ABSTRACT
Objective@#To evaluate the sensitivity and specificity of immunohistochemistry (IHC) in the classification of cardiac amyloidosis on endomyocardial biopsy (EMB) and heart allograft.@*Methods@#Twenty cardiac tissues from 19 patients at Fuwai Hospital from January, 1990 to April, 2017 with histopathologic features of amyloidosis and Congo red staining positivity were included. IHC was performed with monoclonal antibodies against AA amyloid and polyclonal antibodies against transthyretin (ATTR), λ-light chain (AL-λ), κ-light chain (AL-κ), ApoAⅠ, ApoAⅡ, ApoA Ⅳ and β2-microglobin. The extent of interstitial staining was evaluated by light microscopy, and three patterns were recognized; these included diffuse pericellular pattern, discrete pericellular pattern, and nodular pattern. Two patterns of vascular deposition were also noted, including arterial pattern and venous pattern. Endocardial involvement was also assessed and recorded.@*Results@#Nineteen cases were divided into three groups according to the pattern of proteins expression in specimens. The first group (5 cases) only showed single protein expression on EMB. The second group (6 cases) showed more than one protein expression, but one of them was intensely stained or any staining of any protein together with ApoA Ⅳ co-staining. The third group (8 cases) also showed more than one protein expression and all of them had intense staining. Amyloid deposits were successfully subtyped as AL-λ, ATTR, AL-κ and ApoAⅠby IHC in the former two groups with the sensitivity of 11/19. In the third group, amyloid deposits could not be subtyped by immunohistochemistry due to their poor specificity. The pericellular pattern tended to favor AL over ATTR amyloidosis and vascular deposition tended to favor ATTR.@*Conclusions@#Amyloid deposits can be reliably subtyped in diagnostic cardiac specimens using IHC. The co-deposition of chaperon proteins, the distribution of amyloid proteins and clinical features are also auxiliary to subtype cardiac amyloidosis.
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Objective@#To evaluate the clinical characteristics and prognosis of patients with mitral valve prolapse (MVP).@*Methods@#We retrospectively analyzed the clinical characteristics and prognosis of 148 MVP patients who underwent mitral valve surgery in Fuwai hospital from January 2012 to December 2015.The patients were divided into mucoid degeneration group (52 cases) and without mucoid degeneration group(19 cases) according to pathological examination of leaflets and chordate.@*Results@#The clinical symptoms of MVP patients included dyspnea (59.5%(88/148)), chest distress and pain (52.7%(78/148)), and palpitations (36.5% (54/148)). Mitral valve repair was performed in 144 cases (97.3%), and mitral valve replacement was performed in 4 cases (2.7%). Posterior leaflet prolapse was the most common form of MVP (68.9%, 102/148). Pathological examination revealed myxomatous degeneration in 73.2% patients (52/71), fibrosis in 8.5% patients (6/71), and fibrinoid necrosis in 8.5% patients (6/71). Patients with mucoid degeneration had less atrial fibrillation before surgery (5.8%(3/52) vs. 42.1%(8/19), P<0.01), smaller preoperative left atrium diameter ((43.2±6.5) mm vs. (48.2±8.9) mm, P<0.05), more posterior leaflet prolapse (94.2%(49/52) vs. 63.2%(12/19), P<0.01), redundant chordae (26.9%(14/52) vs. 0, P<0.05) and leaflet thickening (76.9%(40/52) vs. 52.6%(10/19), P<0.05) when compared with patients without mucoid degeneration.Echocardiography examination at the postoperative follow-up of 39.0(22.3, 57.0) months revealed smaller left atrium diameter((38.5±7.1) mm vs. (45.3±8.3) mm, P<0.01), left ventricular end-diastolic diameter ((48.9±6.2) mm vs. (57.5±7.6) mm, P<0.01), reduced left ventricular ejection fraction ((61.2±7.1)% vs. (65.1±6.2)%, P<0.01) and less moderate or severe mitral regurgitation (1.4%(2/148) vs. 100.0%(148/148), P<0.01) compared with the corresponding preoperative values.@*Conclusions@#Dyspnea is the main symptom, and mucoid degeneration characterized by redundant chordae and leaflet thickening are the main pathological features of MVP patients.The surgical treatment of MVP patients is related with satisfactory outcome results.
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<p><b>OBJECTIVE</b>To study the clinical and pathologic features of primary cardiac inflammatory myofibroblastic tumor.</p><p><b>METHODS</b>A total of 4 patients with primary cardiac inflammatory myofibroblastic tumor were encountered during the period from 1993 to 2013 in National Center for Cardiovascular Disease. The clinical features, imaging findings and outcomes of the 4 patients were evaluated. ALK protein expression and ALK gene status were studied using the archival tumor tissues.</p><p><b>RESULTS</b>There were 1 female and 3 male patients. The age of patients ranged from 5 months to 30 years (mean = 16 years). The tumor was located in right ventricle (n = 2), right atrium (n = 1) or pericardium (n = 1). Histologic patterns included 2 cases of fibrous histiocytoma type, 1 case of granulomatous type and 1 case of sclerosing type. Immunohistochemical study showed that 2 cases expressed ALK protein. Fluorescence in-situ hybridization however did not reveal any ALK gene rearrangement.</p><p><b>CONCLUSIONS</b>Inflammatory myofibroblastic tumor of the heart is rarely encountered and easily misdiagnosed. It carries distinctive clinical and pathologic features. ALK protein expression is helpful in arriving at the correct diagnosis.</p>
Subject(s)
Adolescent , Adult , Child , Female , Humans , Infant , Male , Biomarkers, Tumor , Genetics , Metabolism , Diagnosis, Differential , Granuloma, Plasma Cell , Pathology , Heart Neoplasms , Pathology , Histiocytoma, Benign Fibrous , Pathology , Immunohistochemistry , In Situ Hybridization, Fluorescence , Receptor Protein-Tyrosine Kinases , Genetics , MetabolismABSTRACT
<p><b>OBJECTIVE</b>To investigate the histopathological features of primary restrictive cardiomyopathy (PRCM).</p><p><b>METHODS</b>Nine extransplanted hearts from heart transplantation recipients were examined. Gross and histopathological findings were observed, photographed and final pathological diagnosis was compared to clinical diagnosis. The myocardial ultrastructure changes were determined using transmission electron microscopy.</p><p><b>RESULTS</b>The hallmark pathologic feature of PRCM was distinguished by myocardial cell degeneration and hyperplastic collagen fibrils around the myocardial cells.Fibrosis was severer in left ventricle free wall than in ventricular septum and right ventricle. The degree of myocardial cell degeneration and poloidal disorder were severer in patients with reduced ejection fraction (EF) than in patients with preserved EF. Transmission electron microscope evidenced severe interstitial fibrosis, myofibrillar changes of sarcomere structure, abnormalities both on intercalated disc number and distribution.</p><p><b>CONCLUSIONS</b>PRCM is characterized by hyperplastic collagen fibrils around the cardiomyocytes. Fibrosis is severer in left ventricle than in right ventricle. Sarcomere dysplasia is the main cause of PRCM, and ultrastructural examination is helpful for PRCM diagnosis.</p>