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Query: UMLS:C0018801 (heart failure)
 72,216 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The authors describe two cases with primary systemic amyloidosis advancing with the picture of progressing cardiac insufficiency, clinically not distinguished. Electron microscopic study of myocardium was performed. The ultrastructural data indicate that the most likely source for synthesis of amyloid in myocardium, in primary systemic amyloidosis, are the active fibroblasts, established in interstitium.
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PMID:[Electron microscopic study of the myocardium in 2 cases of primary systemic amyloidosis]. 403 92

An autopsy case of primary systemic amyloidosis with severe cardiac amyloidosis in a 75-year-old Japanese man is reported. The initial signs were dyspnea and cough, followed by general fatigue and loss of appetite. Heart failure, pleural effusion, and liver cysts were found on physiological examination. Autopsy findings were primary systemic amyloidosis with severe diffuse cardiac amyloidosis and cardiac hypertrophy (585 g). Focal necrosis by infarction was found in the liver and spleen. The cause of amyloidosis was unknown.
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PMID:Primary systemic amyloidosis with severe cardiac amyloidosis. 407 77

Even severe generalized amyloidoses are difficult to diagnose clinically. By way of example the case is reported of a 64-year-old patient hospitalized for severe abdominal pain who died within a few weeks of heart and circulatory failure. The unusual disease picture is dominated by three cardinal symptoms: large, hemorrhagic-bullous lesions of the skin, recurrent ventricular ulcers with perforation and local peritonitis, and progressive heart failure refractory to therapy. Pathologico-anatomically, amyloid deposits were found in all three organs and primary systemic amyloidosis was diagnosed. Stenotic deposits in the blood vessels and extensive involvement of the ventricular wall suggest that the ulcer is a consequence of the primary disease. The cases of amyloidosis found at autopsy at the Institute of Pathology, Zurich, over the last seven years are reviewed.
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PMID:[Stomach perforation in generalized primary amyloidosis]. 730 41

We reviewed the clinical records of 769 patients with primary systemic amyloidosis who had been examined at Mayo Clinic Jacksonville (Jacksonville, Florida) or Mayo Clinic Rochester (Rochester, Minnesota) during a 12-year period (1978 through 1989). Of these 769 patients, 59 (8%) had biopsy-established gastrointestinal amyloidosis, and 8 (1%) had symptomatic gastric amyloidosis. All eight patients with symptomatic gastric amyloidosis had hematemesis or prolonged nausea and vomiting in association with weight loss. Additional findings were gastroparesis (in three patients), gastric tumor (in one), and gastric outlet obstruction (in one). Macroglossia was present in two patients, and multiple myeloma was diagnosed in three. Six of the eight patients had coexisting small bowel amyloidosis and weight losses of 6.5 to 22.5 kg. Congo red staining identified gastric amyloid in the media of blood vessels in all cases. All cases stained selectively for lambda (seven cases) or kappa (one) light chain. All eight patients died; the median duration of survival after diagnosis was 13.8 months (range, 0.5 to 39.5). Death was due to cardiac failure (three patients), renal failure (two), chronic gastrointestinal obstruction and severe cachexia (two), or hepatic failure (one). Chemotherapy was given to seven patients but was only partially effective for ameliorating symptoms in one.
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PMID:Symptomatic gastric amyloidosis in patients with primary systemic amyloidosis. 833 78

In a 71-year-old woman with macroglossia and heart failure, a monoclonal protein (kappa-light chains) was found in the serum. Congestive heart failure was diagnosed on the basis of heart catheterization and echocardiography. The tongue biopsy showed numerous amyloid fibrils, and plasmacytosis was present in the bone marrow. All these findings indicated a restrictive cardiomyopathy as part of a primary systemic amyloidosis.
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PMID:[Primary amyloidosis AL as a cause of restrictive cardiomyopathy]. 945 48

Dose-intensive chemotherapy with PBSC support was recently reported to be feasible in cardiac amyloidosis with some patients achieving post-transplant improvement in performance status. At our center, 11 patients with symptomatic primary systemic amyloidosis and predominant cardiac involvement confirmed by biopsy or increased wall thickness on echocardiogram were evaluated for high-dose therapy. The average time from diagnosis to referral was 11 months (4-26 months). Of the 11 patients, two were not candidates for high-dose therapy, based on poor performance status. The remaining nine patients proceeded to PBSC collection. Three patients died during the mobilization period: two of rapid atrial fibrillation, and the third secondary to progressive heart failure. Six patients proceeded to transplantation. However, one died of sudden cardiac arrest the day of melphalan administration, one following hypotension related to stem cell infusion, and one of hypotensive shock the day following stem cell infusion. Three patients recovered and left the hospital, but one died of a cardiorespiratory event at home within 6 weeks of discharge. Both surviving patients demonstrate objective improvement. A decision to use high-dose therapy and stem cell support in cardiac amyloidosis must balance the substantial morbidity of the procedure with the potential benefits. Transplant regimens should avoid cardiotoxic agents such as cyclophosphamide and DMSO and patients should receive anti-arrythmic therapy.
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PMID:High treatment-related mortality in cardiac amyloid patients undergoing autologous stem cell transplant. 1051 96

We describe a 76-year-old Japanese woman with primary systemic amyloidosis who presented with angina pectoris associated with ST-segment and T-wave abnormalities resulting from intramyocardial coronary artery amyloidosis. The patient was admitted to our hospital because of dyspnea and pretibial edema 7 years after the diagnosis of variant angina. A diagnosis of primary systemic amyloidosis (AL amyloid protein) was made after examination of gastric and endomyocardial biopsy specimens. The patient died of progressive, uncontrolled heart failure 3 months later. An autopsy study demonstrated only mild-to-moderate atherosclerosis in the epicardial coronary arteries. However, histological examination of the heart revealed diffuse stenoses and obstructions in the intramural coronary arteries by amyloid deposits. This patient had small-vessel coronary disease with ST-segment changes and angina caused by cardiac amyloidosis. A correct diagnosis of ischemic heart disease due to primary amyloidosis is important for estimation of the prognosis and for appropriate management.
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PMID:Primary systemic amyloidosis presenting as angina pectoris due to intramyocardial coronary artery involvement: a case report. 1222 87

Cardiac amyloidosis is a well-known but clinically rare cause of heart failure that has historically been associated with a poor prognosis. Cardiac amyloidosis involves fibril formation from one of several underlying conditions, and the course of illness and prognosis varies among these conditions. Evolving treatment strategies for patients with primary systemic amyloidosis have given this subset of cardiac amyloidosis patients a cause for hope. The identification and appropriate referral of patients in whom this condition is suspected will help to improve the likelihood of successful therapy and long-term survival.
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PMID:Cardiac amyloidosis: shifting our impressions to hopeful. 1692 39

A 73-year-old woman was admitted due to exertional dyspnea. It was considered that a large amount of pericardial effusion caused diastolic heart failure; pericardial paracentesis showed bloody effusion. There were no findings of malignancy or other abnormal findings in the examination. Further examinations were planned but she died of ventricular tachycardia attack. Pathological autopsy revealed primary systemic amyloidosis. Pathologically it was possible that the local inflammation (epicarditis) due to the deposition of amyloid in the epicardium and perivascular tissue caused the bloody effusion. There are no reports of primary systemic amyloidosis with hemorrhagic pericardial effusion. We report this rare case with pathological consideration.
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PMID:Primary systemic amyloidosis with bloody pericardial effusion. 1944 78

We present a case of a 54-year-old female presenting with renal failure and, two years later, heart failure, both due to primary systemic amyloidosis. The case gives us the opportunity to review the litterature on the topic.
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PMID:[Cardiac amyloidosis]. 1994 12


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