Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0018799 (heart disease)
 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A systematic investigation was performed in patients with familial amyloidotic polyneuropathy, Portuguese type (AFp) to assess the pattern and incidence of cardiac involvement. Of 327 patients investigated, ECG abnormalities were present in 285 (87.2%). Low voltage and QS pattern in V1, V2, V3 were found in 51.3% and 35.7% patients respectively. Conduction disturbances were present in 211 (64.5%). Sinus node disease, 1st degree and Wenckebach interventricular blocks were frequent. Complete atrioventricular block was observed in only 2 patients (0.6%). Left anterior hemi-block was present in 30.8%, left bundle branch block in 3.9%, left posterior hemi-block in 2.4% and right bundle branch block in 2.1%. Holter monitoring showed a much higher incidence of conduction disturbances, most of these occurring at night. The mean values of septum and posterior wall thickness and mass evaluated by echocardiography in 72 patients were normal. The systolic and diastolic global and regional functions, determined in 12 patients, analysing the echo by a digitization computer technique, were normal. In 7% a trivial pericardial effusion was observed. In 16 patients with ECG changes and normal echocardiograms the technetium 99m pyrophosphate scanning was negative. We conclude that the ECG is the most precise, sensitive and clinically useful method for detecting cardiac amyloidosis in patients with AFp. In spite of the rarity of congestive and restrictive patterns, the incidence and severity of conduction disturbances does not allow us to consider heart disease in AFp as a benign entity.
 ...
PMID:The heart in Portuguese amyloidosis. 377 1

Ten consecutive patients with tissue-proven amyloidosis, seven of whom presented with congestive heart failure, were found to exhibit intense diffuse uptake of technetium-99m-pyrophosphate (Tc-99m-PYP) on cardiac radionuclide imaging. The patients exhibited echocardiographic and systolic time interval abnormalities suggesting combined restrictive and congestive cardiomyopathic changes. On M-mode echocardiograms, there was symmetrically increased thickness of the interventricular septum and left ventricular (LV) posterior wall in diastole (10 of 10), decreased fractional shortening of the LV minor axis diameter in systole (eight of nine), and decreased percent thickening of the LV minor axis diameter in systole (eight of nine) and LV posterior wall (10 of 10) in systole. Three patients demonstrated enlarged LV end-diastolic diameter. All 10 patients had abnormal PEP/LVET and eight had shortened LVETI. When combined with noninvasive tests of LV performance, positive myocardial pyrophosphate (PYP) scanning provides a new and useful adjunct in the diagnosis of amyloid heart disease.
 ...
PMID:Value of positive myocardial technetium-99m-pyrophosphate scintigraphy in the noninvasive diagnosis of cardiac amyloidosis. 627 6

To determine the value of technetium-99m-pyrophosphate myocardial scintigraphy in the diagnosis of amyloid heart disease this procedure was prospectively performed in 20 consecutive patients with biopsy-proven primary amyloidosis. Eleven patients had echocardiographic abnormalities compatible with amyloid cardiomyopathy, 9 of whom had congestive heart failure. Diffuse myocardial pyrophosphate uptake was of equal or greater intensity than that of the ribs in 9 of the 11 patients with echocardiograms suggestive of amyloidosis, but in only 2 of the 9 with normal echocardiograms, despite abnormal electrocardiograms (p less than 0.01). Increased wall thickness measured by M-mode echocardiography correlated with myocardial pyrophosphate uptake (r = 0.68, p less than 0.01). None of 10 control patients with nonamyloid, nonischemic heart disease had a strongly positive myocardial pyrophosphate uptake. Thus, myocardial technetium-99m-pyrophosphate scanning is a sensitive and specific test for the diagnosis of cardiac amyloidosis in patients with congestive heart failure of obscure origin. It does not appear to be of value for the early detection of cardiac involvement in patients with known primary amyloidosis without echocardiographic abnormalities.
 ...
PMID:Sensitivity of technetium-99m-pyrophosphate scintigraphy in diagnosing cardiac amyloidosis. 629 87

Clinically significant cardiovascular abnormalities may occur as secondary manifestations of noncardiac neoplasms. The principal cardiac effects of noncardiac tumors include the direct results of metastases to the heart or lungs, the indirect effects of circulating tumor products (causing nonbacterial thrombotic endocarditis, myeloma-associated amyloidosis, pheochromocytoma-associated cardiac hypertrophy and myofibrillar degeneration, and carcinoid heart disease), and the undesired cardiotoxicities of chemotherapy and radiotherapy.
 ...
PMID:Cardiac effects of noncardiac neoplasms. 640 9

Amyloidosis is a family of disorders of the immune system. Each member of the family is characterized clinically by a unique syndrome and chemically by a specific amyloid fibril protein. The diagnosis of amyloidosis requires histopathologic identification of amyloid deposits in the affected tissues. Since none of the commonly used histochemical stains is specific for amyloid and their sensitivity is variable, application of a battery of staining methods (two or more) is essential for the histologic diagnosis of amyloidosis. The heart may be affected in any form of systemic amyloidosis and in senile amyloidosis. Cardiac amyloidosis is an important cause of progressive heart failure and refractory arrhythmia of obscure origin, especially in elderly persons. The average survival time of amyloid heart disease after the onset of symptoms is less than 3 years. Clinically, amyloid heart disease may mimic constrictive pericarditis, coronary artery disease, valvular heart disease, and idiopathic hypertrophic or congestive cardiomyopathy. A confirmatory biopsy is needed for diagnosis since cardiac amyloidosis has no pathognomonic symptoms and signs, nor diagnostic electrocardiographic, radiologic, cardioangiographic and echocardiographic findings.
 ...
PMID:Pathology of amyloidosis and amyloid heart disease. 640 Apr 65

Between 1973 and 1981, bilateral total replacement of hip and knee joints was performed in 22 patients with rheumatoid arthritis. During follow-up, 2 patients died of diseases not directly related to the operation. Besides these patients, there were 2 patients who could not be followed up. The remaining 18 patients constituted the subjects for study. We studied the postoperative results, emphasing improvement in the ability to perform everyday activities. Walking ability was improved in 16 patients. There was an increase in the number of patients able to rise from a chair, go up and down stairs, and get in and out of a car, bus or train. Of the 14 married patients (not including 2 patients who were unable to walk and 5 patients over 60 years of age) 7 were able to perform sexual intercourse as well as they had been able to when healthy. The patients who underwent bilateral total replacement of hip and knee joints often presented various problems, for example with regard to indications, the operative technique to use, as well as complications such as heart disease, pulmonary disease, and secondary amyloidosis. We have found, however, that bilateral total replacement of hip and knee joints can accomplish the operative objectives of eliminating pain and improving the quality of daily life in severely handicapped rheumatoid patients.
 ...
PMID:Bilateral joint replacement of hip and knee joints in patients with rheumatoid arthritis. 646 58

Twenty-five years ago clinical investigators began to appreciate that cardiomyopathy is an important and reasonably common form of heart disease. Since then, several functional classifications have been proposed, the specific myocardial diseases have been classified and chronic ischemic ventricular failure has been described. The boundary separating myocarditis from dilated cardiomyopathy remains hazy and, despite intensive research, the causes of dilated cardiomyopathy remain obscure. In particular, we still do not understand the role that may be played by viral infection and alcohol. Myocardial biopsy has proved useful in patients with specific myocardial disorders, heart transplant recipients and patients receiving Adriamycin, but is disappointing in patients with dilated cardiomyopathy. It has become increasingly evident that exercise capacity does not correlate with ventricular function, being highly dependent on peripheral factors. Measurements of oxygen consumption during exercise promise to be useful in assessing treatment of dilated cardiomyopathy. True restrictive cardiomyopathy is uncommon, and the term should be reserved for cardiomyopathies that meet strict criteria. A restrictive component to filling is common to many cardiac disorders, including some cases of cardiac amyloidosis. The concept of hypertrophic cardiomyopathy has evolved rapidly over the past 25 years, and continues to evolve. The importance of arrhythmia as a cause of sudden death is becoming increasingly clear. The place of calcium channel blocking agents in the treatment of hypertrophic cardiomyopathy will probably emerge soon. Amiodarone is finding an increasing role in the treatment of dilated and hypertrophic cardiomyopathy. Surgical treatment is still required for some patients despite unanswered questions on how it works.
 ...
PMID:Cardiomyopathy: how far have we come in 25 years, how far yet to go? 668 23

A patient with Bence-Jones myeloma and amyloidosis was treated with cytotoxic drugs and plasmapheresis, resulting in rapid improvement of myeloma-associated symptoms and signs. However, amyloidosis-associated symptoms, especially hypotension, grew worse. Echocardiographic examination demonstrated hypertrophy and a hyperrefractile appearance of the myocardium, thought to be pathognomonic of amyloid heart disease. A permanent pacemaker was inserted for treatment of Adams-Stokes attacks caused by sick sinus syndrome and atrioventricular conduction disturbances. Postmortem examination of the heart demonstrated abundant amyloid deposits corresponding to the altered acoustic qualities of the myocardium. Echocardiographic examination is a valuable non-invasive method for demonstration of amyloid deposits in the heart in multiple myeloma.
 ...
PMID:Myeloma-associated cardiac amyloidosis. A case report. 670 98

AA-protein was identified by SDS-acrylamide electrophoresis in amyloid fibrils fixed in formalin after isolation from fresh-frozen tissues obtained from patients with familial Mediterranean fever (FMF) amyloidosis and idiopathic AA-amyloidosis and, following deparaffination, rehydration and homogenization of embedded formalin-fixed tissues of old autopsy cases of the hereditary amyloidosis of FMF and amyloidosis acquired in association with tuberculosis, bronchiectasis, and rheumatoid arthritis. That AA-protein is unaltered by formalin was firmly established by agar gel diffusion using specific rabbit anti-AA serum. By contrast, AL proteins could not be demonstrated either in formalin-fixed amyloid fibrils derived from fresh-frozen tissues of a patient with presumably AL-amyloidosis dominated by cardiomegaly and one with AL-kappa amyloidosis or in blocks of cases of familial neuropathic amyloidosis, multiple myeloma, and idiopathic amyloidosis with cardiopathy. AA-protein is not denatured by formalin and retains its typical electrophoretic, chromatographic, and immunologic characteristics even 30 years after fixation and paraffin-embedding.
 ...
PMID:Demonstration of AA-protein in formalin-fixed, paraffin-embedded tissues. 706 12

With advancing age of the population and with echocardiographic means of diagnosis, amyloid disease of the heart is of increasing clinical interest. Advanced age, restrictive myocardiopathy, arrhythmias, and conduction disorders are familiar features of this disease. A 92 year old man with past history of hemiblock followed by complete heart block and transvenous pacemaker was admitted to the hospital because of increasing fatigue and the abrupt development of dyspnea. Examination revealed paradoxic pulse, markedly elevated central venous pressure, and echocardiographically demonstrated large pericardial effusion. Shortly after admission signs of tamponade developed; 1,000 ml of pericardial fluid was removed with prompt relief of dyspnea dna disappearance of paradoxic pulse and return of central venous pressure to normal. However, dyspnea soon recurred and subsequent hemodynamic measurements indicated increased right ventricular and left ventricular filling pressures. Echocardiography revealed no recurrent effusion or ventricular hypokinesis. Left ventricular ejection fraction by radionuclide ventriculogram was 64 percent. Echocardiography revealed ventricular wall thickening, normal chamber size, and glittering, sparkling myocardial echoes. On postmortem examination, there was extensive myocardial amyloidosis. There was no evidence of constrictive pericarditis or recurrent effusion. The unique aspect of this case was the combined presence of restrictive myocardiopathy and pericardial tamponade. To our knowledge, no previous case of tamponade due to amyloid heart disease had been reported.
 ...
PMID:Pericardial tamponade, a new complication of amyloid heart disease. 709 Nov 68


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>