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

Cardiac amyloidosis produces a restrictive cardiomyopathy with impaired diastolic function. We report a case in which low-dose verapamil resulted in marked worsening of congestive heart failure, as a result of a profound negative inotropic effect. Withdrawal of verapamil therapy demonstrated a return of systolic function to normal with improvement in heart failure. We postulate that patients with cardiac amyloidosis may be exceptionally sensitive to the negative inotropic effects of calcium-channel blockers either because of abnormal binding to amyloid fibrils or because their usual vasodilator effects are blunted.
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PMID:Left ventricular systolic dysfunction precipitated by verapamil in cardiac amyloidosis. 833 58

A combination of hypertrophic obstructive cardiomyopathy (HOCM) and cardiac amyloidosis in the same patient is very rare. Clinical diagnosis could be extremely difficult and may require myocardial biopsy. We are reporting a patient with this combination who was referred to our institution because of features of HOCM based on clinical, echocardiographic and Doppler criteria. Cardiac amyloidosis was only recognized after myocardial biopsy that failed to reveal evidence of HOCM. Only after the patient expired from severe, intractable heart failure did the autopsy findings confirm the association of HOCM. We believe that the combination of the two cardiomyopathic processes is very rare and makes treatment extremely difficult.
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PMID:Double cardiomyopathy: coexistent cardiac amyloidosis and hypertrophic obstructive cardiomyopathy. 843 28

Amyloid cardiomyopathy may exist when echocardiography does not suggest infiltration. Clinicians should be alert for the presence of a monoclonal protein, nephrotic range proteinuria, or peripheral neuropathy in a patient with heart failure.
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PMID:Endomyocardial biopsy-proven light chain amyloidosis (AL) without echocardiographic features of infiltrative cardiomyopathy. 920 31

An 83 year old white man with atrial fibrillation was admitted to hospital after a cerebral infarct. Echocardiography was characteristic of cardiac amyloid deposition and subsequent tests confirmed amyloidosis of transthyretin (TTR) type, in association with the Ile122 mutation of the TTR gene; this has only been reported previously in African Americans in whom it occurs with an allele frequency of 2%. Haplotype analysis did not suggest a different founder than for the African Ile122 mutation. Cardiac amyloidosis should be considered among elderly patients presenting with cardiac failure and/or arrhythmia, particularly if they are resistant to conventional treatment; if confirmed, it should be followed by precise characterisation of amyloid fibril type. The prevalence of autosomal dominant cardiac TTR amyloidosis in elderly white people is unknown but early diagnosis and supportive treatment may prevent complications among affected family members.
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PMID:Hereditary cardiac amyloidosis associated with the transthyretin Ile122 mutation in a white man. 1045

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.
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PMID:Cardiac amyloidosis presenting as microvascular angina--a case report. 1133 May 10

Cardiac amyloidosis should be considered in a patient with heart failure, who is normotensive with decreased left ventricular systolic function and marked left ventricular hypertrophy by echocardiogram and has decreased voltage by ECG. Furthermore, when the diagnosis of cardiac amyloid is made, it is important to classify the subtype of disease to be able to offer appropriate treatment. Contrary to traditional belief that the prognosis for patients with amyloidosis is dismal, some forms of this disease are curable and other forms are characterized by slow progression of disease.
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PMID:Cardiac amyloidosis. 1138 76

In a post-mortem analysis of 1,500 patients, aged 70 years or more, heart disease and malignancy were the two major causes of death. Coronary disease was exceedingly prevalent, almost one in five being subject to this form of heart disease, and 13% dying from its effects. The levelling of the sex ratio in coronary disease in the aged was a striking feature. The clinical manifestations of coronary thrombosis were often atypical. The incidence of demonstrable coronary thrombosis was as high as in younger age groups. The pathological findings and the incidence of local complications in coronary disease were similar to those in other age groups. Other causes of heart failure were relatively infrequent. Certain features of these conditions in old age are discussed. Cardiac amyloidosis, in the form associated with senility, occurred in a very few cases but did not contribute to heart failure.
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PMID:HEART DISEASE IN OLD AGE. 1407 70

Primary amyloidosis is a systemic disorder characterized by the clonal production and tissue deposition of immunoglobulin light chain (LC) proteins. Congestive heart failure remains the greatest cause of death in primary amyloidosis, due to the development of a rapidly progressive amyloid cardiomyopathy. Amyloid cardiomyopathy is largely unresponsive to current heart failure therapies, and is associated with a median survival of less than 6 months and a 5-year survival of less than 10%. The mechanisms underlying this disorder, however, remain unknown. In this report, we demonstrate that physiological levels of human amyloid LC proteins, isolated from patients with amyloid cardiomyopathy (cardiac-LC), specifically alter cellular redox state in isolated cardiomyocytes, marked by an increase in intracellular reactive oxygen species and upregulation of the redox-sensitive protein, heme oxygenase-1. In contrast, vehicle or control LC proteins isolated from patients without cardiac involvement did not alter cardiomyocyte redox status. Oxidant stress imposed by cardiac-LC proteins further resulted in direct impairment of cardiomyocyte contractility and relaxation, associated with alterations in intracellular calcium handling. Cardiomyocyte dysfunction induced by cardiac-LC proteins was independent of neurohormonal stimulants, vascular factors, or extracellular fibril deposition, and was prevented through treatment with a superoxide dismutase/catalase mimetic. This study suggests that cardiac dysfunction in amyloid cardiomyopathy is directly mediated by LC protein-induced cardiomyocyte oxidant stress and alterations in cellular redox status, independent of fibril deposition. Antioxidant therapies or treatment strategies aimed at eliminating circulating LC proteins may therefore be beneficial in the treatment of this fatal disease.
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PMID:Human amyloidogenic light chains directly impair cardiomyocyte function through an increase in cellular oxidant stress. 1504 25

Amyloidosis is a term for diseases with extracellular deposition of insoluble beta-fibrillar proteins in different organs. The heart is primarily involved in more than half of patients with immunoglobulin light-chain amyloidosis or hereditary amyloidosis and associated with poor prognosis. Different traditional diagnostic tools that have been described for risk stratification lack of sufficient sensitivity and specificity for patient survival. Until November 2004 in 50 consecutive patients with light chain amyloidosis and 15 patients with hereditary amyloidosis electrocardiography, echocardiography, Holter monitoring, cardiopulmonary exercise test, lung function testing, tilt-test, and laboratory investigations have been performed at our department. Cardiac amyloidosis was found in 32 patients. Interventricular septum (14.3+/-0.5 mm vs. 12.3+/-0.7 mm, P<0.05), plasma NT-proBNP (7154+/-2122 ng/l vs. 380+/-113 ng/l; P<0.01), cardiac Troponin T (0.105+/-0.030 vs. 0.019+/-0.010 microg/l; P<0.05) were increased in patients with cardiac amyloidosis as compared to patients light chain amyloidosis but no cardiac involvement. Maximal inspiratory (Pimax) and expiratory (Pemax) mouth pressure were decreased with CA compared to controls. Correlation of NT-proBNP and interventricular septum thickness (r=0.53, P=0.029) as well as and Pimax (r=0.72, P<0.01) or Pemax (r=0.69; P<0.01) was noticed. A correlation of grade of arrhythmias in Holter monitoring and syncopes was not observed. Cardiac involvement of amyloid disease carries a poor prognosis and is not well characterized by classic heart failure determinants. Heart transplantation based on novel risk markers including NT-proBNP might be a suitable therapeutic approach for patients with manifest cardiac amyloidosis, but will require alternative patient selection and listing criteria.
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PMID:Risk stratification in cardiac amyloidosis: novel approaches. 1628 95

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


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