ; Male ; Middle Aged ; Myocarditis ; etiology ; pathology ; Myocardium ; pathology ; Young Adult

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a heart muscle disease of unknown etiology characterized by fatty or fibrofatty infiltration of the right ventricular myocardium. It is well known that ARVC is one of the important causes of sudden death in young individuals. The significant pathological change of ARVC is atrophy and fibrofatty myocardial replacement of the right ventricular free wall. According to the histopathological substrate ARVC could be divided into 2 types: fatty and fibrofatty myocardial replacement. Possible etiology including: (1) apoptosis or programmed cell death; (2) inflammatory; (3) myocardium dysplasia and (4) myocyte transdifferentiation theory.
Adipose Tissue/pathology* ; Adolescent ; Adult ; Apoptosis ; Arrhythmias, Cardiac/etiology* ; Arrhythmogenic Right Ventricular Dysplasia/pathology* ; Child ; Child, Preschool ; Death, Sudden, Cardiac/etiology* ; Female ; Forensic Medicine ; Humans ; Infant ; Male ; Middle Aged

OBJECTIVETo study the pathologic features of dilated heart in cardiac transplant recipients, with clinicoradiologic correlation.

METHODSSixty 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.

RESULTSAmongst 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.

CONCLUSIONPathologic examination is essential in analysis of transplant recipient heart and helps to rectify clinical diagnostic discrepancy.

Adolescent ; Adult ; Arrhythmogenic Right Ventricular Dysplasia ; diagnosis ; pathology ; Cardiomyopathy, Alcoholic ; diagnosis ; pathology ; Cardiomyopathy, Dilated ; diagnosis ; pathology ; Diagnostic Errors ; Dilatation, Pathologic ; diagnosis ; pathology ; Female ; Giant Cells ; pathology ; Heart Transplantation ; pathology ; Humans ; Hypertension ; complications ; Male ; Middle Aged ; Myocardial Ischemia ; diagnosis ; pathology ; Myocardium ; pathology