Nonspecific Myocarditis Research Articles

OP-085 The Electrical Heterogeneity of the Ventricle Repolarization Assessed by Frontal Planar Qrs/T Angle Is Increased with Late Gadolinium Enhancement in Patients with Non-Specific Myocarditis

OP-085 The Electrical Heterogeneity of the Ventricle Repolarization Assessed by Frontal Planar Qrs/T Angle Is Increased with Late Gadolinium Enhancement in Patients with Non-Specific Myocarditis

Prevalence of Cardiac Sarcoidosis in Patients Presenting with Monomorphic Ventricular Tachycardia

Sarcoidosis is a granulomatous disease of unknown etiology, which involves the heart in 5-25% of cases. Although ventricular tachycardia (VT) has been reported as the first presentation of sarcoidosis, its prevalence has not previously been investigated. In this prospective study, we sought to systematically investigate the prevalence of cardiac sarcoidosis (CS) in patients presenting with monomorphic VT (MMVT) and no previous history of sarcoidosis. Consecutive patients presenting with MMVT to a tertiary care center were screened for inclusion. Patients with idiopathic VT, VT secondary to coronary artery disease, or prior diagnosis of sarcoidosis were excluded. Included patients underwent F-18-fluorodeoxyglucose positron emission tomography (PET) scan. In subjects with PET scanning suggestive of active myocardial inflammation, histological diagnosis was confirmed through extracardiac or endomyocardial biopsy (EMB). A total of 182 patients presented to our institution with VT between February 2010 and September 2012 and 14 subjects met inclusion criteria. Within this group, six of 14 (42%) patients had abnormal PET scans suggesting active myocardial inflammation. Four of the six patients had tissue biopsies that were diagnostic of sarcoidosis; the remaining two patients had guided EMB indicating nonspecific myocarditis. Atrioventricular block was observed in three of four (75%) patients with CS and none in 10 of the others (P = 0.022). Three of the four patients had pulmonary sarcoidosis and one patient had isolated CS. All four patients were treated with corticosteroids. In this prospective study, four of 14 (28%) patients presenting with MMVT (without idiopathic VT, ischemic VT, or known sarcoidosis) had CS as the underlying etiology. Clinicians should consider screening for CS in patients with unexplained MMVT.

Human immunodeficiency virus (HIV) related heart disease: A review

Recent advances in the knowledge of human immunodeficiency virus (HIV) replication and transmission as well as the emergence of effective antiretroviral therapies are leading to longer survival times for HIV-infected individuals. As a result, organ related manifestations of late stage HIV infection, including HIV-related heart diseases have emerged. It is now clear that cardiac involvement in HIV seropositive patients is relatively common and is associated with increased morbidity and mortality. Cardiac involvement in HIV infection is multifactorial. The epidemiology has changed dramatically since the introduction of highly active antiretroviral therapy (HAART), but studies carried out before the introduction of HAART remain relevant because of limited access to this treatment in many areas of the world. A variety of cardiac lesions have been reported in HIV infection and AIDS, including pericardial disease with effusion and tamponade, nonspecific or infectious myocarditis, dilated cardiomyopathy with global left ventricular dysfunction, endocardial valvular disease due to marantic or infective endocarditis, arrhythmias, pulmonary hypertension and neoplastic invasion. In the post HAART-era, coronary artery disease and dyslipidaemia, drug related cardiotoxicity and cardiac autonomic dysfunction are becoming increasingly prevalent. In this review, we highlight the importance of cardiac complications in HIV disease and discuss measures that can be taken to improve survival.

Management of Left Ventricular Aneurysm by Intracavitary Repair

Left ventricular aneurysms (LVAs) occur in up to 40% of patients after myocardial Most LVAs are caused by occlusion of the coronary arteries, most frequently the left anterior descending coronary artery, and are located in the anteroapical region of the left ventricle. They appear as circumscribed, noncontractile areas of tissue that can be identified by systolic thinning of the infarcted area of myocardium. Occasionally, LVAs result from trauma, nonspecific myocarditis, bacterial endocarditis, or congenital malformations of the heart. Whether to treat a patient with an LVA medically or surgically depends on the status of left ventricular function and on the severity of symptoms. Patients with LVAs usually have (in order of frequency) congestive heart failure (with or without angina), thromboembolism, and ventricular tachyarrhythmias. In patients with severe symptoms and medically intractable heart failure or angina, surgery is the only option. In most patients, symptoms and prognosis can be improved by repairing the aneurysm and, when necessary, by performing concomitant coronary artery bypass or valve repair. The goal of treatment is to restore normal filling volume and normal geometric chamber configuration. Until cardiopulmonary bypass became a reality, few attempts were made to correct left ventricular aneurysms. Soon after the introduction of cardiopulmonary bypass, however, we reported the first successful repair of a ventricular ane~rysm.~ In that procedure, we dissected the adherent parietal pericardium from the aneurysmal surface and then completely excised the aneurysm, trimming the edges back to the functional myocardium. Since then, we have used a variety of other techniques to repair ventricular aneurysms, including plication, septoplasty with plication, patches, septa1 patches, and overlapping and felt buttre~ses.~ Although these methods were satisfactory, each produced either distortion, papillary dysfunction, or compromised ventricular volume. In 1988, I tried a new method of repair in a few patients whose ventricular aneurysms were so large that repair by excision would have left too little normal myocardium to restore ventricular function. This technique of intracavitary repair, which I called ventricular endoaneurysmorrhaphy, differed from earlier techniques I had used4q5 and from similar techniques used by other^.^.^ Our results (detailed later) were excellent, and as our experience broadened, we began to use the endoaneurysmorrhaphy technique on even the highest-risk patients with good results. Furthermore, we found that endoaneurysmorrhaphy could be easily adapted to any situation where active myocardium was present. We now prefer this technique over any other. This report will detail the different indications and techniques of intracavitary repair that we use in the treatment of left ventricular aneurysms.

Opportunistic Invasion of the Heart in Hispanic Patients with Acquired Immunodeficiency Syndrome

One hundred consecutive patients who died of the acquired immunodeficiency syndrome (AIDS) were studied with an emphasis on the heart. Thirty-two patients showed pathologic changes. The pathologic findings included infection by Histoplasma capsulatum, Toxoplasma gondii, Mycobacterium tuberculosis, cytomegalovirus. Cryptococcus neoformans, and atypical mycobacteria. Noninfectious pathologic findings included nonspecific myocarditis, focal necrosis, focal fibrosis, and acute subendocardial infarction.

Cardiac manifestations of acquired immune deficiency syndrome: A 1991 update

Cardiac involvement is being identified more often clinically and at autopsy in patients with AIDS. Recent estimates suggest that in the United States as many as 5000 patients per year may have cardiac complications resulting from HIV infection. Patients with AIDS may have pericardial, myocardial, and/or endocardial disease. Pericardial tamponade and/or constriction may be related to neoplasms, infections, or nonspecific effusions. Myocardial dysfunction may result from specific neoplastic infiltration or myocarditis. Particularly intriguing is the role of HIV-1 in the nonspecific myocarditis and dilated cardiomyopathy that occurs in patients with AIDS. As in other debilitating conditions patients with AIDS can have nonbacterial thrombotic endocarditis. Infective endocarditis may be a complication, especially in AIDS associated with intravenous drug abuse. Most patients with AIDS have no overt clinical evidence of cardiac disease. When cardiac dysfunction does develop, the signs and symptoms are often misinterpreted to be the result of noncardiac causes (pulmonary failure or infection), which can mimic heart failure. This review is intended to alert the reader to the cardiac manifestations of AIDS, which present a number of diagnostic and therapeutic challenges.

Conduction System in Children with Acquired Immunodeficiency Syndrome

Six children died of acquired immunodeficiency syndrome (AIDS), four of them females, ages 7 months, 13 months, 2 years 8 months, and 4 years; and two of them males, aged 2 1/2 and 7 years. They were born to IV drug-addicted parents. The conduction system (CS) and the entire heart were studied by serial section. In all cases the heart was hypertrophied and enlarged; one had total thrombotic occlusion of the right coronary artery with extensive infarction of the ventricular septum. Vascular changes also were found in all hearts, involving small arteries, arterioles, and venules. In the arteries, they involved the intima, media, and adventitia, and perivascular areas in a degenerative and inflammatory process. The elastic tissue was especially affected. A nonspecific myocarditis was present in four cases and epicarditis in all. Changes in the summit of the ventricular septum were present in four cases, consisting of increased fibrosis and arteriolosclerosis. The CS changes varied in location, showing either vasculitis, myocarditis, or fragmentation of the bundle with lobulation and fibrosis. The changes in the conduction system were not as severe as the changes in the surrounding myocardium. In one case the ECG was abnormal, showing left hemiblock. This corresponded to the finding of fibrosis, vacuolization of cells, and space formation in the left bundle branch.

Nonspecific myocarditis. A statistical and clinicopathological study of autopsy cases.

Among a total of 634,440 autopsy cases in "The Annuals of Pathological Autopsy Cases in Japan" from 1958 to 1984, 929 cases with nonspecific myocarditis were registered. The average incidence was 0.15%, fluctuating around 3- to 5-year intervals with a remarkable rise observed after 1974. The major complications in cases of myocarditis were pneumonitis, hepatitis or hepatic cirrhosis, pancreatitis, malignancies, lymphatic or thymic involvements. A clinicopathological study of 36 cases of myocarditis and 27 cases of postmyocarditic cardiomegaly indicated a classification of acute, subacute, healing and chronic or recurrent stages as well as dilatation-hypertrophy- and right ventricle-dominant types. Acute myocarditis was characterized by diffuse inflammatory cell infiltration and showed various types of arrhythmias and shock. Subacute myocarditis showed ventricular dilatation, edematous interstitium and severe congestive heart failure. Chronic myocarditis with dilatation and/or hypertrophy and irregular fibrosis included right ventricular involvement, endomyocardial disease, sick sinus syndrome in selected cases, congestive heart failure in most cases, and showed a male predominancy. Postmyocarditic cardiomegaly was similar to chronic myocarditis but showed more hypertrophy, preexcitation waves and prominent negative T waves in electrocardiography and sudden death.

Is immunoperoxidase staining of endomyocardial biopsies for interstitial inflammatory cells helpful in the diagnosis of myocarditis?

Quantitative evaluation of interstitial inflammatory infiltrates has been proposed as a criterion to distinguish myocarditis from noninflammatory heart disease. Leucocytic infiltrates were quantitated by an immunoperoxidase technique on a single frozen fragment in 199 consecutive endomyocardial biopsies from 160 patients. Biopsies with a histological diagnosis of myocarditis showed a higher mean number of leucocytes per mm2 compared to biopsies diagnosed as normal, nonspecific or resolved myocarditis; however, the range of counts revealed considerable overlap with the other diagnostic categories. Therefore, myocarditis cannot be diagnosed based on interstitial inflammatory cell counts alone. However, quantitation of immunoperoxidase-stained leucocytes may prove useful in identifying patients with increased interstitial inflammation to determine the nature of ‘borderline’ myocarditis.

The usefulness of an endomyocardial biopsy in heart disease of unknown etiology

Light-, electron microscopic and enzyme histochemical examinations (phosphorylase, LDH, NADH:TR, SDH and 3-HBDH) were performed on endomyocardial biopsies of 26 patients with heart diseases of unknown etiology. On the basis of the clinical findings the patients were grouped into hypertrophic cardiomyopathy (3 patients), dilated-congestive cardiomyopathy (8 patients), latent cardiomyopathy and small vessel disease (11 patients) and myocarditis (4 patients). Morphologic changes which might characterize the pathogenesis, were found in 7 patients: small vessel disease in 3 patients, nonspecific myocarditis in 1, iron storage disease in 1, adriamycin cardiomyopathy in 1 and cardiomyopathy with inclusions typical of Fabry's disease in 1 patient. In the other patients the morphologic changes were not sufficiently characteristic to be indicative of an etiopathogenesis. Several pathologic alterations did, nonetheless, appear to have a certain prognostic value such as endocardial and interstitial fibrosis, myofibrillolysis, myolysis, mitochondrial degeneration and increased lipid content in the muscle fibers. The frequency of these changes was evaluated partly semiquantitatively, partly by means of the point-counting method and graded with 1–3 points. Three patients with congestive cardiomyopathy scored at least 7 points. Two of them died within 8 weeks, 1 patient with adriamycin cardiomyopathy recovered after discontinuation of the therapy but he died 4 years after the biopsy. Six to 50 months after the biopsy (mean 31.5, median 6.5) the score was less than 7 in the other patients and all these patients were still alive. The histochemical changes manifested as an increase and/or a decrease of the enzymatic activities, involving scattered muscle fibers or their segments. A decrease of the activities of all dehydrogenases examined appeared to be prognostically ominous, correlating with a score of 7 or higher. A decrease of SDH activity in 7 cases, in combination with a decrease of the HBDH activity in 4 of them, was indicative of a disturbance in the Krebs cycle and lipid metabolism in the absence of ischemic damage. The alterations in the phosphorylase activity did not, however, appear to have a prognostic significance. Normal activity of the phosphorylase seemed to be prognostically favorable.

Bone Marrow Emboli Versus Fat Emboli as the Cause of Unexpected Death

Thromboemboli were found diffusely throughout the pulmonary vasculature system of a 76-year-old white female who died unexpectedly 3 days following injury and repair of a left hip intracapsular fracture. A diffuse chronic nonspecific myocarditis with marked fat atrophy, an acute myocardial infarct of the posterior left ventricular papillary muscle, and an acute right lower lobe bronchopneumonia are believed to be the cause of death.

Granulomatous inflammation of the heart.

Morphologic characteristics of granulomatous inflammation in the heart and pericardium are discussed. In rheumatic fever, two types of myocardial lesion are present--a nonspecific myocarditis and a specific lesion characterized by granulomas known as Aschoff's nodules. The latter undergo a cycle of development and resolution; in their mature stage, they contain Aschoff's cells which are uni- or multinucleated histiocytes with a serrated nuclear chromatin bar. Ultrastructural studies do not suggest a relationship between these cells and cardiac or smooth muscle cells. In metabolic disorders, granulomas occur in Farber's disease (lipogranulomatosis), gout (in which tophi are associated with calcific deposits and with a foreign body cellular reaction), the various syndromes of oxalosis (in which oxalate deposits also lead to a foreign body reaction), and in chronic granulomatous disease of childhood. Foreign body giant-cells can also be found in association with calcification of necrotic myocytes and in the syndromes of "cholesterol pericarditis." Well-developed granulomas occur in sarcoidosis, giant cell myocarditis, as a reaction to foreign bodies and devices implanted within the cardiovascular system, and in certain diseased caused by infective agents (tuberculosis, fungal and parasitic disorders). Infiltration of the heart by nongranulomatous masses of histiocytes can occur in Whipple's disease, Niemann-Pick disease, the hyperlipoproteinemias, Gaucher's disease, and in proliferative disorders of the mononuclear phagocyte system (juvenile xanthogranuloma, Chester-Erdheim syndrome, and malignant histiocytosis).

Cardiovascular lesions in collagen-vascular diseases.

In this review, the cardiac lesions which develop in association with the various collagen-vascular diseases are described. In rheumatoid arthritis, the most frequent lesions are: fibrous obliterative pericarditis, with pericardial deposits of calcium, fibrin, cholesterol, and rheumatoid granulomas; granulomatous or nonspecific myocarditis; valvulitis, vasculitis, and amyloid deposits. In ankylosing spondylitis, the lesions involve mainly the valves (aortic and mitral valves) and the aorta. In systemic lupus erythematosus, the predominant cardiovascular lesions are: pericarditis, Libman-Sacks endocarditis, nonspecific myocarditis, vasculitis with fibrinoid necrosis, and acceleration of atherosclerosis. In scleroderma, the main cardiac lesion is fibrosis with only scanty inflammatory cells; pericarditis and nonbacterial thrombotic endocarditis also occur. In dermatomyositis/polymyositis, fibrous or fibrinous pericarditis can occur, as well as myocarditis with infiltrates of lymphocytes and plasma cells and with degeneration and necrosis of myocytes; valvulitis is uncommon except when the disease is related to mucinous adenocarcinoma. In polyarteritis nodosa, various stages of necrotizing vasculitis involve all layers of the arterial walls; foci of myocardial necrosis of various sizes can occur in association with these lesions; cardiac hypertrophy related to hypertension and pericarditis related to uremia, may also be found. In Wegener's granulomatosis, pericarditis, inflammatory infiltrates, necrotizing granulomas, and vasculitis have been observed in the heart.

Stokes-Adams attacks due to acute nonspecific myocarditis in childhood.

Six patients aged between 1 to 11 years (4 females and 2 males) developed Stokes-Adams attacks with complete heart block due to acute nonspecific myocarditis. Transvenous pacing was instituted in 2 patients, but in the other 4 patients the ECG returned to normal by isoproterenol. All ECGs in complete heart block showed QRS complexes of right bundle branch block with left posterior hemiblock pattern, except for 1 which showed QRS complexes of complete left bundle branch block pattern. The ECG improved sequentially in order and left anterior hemiblock pattern of QRS complexes remained to the last during the convalescent period. Normal atrioventricular conduction returned by 2 to 24 hours in all but 1 patient who was dead and 1 of the 2 patients with shock. In another patient bifascicular block has persisted.

LONG-TERM EXPOSURE TO DOCOSENOIC ACIDS IN ANIMALS: ABSENCE OF MYOCARDIAL LESIONS IN HARP SEALS (PAGOPHILUS GROENLANDICUS) AND GREY SEALS (HALICHOERUS GRYPUS)

Hearts were collected from 46 harp or grey seals of various ages and sexes. After fixation in formalin, sections were cut and stained with hematoxylin-eosin, and in a limited number of cases with Oil-Red-0. Histopathological examination revealed the presence of non-specific myocarditis in two instances only and no lipid deposits. The percentage of docosenoic acid (primarily 22:1ω11) in blubber and heart muscle triglyceride, tabulated for grey seal dam-pup pairs is evidence of exposure from birth. These results indicate that the susceptibility of the rat myocardium to docosenoic acids may not be as broadly relevant in other species such as the seal, pig and man as has been proposed.

Stokes-Adams attacks due to acute nonspecific myocarditis

Ten patients, all below 30 years of age (8 females and 2 males) developed Stokes-Adams attacks from complete heart block due to acute nonspecific myocarditis. Coexisting thyrotoxicosis was present in two patients and cardiogenic shock was seen in four. Temporary transvenous pacing was instituted in all but one patient. Except for two patients who developed permanent complete heart block, normal A-V conduction returned in between 1 to 12 hours after ventricular pacing in seven patients and after 12 hours of isoprenaline therapy in the final patient. The ECG returned to normal in six patients and all 10 patients survived their acute illness.

Pseudo-LE syndrome after treatment with Venopyronum dragees] (author's transl)

During the past three years a pseudo-LE syndrome (with antimitochondrial antibodies) was diagnosed in 14 patients. Information from the patients and their doctors disclosed that twelve of them had taken Venopyronum dragees over a period of two months to five years before onset of first symptoms. Two of the patients died of nonspecific myocarditis. Clinical investigations strongly suggest that the drug may have played a role in the pathogenesis of the pseudo-LE syndrome which is likely to take a severe course if, as in the two patients, the cardiovascular system is involved.

Primary myocardial disease in children, with special references to idiopathic myocarditis.

Three cases of endocardial fibroelastosis, seven cases of acute nonspecific myocarditis, two cases of obscure cardiomyopathy and one autopsied case of idiopathic hypertrophic subaortic stenosis with Nielsen's syndrome were described. Development of myocardial hypertrophy along the course after the acute stage of nonspecific myocarditis in certain cases was suggested. For clarifying the possible correlation between idiopathic cardiomyopathy and nonspecific myocarditis further investigation is considered to be necessary.

The clinical and hemodynamic pattern in nonspecific myocarditis: A comparison with other entities also impairing myocardial efficiency

The clinical and hemodynamic pattern in nonspecific myocarditis: A comparison with other entities also impairing myocardial efficiency

Non-Diagnostic Electrocardiographic Patterns

Non-diagnostic electrocardiographic tracings are encountered frequently which do not coincide with the criteria of established picture. They are occasionally confused with more characteristic patterns. Such variations which are included in this discussion are found in neurocirculatory asthenia, acute nonspecific myocarditis or pericarditis, chronic constrictive pericarditis, thyroid dysfunctions, beriberi, and various electrolyte imbalances. Recognition of suggestive patterns in conjunction with a careful history, examination, and other laboratory studies may prove vital since most of these conditions are reversible with appropriate therapy.