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Query: UMLS:C0018799 (heart disease)
 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

With the advent of cardiac catheterization, cardioangiography, and selective coronary arteriography, specific types of cardiac disease can be recognized and clearly defined. This is appropriate because myocardial biopsy alone rarely plays a major role in cardiac diagnosis. Excluding Aschoff's nodules in patients with rheumatic valve disease, the light microscopic findings in patients with rheumatic heart disease, congenital heart disease, pericardial disease, hypertensive and arteriosclerotic heart disease are similar and nonspecific. In these, interstitial fibrosis and/or myocardial hypertrophy is the dominant tissue diagnosis. Occasionally a pericardial and myocardial specimen is helpful to distinguish constrictive pericarditis and restrictive cardiomyopathy. Myocardial biopsy has provided the only method for diagnosis in a small number of patients with normal hemodynamics, normal coronary arteriograms and normal ventriculograms. The patients were studied because of chest pain and/or cardiac arrhythmias. Supraventricular and/or ventricular arrhythmias were encountered. In these patients the tissue diagnosis was interstital fibrosis and/or myocardial hypertrophy. These findings are consistent with primary myocardial disease which was not recognized clinically or by angiographic studies. The procedure seems to play a major role in the diagnosis of specific types of primary myocardial disease. It is valuable in the recognition of glycogen storage disease, amyloidosis, hemochromatosis, and myocarditis. On the basis of current experience, the indications for myocardial biopsies depend on the need for a tissue diagnosis in determining the management of the patient and the availability of adequately trained personnel to perform the procedure and manage the complications.
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PMID:The role of myocardial biopsy in cardiac diagnosis. 12 52

Echocardiography was used to compare the left ventricular function in 35 cases of congestive cardiomyopathy (COCM), 16 cases of obstructive and 28 cases of non-obstructive asymmetric septal hypertrophy, 43 cases of scleroderma heart disease, 21 cases of ischaemic cardiomyopathy (ICM), 2 cases of restrictive cardiomyopathy, and one case of cardiac amyloidosis. COCM was characterized by left ventricular dilatation, decreased contractility and signs of elevated end-diastolic pressure. In asymmetric septal hypertrophy there was a decreased distensibility of the obstructed left ventricle, both in early and end-diastole, but the pump function remained normal. In scleroderma and amyloid heart disease both the contractility and distensibility of the small, stiff heart were reduced. In restrictive cardiomyopathy the only abnormality was the impaired end-diastolic distensibility. Reduced contractility confined primarily to the interventricular septum and impaired early diastolic distensibility are the characteristic features distinguishing ischaemic cardiomyopathy from COCM. However, echocardiography is not suitable for individual differentiation of ICM and COCM. The COCM can be differentiated from left ventricular aneurysm by M-mode sector scanning technique.
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PMID:Functional differentiation of various types of cardiomyopathies by echocardiography. 15 92

Six patients with cardiac amyloidosis (four males, two females; age 27-60 years) were evaluated by us. Four patients presented with congestive heart failure, while one patient each presented with effort angina and giddiness. Extracardiac clues to the diagnosis in the form of involvement of other systems were present in only two patients. The electrocardiogram was abnormal in four patients while three exhibited roentgenographic evidence of cardiomegaly or pulmonary venous hypertension. Echocardiography suggested the diagnosis of amyloidosis in only two patients, restrictive cardiomyopathy in two other patients and dilated and hypertrophic cardiomyopathy in one patient each. Cardiac catheterisation and angiography suggested restrictive heart disease in four patients and hypertrophic cardiomyopathy in one. One patient, whose initial haemodynamic study was normal, had features of dilated cardiomyopathy at repeat study after 11 months. Endomyocardial biopsies showed amyloid deposits in all patients. We emphasise the varied clinical manifestation of cardiac amyloidosis and the need for a high index of suspicion. The diagnosis can be safely and reliably confirmed by endomyocardial biopsy.
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PMID:Cardiac amyloidosis: hemodynamic, echocardiographic and endomyocardial biopsy studies. 130 87

Cardiomyopathies are defined as 'heart muscle diseases of unknown cause' and classified into hypertrophic, dilated and restrictive types, respectively. Hypertrophic cardiomyopathy is notable for massive ventricular hypertrophy without obvious cause, impaired diastolic and systolic function, a tendency for sudden death and a familial propensity. Dilated cardiomyopathy by contrast, demonstrates severe systolic failure progressing to congestive heart failure, with usually no familial tendency. Restrictive cardiomyopathy and diastolic heart disease represent syndromes with restriction to ventricular filling due to restrictive forces in the endomyocardium (and in constrictive pericarditis in the pericardium). The commonest cause of restrictive cardiomyopathy is endomyocardial fibrosis now usually known as hypereosinophilic endomyocardial disease. Specific heart muscle diseases are those conditions in which myocardial disease is due to a known cause: they usually produce systolic failure though occasionally a restrictive syndrome is evident. Amyloid heart disease occupies a place intermediate between cardiomyopathies and specific heart muscle diseases. The major features of the above conditions are described and current and future advances noted. Examples are the identification of the gene probably responsible for hypertrophic cardiomyopathy located on chromosome 14, and the identification of virus RNA particles in the myocardium in both myocarditis and in dilated cardiomyopathy, which strengthens the growing evidence suggesting that some cases of dilated cardiomyopathy may be due to previous myocarditis.
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PMID:Cardiomyopathies and specific heart muscle diseases. Definitions, terminology, classifications and new and old approaches. 140 13

All the transplantation units within the Italian Heart Transplantation Project are supported by a section of pathology, devoted to the study of the recipient's heart, to patient monitoring by means of a schedule of endomyocardial biopsies, and, if that was the case, to examine the donor's heart and to analyse the causes of death. When successes and failures of the first five years of the Project's activity are weighed up, good results are observed: of the 847 operations performed (orthotopic, heterotopic and heart-lung transplants, and re-transplants) an actuarial survival rate of 77% at 5 years has been achieved. The sections of pathology believe to have contributed significantly to these results, examining as many as 10,446 endomyocardial biopsies. The indications for transplantation were: dilated cardiomyopathy (48.5%); ischemic (35.3%); valvular (5.9%) and congenital (2.4%) heart disease; hypertrophic cardiomyopathy (2.2%); endocardial fibroelastosis (1.7%); restrictive cardiomyopathy (1.4%); anthracycline cardiotoxicity (0.8%); myocarditis (0.8%); cardiac tumours (0.5%) and arrhythmogenic cardiomyopathy (0.2%). Distribution of recipients by sex and age varied according to the indications for transplantation: males were more common among the patients transplanted for ischemic (97%) and valvular (84%) heart disease, as well as for dilated (82%) and hypertrophic (78%) cardiomyopathy, whereas the opposite was true for endocardial fibroelastosis (males constituting 21%) and cardiac tumours (25%). Mean age at transplantation ranged from 49 years (ischemic heart disease) to 6 years (endocardial fibroelastosis). In the follow-up period, a 17.5% death rate was recorded; the main causes of death were the early failure of the transplanted heart (27 pts), postoperative complications (16), hyperacute rejection (4), acute rejection (18), infections (the singular most frequent cause of death, 35 pts), the proliferative endoarteritis of coronary branches (the so-called chronic rejection, that caused 21 deaths and required 14 re-transplants) and the development of neoplasms (11). The actuarial survival curve drops to 89% after the first postoperative month, abates to 82% at the end of the first year, and progressively decreases to 77% at the end of the fifth follow-up year. Rejection monitoring required an average number of 12.5 endomyocardial biopsies per recipient, and allowed 1.7 rejection episodes per patient to be diagnosed. The fewer were the rejection episodes occurring in a unit, the higher was the percentage of deaths due to infections.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:[The contribution of pathology sections to the Italian Heart Transplant Project in the first 5 years of its activities (1985-1990)]. 147 59

Cardiomyopathies are an important cause of congestive heart failure in the elderly, and the magnitude of the problem is compounded by changing population demographics and the frequency of congestive heart failure in the elderly. Although the data are far from complete, differences in the clinical presentations and natural history of the cardiomyopathies in older and younger patients are becoming more clearly appreciated. Dilated cardiomyopathy (DCM) is clearly more common than previously appreciated, and elderly patients have a worse prognosis than their younger counterparts with this disease. The medical management of DCM is often more difficult in the elderly, and the problem is compounded by the relatively infrequent use of cardiac transplantation as a therapeutic option. Hypertrophic cardiomyopathy is also more common than previously appreciated, and changes in left ventricular structure often create difficulties in differentiating pathologic states from physiologic. Fortunately, the prognosis for HOCM is more favorable in the elderly than in younger patients and may be partly accounted for by the different structure of the left ventricle. If needed, surgery is an option for elderly patients with medically refractory HOCM, but particular attention must be paid to the presence and severity of associated cardiovascular disease. Restrictive cardiomyopathy with diastolic dysfunction is increasingly recognized as a cause of congestive heart failure. The differentiation from systolic dysfunction is crucial, because the treatments are so markedly different. Age-related changes in diastolic function are becoming more apparent and better characterized, but standardization of age-related "normal" values is still not available. The diagnosis of restrictive heart disease should stimulate a search for an underlying cause, inasmuch as restrictive cardiomyopathy remains a diagnosis of exclusion. Advances in noninvasive imaging have led to a resurgence of interest and have enhanced our knowledge of the cardiomyopathies. Further investigation should proceed in conjunction with studies aimed at defining the characteristics and variables of "normal" aging. For the present, the enigmatic, poorly identified "cardiomyopathies" remain a problem for both young and old.
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PMID:Cardiomyopathies in the elderly. 158 13

This study investigated the presence of atrial natriuretic factor in ventricular tissue obtained from humans with dilated or restrictive heart disease. In 17 patients with ventricular dilation and impaired systolic function and in 8 patients with restrictive heart disease and preserved systolic function, the presence of ventricular atrial natriuretic factor was investigated in tissue obtained by ventricular endomyocardial biopsy. The objective of the study was to determine if the ventricular presence of atrial natriuretic factor is dependent on ventricular dilation. Left ventricular end-diastolic volume index was greater in the group with dilated cardiomyopathy than in the group with restrictive cardiomyopathy (134 +/- 13 versus 78 +/- 5 ml/m2, p less than 0.05); end-diastolic pressure was elevated in the two groups (20 +/- 2 versus 25 +/- 4 mm Hg, p = NS). With the use of immunohistochemical techniques, ventricular atrial natriuretic factor was clearly detected in 15 of the 17 patients with dilated cardiomyopathy and in 6 of the 8 patients with restrictive cardiomyopathy. This study demonstrates the high prevalence of ventricular atrial natriuretic factor in living patients with either systolic or diastolic dysfunction. Whereas in the atria, stretch or dilation may be an important stimulus, atrial natriuretic factor in the ventricular chamber occurs independent of dilation.
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PMID:Expression of atrial natriuretic factor in the human ventricle is independent of chamber dilation. 182 93

There are obviously several causes of myocardial dysfunction but energy deficiency of the myocytes may play a significant role and probably is a common mechanism during the progression of myocardial failure. Theoretically, a poor utilization efficiency of oxygen may be due to exhaustion of the myocardial stores of bioenergetics. In this report the authors review their biochemical results from measurements of coenzyme Q10 (CoQ10) levels in blood and human endomyocardial biopsies using an HPLC method from patients with suspected myocardial disease (n = 45). The levels of CoQ10, which has a key role in the respiratory chain and the synthesis of ATP, was found to be significantly decreased in various groups of patients with myocardial failure (dilated and restrictive cardiomyopathy and alcoholic heart disease) as compared to "normal" myocardium (0.42 +/- 0.04 micrograms/mg dry weight). The deficiency of CoQ10 was more pronounced with increasing symptoms; e.g. patients with dilated cardiomyopathy in NYHA Classes III and IV had lower tissue CoQ10 content than those of Classes I and II (0.28 +/- 0.04 vs. 0.37 +/- 0.06 micrograms/mg, p less than 0.001). Nearly two thirds of a series of 40 patients in severe heart failure (Classes III and IV) treated with CoQ10, 100 mg daily, in an open, controlled design showed subjective and objective improvement. Clinical responders were 69% and 43% of patients with cardiomyopathy and ischaemic heart disease, respectively. The results suggest that CoQ10 is a novel and effective breakthrough in heart-failure therapy and it appears safe, as no adverse reactions were registered. The through in heart-failure therapy and it appears safe, as no adverse reactions were registered.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Coenzyme Q10: clinical benefits with biochemical correlates suggesting a scientific breakthrough in the management of chronic heart failure. 227 93

Five generations of an Italian family with an autosomal dominant restrictive cardiomyopathy are described. Members of four generations were examined. Symptoms usually developed in the third or fourth decade but the disease did occur in childhood. Initially the condition was characterised by normal ventricular size and systolic function with increased diastolic filling pressures in both ventricles and consequent bi-atrial enlargement. Cardiac catheterisation showed a left ventricular filling pattern of "dip and plateau". The electrocardiogram typically showed non-specific changes in the ST segment and T wave and changes indicating considerable atrial enlargement, which were confirmed by echocardiography. Light microscopy of two endocardial biopsy specimens showed no specific features but excluded the endomyocardial fibrosis of eosinophilic heart disease, amyloid, and specific heart muscle diseases. At necropsy in one case examined under light microscopy extensive patchy fibrosis was found throughout the endocardium, myocardium, and subepicardium, but there were no features typical of eosinophilic heart disease. Histopathological and biochemical examination of skeletal muscle identified no abnormality. The disease often had an insidious course over five to ten years after presentation. Bundle branch blocks, leading to complete atrioventricular block, however, often occurred and may be the first manifestation. Some individuals who survived into the fifth decade developed a progressive, non-wasting skeletal myopathy.
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PMID:Familial restrictive cardiomyopathy with atrioventricular block and skeletal myopathy. 231 4

Endomyocardial fibrosis, an uncommon condition, is the most important cause of restrictive cardiomyopathy. Etiology of endomyocardial fibrosis is unknown, but evidence continues to accumulate that a close connection exists between eosinophilia and endomyocardial disease. Patients with this condition respond poorly to medical treatment, and survival is only for a few months or years when late stage heart disease is present. Surgery is nowadays recommended in these cases. A marked eosinophilia was found in a 58 years old woman that was admitted to the hospital because of a severe congestive cardiac insufficiency. Two-dimensional echocardiographic study and typical angiographic appearance suggested the presence of endomyocardial fibrosis. Endocardectomy and mitral valvular replacement were performed.
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PMID:[Restrictive myocardiopathy caused by endomyocardial fibrosis]. 232 31


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