Cardiac amyloidosis from the pathologist's perspective

Amyloidosis is a systemic, yet an uncommon and probably underdiagnosed disorder which may have cardiac manifestations drawing the cardiologist's attention to its existence. [1-3] Endomycardial biopsy material is then referred to the pathology laboratory, mainly as a means to establish or confirm the clinical diagnosis. Although the disease has four major types of clinical presentation, the histopathological findings are the same. Microscopically, amyloid is deposited in different parts of the heart such as the interstices of the myofibers to endocardium, blood vessel walls, valve structures, and epicardial fat. The SA and AV nodes as well as the bundle branches are also shown to be involved by the deposits. Based on the routine staining methods, amyloid is pale pink in color and looks homogeneous, distributing in the subendocardial tissue and interstitial myocardium thus producing myocyte compression and atrophy. Conventionally, the Congo red staining method reveals the characteristic amyloid material under the polarized light, where the apple-green birefringence is noted. Alcian blue stain, crystal violet, and thioflavin-T fluorescence are also employed to demonstarate amyloidosis in biopsy samples [1-2]

Long sheaths for endomyocardial biopsy via femoral approach

Endomyocardial biopsy has long been a useful tool to evaluate myocardial diseases. It is a gold standard in diagnosing cardiac rejection and certain types of myocarditis, but there is always a risk for related complications. We have assessed the safety and feasibility of applying long sheaths in endomyocardial biopsy via the femoral approach. We performed endomyocardial biopsy in 63 patients via the femoral approach and used a long sheath to guide bioptome from the venous access to the interventricular septum and evaluate the procedure success and complications. Success rate [acceptable endomyocardial specimen] was 96%, and there were no complications [death, no death, urgent cardiac surgery, advanced cardiac life support, hemothorax, and pneumothorax]. Using long sheaths for the femoral approach seems to be safe and feasible; it confers an acceptable room to grab the endomyocardial samples while obviating the possibility of complications

Different histopathological appearances of cardiac tuberculosis: presentation of a rare case with review of literature

Tuberculosis is caused by a mycobacterium and is a multipotential, omnipresent infectious disease. Generally, two major categories, pulmonary and extrapulmonary exist. Whereas pericardial involvement is relatively common, myocardial tuberculosis has been reported in not more than 0.3% of all tuberculosis patients post-mortem. A 21-year-old woman sought medical attention due to progressive dyspnea upon exertion, weight loss, and general weakness. She underwent a heart operation and a well-circumscribed, solid, and creamy egg-shaped mass with a lobulated surface was seen in the right atrium. The histopathological study showed multiple well-formed granulomas with multinucleated giant cells and extensive caseous necrosis. These were compatible with a diagnosis of tuberculoma; however, the Ziehl-Neelsen stain failed to reveal the acid-fast bacilli. Her postoperative course was uneventful and soon she was discharged with advice to continue her medical therapy

Hydatid cyst mimicking heart tumor

We present two women who lived in a rural community. The presence of a semi-solid mass, a hydatid cyst or tumor, in the heart was diagnosed by echocardiography, computed tomography, and Magnetic Resonance Imaging. The hydatid cyst was seen during surgery. Pathological examination confirmed an infected hydatid cyst

What do we know about the cardiac CAT? [a case report]

We present a 16-year-old male whose chief complaints were exertional dyspnea, atypical chest pain, and a weight loss of about 5 kg of recent onset. His past medical history and laboratory tests were unremarkable. Echocardiography showed a calcified and mobile mass with irregular borders, measuring 3 X 2 cm in the left atrium. Grossly, the specimen was creamy-yellow and fragmented with a firm to hard consistency. Microscopically, multiple calcium deposits were seen within a dense background of fibrocollagenous tissue. Based on these findings, a diagnosis of cardiac calcified amorphous tumor [CAT] was made

Malignant melanoma in the heart, with focus on the histopathological diagnosis: review of our two cases in a five-year period

Over a five-year period, we encountered two cases of malignant melanoma with metastasis to the heart and pericardium. Both patients had a relevant medical history, showing their previous involvement by this tumor. A high index of suspicion, simply provided by a precise clinical history, together with histopathological and cytological studies can be used to diagnose such patients in due course. The preliminary diagnosis is made by echocardiography, by which the tumoral masses are seen in different parts of the heart. Nevertheless, their primary or secondary origin as well as the histogenesis can only be ascertained by pathological studies. Routine staining methods are useftil in the demonstration of malignant cells in the tissue or pericardial fluid samples

Fungal endocarditis: review of our cases over a four-year period

Over a 4-year period, we studied six cases of fungal endocarditis, four of which were caused by Aspergillus sp. And the other two were candidal in origin. Both sides of the heart showed involvement by these infections. The vegetations of infective endocarditis tend to vary in size, but fungal lesions are often large. Our youngest patient was four months old and the oldest one was 62 years old. A high index of clinical suspicion, together with histopathological and microbiological studies, can be used as tools to diagnose and treat such patient in due course. It is important to send fresh specimens for tissue culture studies in sterile normal saline and not in fixatives such as formaladehyde. Pathologically, not only are the special staining methods such as Periodic acid-Schiff useful in the demonstration of fungal elements in tissue or vegetation sections, but also the routine hematoxylin and eosin stain is capable of showing these structures clearly

18-year-old man with cardiomegaly and pericardial effusion

We present an 18-year-old male who sought medical attention due to exertional dyspnea of a few weeks' duration. His physical exam revealed an elevated jugular venous pressure, facial puffiness, muffled heart sounds, and mild lower extremity pitting edema. Chest X-ray showed cardiomegaly; and in echocardiography, huge intrapericardial masses with massive pericardial effusion were noted. The oinly noteworthy finding on abdominal ultrasonography and CT scan was the presene of ascites. The only abnormal laboratory results consisted of a hemoglobin level of 10.8 g/dl, a 1-hr ESR 77, CRP 34 mg/dl and LDH 771. Some 1500cc pericardial fluid was aspirated, and tow multilobated creamy-brown masses with foci of necrosis and hemorrhage were excised. Microscopically, hypercellular sheets of malignant round cells were seen. Based on morphology, a diagnosis of high-grade round cell sarcoma was made. Immunohistochemical markers were negative for cytokeratin, CD 34, desmin, and smooth muscle actin, while positive reactivity was noted only for vimentin. Therefore, the cells were mesenchymal in origin with no vascular, skeletal, or smooth muscle differentiation and the final diagnosis was undifferentiated sarcoma. The patient was discharged in good clinical condition and underwent chemoradiation therapy

Intraluminal ascending aorta fibroma

Primary cardiac tumors are quite rare, especially in the pediatric age group, and their atypical presentation often prevent a timely diagnosis. Most primary cardiac tumors in the pediatric age group are benign. Fibromas are generally reported as the second most common primary cardiac tumors in the pediatric age group. These neoplasms are often intramural and involve the left ventricular free wall or the interventricular septum. Although benign, fibromas may become life-threatening by causing arrhythmias or obstruction to the blood flow. A case of supravalvular intraluminal ascending aorta fibroma in a 23-month-old girl, presenting with syncope, is described here; the location is rare and the presentation atypical for this type of tumor. Transesophageal echocardiography helped us to evaluate the anatomic details of the tumor and plan surgery