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

We have presented two patients with heart failure due to cardiac amyloidosis. The diagnosis was suspected from the echocardiogram and was confirmed by rectal biopsy. The echocardiogram features, which include thickening of the heart walls and other intracardiac structures, differentiate cardiac amyloidosis from other forms of heart disease. Thus, echocardiography should be helpful in the preliminary evaluation of patients with obscure heart failure and may obviate the necessity for more invasive studies.
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PMID:Amyloid heart disease. 730 46

Nineteen out of 121 consecutive cardiac biopsies from 107 patients were found to contain amyloid deposits on routine Congo red screening. Seventeen were left atrial appendages removed during mitral valvotomy for chronic rheumatic mitral valve disease while the remaining two were right atrial appendages excised during surgical repair of atrial septal defects. The distribution of amyloid deposits within the atria and their tinctorial characteristics are described. The high prevalence of atrial amyloidosis observed could not be attributed to generalized or senile amyloidosis. The possibility that this is a distinctive localized form of amyloidosis secondary to chronic heart disease is discussed.
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PMID:Microscopical atrial amyloidosis in chronic heart disease. 731 80

The authors described their experiences with endomyocardial biopsy (EMB) in 21 adult patients aged from 18 to 59 years with the preliminary diagnosis of cardiopathy of uncertain origin (CPUO). Their definition of CPUO is as symptoms and/or findings of cardiac disease, which cannot be exactly identify using even complex clinical and noninvasive cardiological diagnostic procedures. Diagnostic value of EMB was substantial in two of their patients (in one hypereosinophilia of myocardium, and in another amyloidosis), non specific results were found in 18 patients, and 1 patient had normal pattern of the biopsy. EMB is useful and safe clinical diagnostic procedure in exactly defined groups of patients.
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PMID:[Endomyocardial biopsy in cardiopathy of unknown origin]. 767 58

Survival of patients with increasingly complex congenital heart disease has produced a population of children and adolescents who are susceptible to subacute bacterial endocarditis (SBE). We report a child whose endocarditis went unrecognised, and who developed amyloidosis. Asymptomatic proteinuria, haematuria and renal impairment are occasionally seen in SBE and usually indicate glomerulonephritis. Amyloidosis should also be suspected in children with long-standing bacterial endocarditis with proteinuria or other evidence of renal impairment, especially if associated with organomegaly. The diagnosis is made by renal biopsy.
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PMID:Secondary amyloidosis from long-standing bacterial endocarditis. 774 18

Familial amyloidosis, Finnish type (FAF), is a gelsolin-related inherited systemic amyloidosis. We report autonomic nervous system and cardiac findings in a study of 30 FAF patients (18 females, 12 males aged 27-74 years; mean 53.9 years). Cardiovascular reflex tests showed a significant decrease in heart rate variation in FAF patients compared with healthy controls. Orthostatic hypotension was found in 9 of 28 FAF patients, but only in 3 of 69 controls. Signs of amyloid cardiopathy were rare at clinical examination and in radio-, echocardio- and electrocardiographic examinations. Histological and immunohistochemical studies revealed amyloid deposition and immunoreactivity against the gelsolin-related FAF amyloid subunit in autonomic nervous system structures and in cardiac tissue in 3 autopsied FAF patients. The results show that minor autonomic nervous system dysfunction can be found in FAF, while clinically significant amyloid cardiopathy or autonomic neuropathy is not characteristic of this type of amyloidosis.
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PMID:Autonomic nervous system and cardiac involvement in familial amyloidosis, Finnish type (FAF). 783 45

Cardiac amyloidosis, most often of AL type, is a non-exceptional disease as it represents 5 to 10% of non-ischemic cardiomyopathies. It realizes typically a restrictive cardiomyopathy. Nevertheless the wide diversity of possible presentation makes it a "big shammer" which must be evoked in front of every unexplained cardiopathy after the age of forty. If some associated manifestations can rapidly suggest the diagnosis, as a peripheric neuropathy especially a carpal tunnel syndrome or palpebral ecchymosis, cardiac involvement can also evolve in an apparently isolated way. The most suggestive paraclinic elements for the diagnosis are, in one hand, the increased myocardial echogenicity with a "granular sparkling" appearance seen throughout all walls of the left ventricle and, in the other hand, the association of a thickened left ventricle and a low voltage (electrocardiogram could also show pseudo-infarct Q waves). In front of such aspects, the proof of amyloidosis is brought by an extra-cardiac biopsy or by scintigraphy with labelled serum amyloid P component, so that the indications of endomyocardial biopsy are very limited today. The identification of the amyloid nature of a cardiopathy has an direct therapeutic implication: it contra-indicates the use of digitalis, calcium channel blockers and beta-blockers. The treatment of AL amyloidosis (chemotherapy with alkylant agents) remains very unsatisfactory especially in the cardiac involvement which is the most frequent cause of death (in AL amyloidosis). Last, cardiac amyloidosis is a bad indication for transplantation which results are burden by rapid progression of deposits especially in the gastro-intestinal tract and the nervous system.
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PMID:[Cardiac amyloidosis. General review]. 805 46

Endomyocardial biopsies (EMBs) of 47 patients with various cardiac disorders were analysed. Clinically 26 patients were diagnosed as having dilated cardiomyopathy (DCM), 9 myocarditis, six restrictive heart disease and six as Duchenne muscular dystrophy. EMB showed normal histological picture in six cases of DCM and findings consistent with DCM in rest of the twenty cases. None of these biopsies showed evidence of myocarditis. Diagnosis of myocarditis could be confirmed on EMB only in three out of nine clinically diagnosed cases. Cases with restrictive heart disease showed normal biopsy in two, infarction in one, findings consistent with endomyocardial fibrosis in one and amyloidosis in two cases. EMB on six cases of Duchenne muscular dystrophy showed nonspecific histological changes. To conclude, in this series, cases of DCM did not show evidence of underlying myocarditis in endomyocardial biopsies.
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PMID:Endomyocardial biopsy--its evaluation in cardiac disorders. 836 41

Distinguishing constrictive pericarditis from restrictive cardiomyopathy, usually due to amyloidosis, is a relatively frequent and difficult diagnostic problem. This report describes, for the first time, a patient with constrictive pericarditis caused by direct, extensive infiltration of the pericardium by amyloid, with only minimal amyloid in the myocardium, and a normal heart weight of 320 g. This patient demonstrates that amyloid may be predominantly deposited in the pericardium and actually cause constrictive pericarditis, as well as simulate its hemodynamic presentation by myocardial deposition. Given a clinical and hemodynamic presentation compatible with either constrictive or restrictive disease, an endomyocardial biopsy or other biopsy revealing amyloidosis does not necessarily rule out pericardial constriction that may be due to amyloid infiltration. The relationship between constrictive pericarditis, seen in this patient, and the other more common manifestations of amyloid heart disease, and the hemodynamic profiles of amyloid cardiomyopathy and constrictive pericarditis are reviewed.
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PMID:A fatal case of constrictive pericarditis due to a marked, selective pericardial accumulation of amyloid. 845 58

Congo red screening of 211 consecutive cardiac biopsy specimens obtained during cardiac surgery from 167 patients revealed 26 (16%) instances of isolated atrial amyloidosis (IAA). The ages of IAA-positive patients ranged from 25 to 52 years (mean age, 39 years). Twenty-three (88%) IAA-positive biopsy specimens were from patients with chronic rheumatic heart disease (CRHD) while three (12%) were from patients with an atrial septal defect (ASD). The prevalence of IAA in the CRHD patients was 23%, appreciably higher than that in the ASD patients (15%) and in other patients with atrial biopsies. The prevalence of IAA in both CRHD and ASD patients was significantly higher (P < .001) than in controls. Controls consisted of 247 healthy adults who were autopsied after traumatic deaths, with an age range of 18 to 89 years (mean age, 38 years). Only seven (3%) control subjects were IAA positive; all were over 40 years of age. Isolated atrial amyloidosis deposits were permanganate resistant and immunohistochemically positive for human amyloid P (AP) protein and negative for human amyloid-associated (AA) protein and immunoglobulin light chains. They were observed as fine congophilic and birefringent deposits in intramyocardial vessel walls, along the myocardial sarcolemma, and in the subendocardium. There was associated myocyte hypertrophy but no atrophy. Electron microscopy demonstrated typical nonbranching amyloid fibrils. It is postulated that stretching of the atria in chronic heart disease results in a raised prevalence of IAA. Recent reports that IAA contains atrial natriuretic peptide, a polypeptide hormone product of atrial myocytes, supports this view.
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PMID:Isolated atrial amyloidosis: a clinicopathologic study indicating increased prevalence in chronic heart disease. 850 38

This study was designed to characterize the geometry and function of the right ventricle and its prognostic significance in patients with primary (AL) cardiac amyloidosis. AL amyloidosis is an infiltrative systemic disease that can result in thickening of heart structures and rapidly progressive congestive heart failure due to restrictive ventricular physiology and eventual systolic dysfunction. Thirty-seven patients with AL amyloid heart involvement and 20 normal control subjects were evaluated using 2-dimensional and Doppler echocardiography. Based on the ratio of left-to-right end-diastolic ventricular chamber areas, patients were classified into 2 groups: 25 patients with disproportionate right ventricular (RV) dilation (left ventricular to RV ratio < or = 2) and 12 with a ventricular area ratio > 2. Patients with a relatively dilated right ventricle (ratio < or = 2) had a shorter median survival (4 months) compared with patients with an area ratio > 2 (10 months, p <0.003). Of multiple clinical, echocardiographic, and Doppler features entered into a multifactorial model, a ventricular area ratio < or = 2 remained the only independent predictor of survival. Patients with AL amyloid heart disease represent a heterogeneous population with regard to both prognosis and the relative degree of right to left ventricular dilation. RV dilation in patients with amyloid heart disease appears to be associated with more severe involvement and is associated with a very poor prognosis with a median survival of only 4 months.
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PMID:Right ventricular dilation in primary amyloidosis: an independent predictor of survival. 928 63


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