Vidhya Nair

CARDIAC TUMOURS: DIAGNOSIS AND MANAGEMENT

Primary cardiac tumours are rare, with an autopsy incidence ranging from 0.001% to 0.030%. Three-quarters of these tumours are benign and nearly half of the benign tumours are myxomas. Metastases to the heart are far more common than primary cardiac tumours. Primary cardiac tumours present with one or more of the symptoms of the classic triad of: cardiac symptoms and signs resulting from intracardiac obstruction; signs of systemic embolisation; and systemic or constitutional symptoms. They are diagnosed by use of transthoracic and transoesophageal echocardiograms, MRI, and CT scan. Whereas surgery is indicated in patients with benign tumours, systemic chemotherapy is indicated in those who have widespread or unresectable malignant disease, and chemotherapy and radiotherapy are usually combined in treatment of patients with primary cardiac lymphomas. The prognosis after surgery is usually excellent in the case of benign tumours but is unfortunately still limited in localised malignant diseases. Patients with sarcomas live for a mean of 3 months to 1 year, and those with lymphomas live up to 5 years if treated, but usually die within 1 month if untreated.

Modes of Failure in Explanted Mitroflow Pericardial Valves

BACKGROUND Bioprosthetic heart valves are a treatment option for patients with significant valvular disease. We present the first morphologic analysis of a series of explanted Mitroflow pericardial valves (model A12) and discuss their modes of failure. METHODS Between 2001 and 2010, 12 Mitroflow (A12) valves (Sorin Group Inc, Vancouver, Canada) were examined, grossly and microscopically, for postimplantation changes. Cusp tears and thickening, calcification (at radiography), pannus formation, inflammation, and thrombus deposition were noted. Microscopically, tissue degeneration including the presence of calcification, pannus formation, thrombus, collagen disruption, fluid insudation, inflammatory cells, and microorganisms were looked for. Causes for dysfunction were then identified. RESULTS There were 12 patients with a mean age of 73.0 ± 5.3 years at implantation. Duration of implantation ranged from 0.25 to 7 years (mean = 2.9 ± 2.5 years). Five valves were explanted for infective endocarditis or thrombotic vegetations. Primary structural deterioration was observed in 7 of 12 (58%) valves; characterized by para-stent post cusp tears (42%) and moderate to severe calcification (50%). Most valves showed cusp thickening and collagen disruption at the stent post and pannus. CONCLUSIONS Structural valve deterioration was observed in 100% of the Mitroflow pericardial valves that had been in place for more than 2 years. The high rate of para-stent post cusp tears, calcification, and tissue degeneration suggests a link to the design, likely associated with mechanical stress, in addition to abrasion with the cross-sutures and Dacron fabric at the stent posts.

Ochronosis and Aortic Valve Stenosis

Abstract  Valvular heart disease has numerous etiologies. These range from congenital malformations to infectious and degenerative diseases. Clinically, these result in significant problems, the management of which can necessitate valve replacement with prosthetic heart valves. A rare cause is the deposition of foreign material in the valvular tissues, and these include inborn errors of metabolism of the essential amino acids. Alkaptonuria, an autosomal recessive trait, can result in the accumulation of excess homogentisic acid. This can manifest as pigmentation in the skin and other tissues, including heart valves. Accumulation of this pigment can lead to an inflammatory reaction and to progressive valve dysfunction.

Carpentier-Edwards Perimount Valves—Morphological Findings in Surgical Explants

Valvular heart disease is a growing medical problem in the 20th century, with an increasing number of patients undergoing valve repair and replacement surgery.1 Worldwide, more than 55% of implanted prosthetic heart valves (PHV) are mechanical heart valves (MHV) and 45% are bioprosthetic heart valves (BHV).2 BHV more closely resemble native valves in design than do mechanical valves, and are, therefore, better able to mimic normal blood flow patterns through a three-cusp valve.1 BHV may comprise porcine aortic tissue or bovine pericardium. The Carpentier-Edwards Perimount (CEP) bioprosthesis is a second-generation pericardial valve made of bovine (calf) pericardial tissue.1 The tissue is cut from selected regions of bovine pericardium and mounted on a lightweight Elgiloy frame covered with a porous, knitted polytetrafluoroethylene (PTFE) fabric. The sewing ring is made of molded silicone rubber covered with PTFE cloth, which enables the surgeon to sew the valve into place. Few studies have examined the postimplantation morphological changes in a series of explanted CEP. Herein, we describe such changes in 23 CEP valves, explanted at our institution since the start of their use in 1981.

Non–Viral-Related Pathologic Findings in Liver Needle Biopsy Specimens From Patients With Chronic Viral Hepatitis

Liver biopsy is a vital part of chronic viral hepatitis management. Pathologists should view these biopsies as screening tools for other liver diseases. We describe our experience in a 6-year period and discuss the pathologists role. Liver biopsies of 1,842 patients with hepatitis B or C, for the 2001-2007 period at the University Health Network, Toronto, Canada, were reviewed; 410 other diagnoses were documented in 377 patients (20.5%; mean age, 25.4 years; range, 15-80 years). These diagnoses included 58 hepatocellular carcinomas and 16 dysplastic nodules, which are recognized complications of chronic viral hepatitis. The remaining findings included the following: steatosis/steatohepatitis, 251; hemosiderosis, 62; granulomatous disease, 7; drug-induced hepatitis, 4; primary biliary cirrhosis, 3; Wilson disease, 2; metastases, 2; and cholangiocarcinoma, atypical lymphoid proliferation, alpha(1)-antitrypsin deficiency, cystic fibrosis, and schistosomiasis, 1 each. Liver biopsies in patients with viral hepatitis revealed other processes with the potential to modify disease progression and/or the management strategy in 20.5% of patients.

Heart transplant biopsies: interpretation and significance

The endomyocardial biopsy(EMB) is a valuable tool that is inadequately utilised, except in monitoring orthotopic, homograft, heart grafts. Performed on a regular, programmed schedule, or on an emergent basis when needed, the EMB is the gold standard for monitoring graft function ( with regard to cellular rejection), often before clinical symptoms develop. This paper addresses these points and discusses handling of tissues and some studies for possible antibody mediated rejection when the lack of morphologic features of cellular rejection does not fit with the patients clinical presentation, days or months after the surgical procedure. In the hands of a skilled operator the EMB procedure is relatively painless and free of signicicant complications.

Papillary fibroelastoma of the interatrial septum: a case report.

Abstract  Primary cardiac tumors are rare, with benign lesions outnumbering malignant ones by a margin of over four to one. Amongst the benign cardiac neoplasms, papillary fibroelastomas are reported second only to myxomas. Papillary fibroelastomas commonly rise from heart valves and their size ranges from a few millimeters to well over 2.0 cm in diameter. While myxomas commonly arise from the interatrial septum, we present the rare example of a large papillary fibroelastoma arising from the left side of the interatrial septum.

Characterizing the inflammatory reaction in explanted Medtronic Freestyle stentless porcine aortic bioprosthesis over a 6-year period.

BACKGROUND The Medtronic Freestyle valve is a stentless porcine valve with reportedly excellent clinical and hemodynamic results, but little has been reported about its long-term pathology. METHODS Seventeen Freestyle valves were explanted (from 2003 to 2009) and reviewed to assess reasons for bioprosthesis failure. All valves were examined in detail, using histochemistry and immunohistochemistry to identify morphological changes, as well as cellular and humoral responses. RESULTS One Freestyle valve, explanted for mitral valve endocarditis on the fifth postoperative day, was excluded from analysis. The average implant duration was 71.1±35.2 months. Six valves were explanted for infective endocarditis, six for aortic insufficiency, and four for aortic stenosis. Calcification was seen in 11 explants, pannus in 15, thrombus in 12, cusp tears in 9, and 10 explants showed needle tract-like injuries. A chronic inflammatory reaction involving the xenograft arterial wall was seen in 15 of 16 valves. The cells were composed of macrophages and lymphocytes, including T cells (CD8 positive) and B cells. Significant damage to the porcine aortic wall was seen in 15 cases, and cusp myocardial shelf damage in 7 cases. All cases stained positively for IgG and C4dpar. CONCLUSIONS The porcine aortic tissue showed T cell-mediated rejection and significant aortic medial damage, consistent with dilatation of the porcine aortic root. The demonstration of IgG suggests the likelihood of humoral rejection, in addition to cellular rejection. One of the underlying possibilities is that the porcine aortic tissues are inadequately fixed, hence the retained antigenicity.

Cardiac fibroma in adults.

We present the case of a 61-year-old woman with nonspecific symptoms who on investigation and treatment had a fibroma of the right ventricular free wall. She underwent surgical resection of the mass and is doing well. The literature pertaining to cardiac fibromas in adults is reviewed and discussed.

Sudden cardiac death due to coronary artery dissection as a complication of cardiac sarcoidosis

Spontaneous coronary artery dissection (SCAD) is a rare cause of acute coronary syndrome (ACS), cardiac tamponade and sudden cardiac death that typically affects young women in the postpartum period. Rarely, it can be caused by systemic inflammatory conditions such as sarcoidosis. Sarcoidosis is an inflammatory disease most often affecting the lung and lymph nodes that can sometimes affect the heart. The authors report a case of sudden cardiac death caused by SCAD in the context of undiagnosed and subclinical cardiac sarcoidosis. The decedent was a 47-year-old male with a relatively innocuous past medical history. He was found dead in bed. At autopsy, there was a lethal hemopericardium resulting in cardiac tamponade. Gross examination of the heart revealed dissection of the posterior descending coronary branch of the right coronary artery. Histologically, the coronary artery showed acute and organizing dissection with evidence of vasculitis. A chronic inflammatory infiltrate consisting of lymphocytes, histiocytes, eosinophils and giant cells was seen. Sections of the myocardium showed myocarditis with a nonnecrotizing granuloma. The death was attributed to cardiac tamponade secondary to SCAD in the context of systemic sarcoidosis. The presented case demonstrates two concurrent rare pathologies and highlights the importance of considering SCAD in cases of sudden cardiac death at autopsy.