Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0018801 (heart failure)
 72,216 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Primary amyloidosis is an uncommon disease which can be considered a diagnostic challenge. A recently observed case, initially characterized by peripheral neuropathy and subsequently by heart failure and sudden death, is described.
 ...
PMID:[Primary amyloidosis. A case report]. 216 Jun 28

The authors describe the cases of two women suffering from AL amyloidosis with affection of the heart muscle in particular. In one of the patients there were also massive amyloid deposits in the lungs which is an exceptional finding. The observations confirm the adverse prognosis of amyloidosis, the rapid progression of the disease after the onset of symptoms of cardiac failure. The first cardial manifestation in both patients were palpitations. The first patient, but not the second one, had a myeloma confirmed on necropsy.
 ...
PMID:[Amyloid light-chain amyloidosis with primary involvement of the cardiorespiratory system--2 case reports]. 814 Jul 57

The authors describe a typical echocardiographic finding in restrictive infiltrative cardiomyopathy which after two years led to the diagnosis of primary amyloidosis in a 51-year-old woman. The predominating manifestations of the disease were renal damage with final failure, heart failure, monoclonal gammapathy and damage of other organs. Aimed treatment of amyloidosis and repeated haemodialysis did not prevent the patient's death from cardiac and renal failure. Primary amyloidosis was confirmed by histological examination and necropsy.
 ...
PMID:[Restrictive infiltrating cardiomyopathy in primary amyloidosis]. 814 Jul 58

The authors report the case of a female patient admitted for complete heart failure with a diagnosis of type AL amyloidosis associated with dysglobulinaemia. While in hospital, she developed femoral embolism found to be secondary to atrial thrombosis in the absence of any supraventricular arrhythmia. The frequency and mechanism of intracardiac thrombosis and embolic accidents in cardiac amyloidosis are discussed in the light of this case.
 ...
PMID:[Systemic embolism and thrombosis of the left atrium in a patient in sinus rhythm with cardiac amyloidosis]. 876 13

Amyloidosis results from protein infiltration of the extracellular space of organs and tissues. Several amyloidosis proteins have been identified. Protein AL, (deriving from immunoglobulin light chain), protein AA and prealbumin are the most involved in this disease. When AL amyloidosis involves the heart, the illness is often terminal. Most clinical symptoms are heart failure and arrhythmia or block conduction. This case was characterised by the unusual combination of hypertension and amyloidosis. The diagnosis suggested by the echocardiographic but was confirmed by the damaged organ's biopsy. The present case concerns a young woman, who has hypertension and a pulmonary oedema. The echocardiographic scan showed a septal hypertrophy with a shining and granite-like aspect which is compatible with heart amyloidosis. Systolic and diastolic disorder with mitral and aortic regurgitation were also revealed. The kidney and rectum biopsies confirmed amyloidosis AL of the Kappa dysglobulinemia type, without extraosseous plasmocytoma. The heart and kidney failure symptoms disappeared after treatment with diuretics and ACE inhibitors.
 ...
PMID:[Heart failure and arterial hypertension disclosing amyloidosis]. 929 35

We reviewed clinical presentation, investigations, therapy, prognosis and outcome of 232 patients with primary (AL) cardiac amyloidosis. There were 142 men and 90 women. Median age at presentation was 59 years (range 29-85). AL heart disease was unusual both in patients under the age of 40 (3.0%) and in non-Caucasians (6.5%). Fatigue and weakness were the commonest presenting symptoms. Hallmark features of periorbital ecchymoses and macroglossia were present in 12.5% and 27.2%, respectively. AL cardiac amyloidosis was unusual in isolation (3.9%), and most frequently patients had features of multiorgan dysfunction; heavy proteinuria and features of malabsorption predominating in this respect. Heart involvement represents the worst prognostic indicator, with a median survival from diagnosis of 1.08 years, falling to 0.75 years with the onset of heart failure. Current therapeutic procedures appear to prolong survival, with left ventricular wall thickness, mass and ejection fraction on echocardiography and late potentials on signal averaged electrocardiography of use in prognostic stratification. Cardiac involvement from AL amyloidosis is rapidly fatal. It should be suspected in all patients with heart failure who have wall thickening on echo, normal chamber sizes, low EKG voltages and evidence suggesting a multisystem disease.
 ...
PMID:The clinical features of immunoglobulin light-chain (AL) amyloidosis with heart involvement. 957 96

AL amyloidosis is a systemic disease caused by the deposition of fibrillar proteins in organs and tissues. These deposits are responsible for organic dysfunctions and may be rapidly lethal. Current therapy is unsatisfactory. We describe three clinical cases of multisystemic AL amyloidosis but with different initial presentation: nephrotic syndrome, cardiac failure and hepatic insufficiency. We review the literature about the clinical features, diagnostic methods and treatment of the disease.
 ...
PMID:[AL amyloidosis]. 1125 6

Four patients with AL amyloidosis underwent high-dose chemotherapy and autologous stem cell transplantation at our institutions. Here, we report the clinical courses and outcomes in these patients. Two patients with multi-organ amyloid deposits including cardiac involvement died within 12 days after high-dose chemotherapy. However, in the other two patients, one of whom was suffering from amyloid-related cardiac failure, a significant improvement of organ function was achieved.
 ...
PMID:Four patients with AL amyloidosis treated with high-dose chemotherapy and autologous stem cell transplantation. 1127 85

Cardiac amyloidosis usually presents with heart failure, but rarely leads to coronary insufficiency. The authors report a case of a 69-year-old Japanese woman with cardiac amyloidosis presenting as microvascular angina. She had exertional angina with positive exercise test and normal coronary angiograms. However, heart failure developed, and she died 3 years after symptom onset. On autopsy, coronary arteries were patent. In contrast to that of the epicardial coronary arteries, histologic examination of the heart revealed severe obstructive alterations of the intramural coronary arteries with amyloid. Furthermore, amyloid was present mainly in the endocardium and the intramural coronary arteries, but there was little present in the myocardium. This case was a rare AL amyloidosis. There have been only 4 reported cases of cardiac amyloidosis that presented with exertional angina, a positive exercise test, and normal coronary angiograms and histologic examination.
 ...
PMID:Cardiac amyloidosis presenting as microvascular angina--a case report. 1133 May 10

The heart is involved in more than one third of patients with primary (AL) amyloidosis at diagnosis and it is by far the most common cause of death. Rhythm and conduction abnormalities generally represent the terminal event. The aims of this study were to determine the spectrum of Holter abnormalities found in AL amyloidosis and to assess their prognostic significance, particularly in relation to sudden death. Fifty-one patients with AL amyloidosis were included, and all of them had a complete history, physical examination, two-dimensional echocardiography, and 24-hour Holter monitoring. Fifty-five percent of these patients had echographic signs of heart involvement and 23% had heart failure. Complex ventricular arrhythmias were found in 57% of patients, couplets in 29%, and nonsustained ventricular tachycardia in 18%. Overall median survival was 23.4 months. Congestive heart failure, echocardiographic abnormalities, and Holter abnormalities adversely affected survival. The multivariate analysis demonstrated that interventricular septum thickness and couplets were independent predictors of survival. The presence of couplets correlated with sudden death. Holter monitoring may contribute to assessing the prognosis of patients with AL amyloidosis.
 ...
PMID:Holter monitoring in AL amyloidosis: prognostic implications. 1152 8


1 2 3 4 5 6 Next >>