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

Type I familial amyloid polyneuropathy (FAP), or Andrade's disease, is an inherited autosomal dominant disease, always fatal, involving mixed progressive polyneuropathy associated with systemic amyloid deposits. The disease is secondary to mutations of a gene (located on chromosome 18) which encodes for a serum protein, transthyretin. This variant protein is essentially (> 90%) produced in the liver and constitutes the biological marker of the disease. Many surgical teams have established a liver transplantation program for this non-cirrhotic pathology. Between January and August 1994, we performed three orthotopic liver transplantations (OLT) in patients with FAP. The patients were men aged between 30 and 33 years and the mean duration of symptoms was 3 years. The diagnosis of FAP was confirmed by rectal biopsy and detection of the genetic mutation (PCR analysis). All patients presented a severe sensory, motor and autonomic neuropathy with major digestive and urologic dysfunction. Two other patients were not accepted for OLT because of advanced disease with ulcerous lesions of the inferior limbs and cardiopathy. All patients survived with excellent post-operative hepatic function. One month after OLT, one patient had hepatocellular rejection which responded favorably to steroids. Another patient developed post-transfusional B hepatitis 10 months after the graft, but without major hepatic lesions. In the three cases, we observed stabilization of the peripheral neuropathy and an objective improvement of the autonomic affection (neurogenic bladder, diarrhea). The patients who did not undergo transplantation died within a year. Thus, in patients suffering from familial amyloid polyneuropathy OLT must be performed, especially in the early stage of the disease and especially in young patients before serious neurological complications set in.
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PMID:[Orthotopic liver transplantation for familial Portuguese amyloidosis]. 928 38

Patients with non-insulin-dependent diabetes mellitus (NIDDM) and microalbuminuria (MA) are at increased risk of early death. In NIDDM patients without evidence of heart disease, we examined the links between MA and autonomic neuropathy (AN) and reduced heart rate variability (HRV), both of which have been linked to a poor prognosis. We have studied 43 asymptomatic NIDDM patients with MA and have matched them with 43 normoalbuminuric patients for age, gender, diabetes duration, and smoking status. AN was assessed by heart rate changes to deep breathing, Valsalva, and posture and blood pressure changes to posture and hand grip. Twenty-four hour Holter monitoring was used to evaluate HRV. Patients with MA showed evidence of AN and reduced HRV when compared with normoalbuminuric patients. In multivariate analysis, with measures of AN and HRV as outcome variables, Log albumin excretion rate was a significant independent predictor but stronger predictors were the presence of diabetic retinopathy, age, body mass index, claudication, alcohol consumption, and calcium channel blocker use. The presence of MA is linked to AN and reduced HRV in asymptomatic NIDDM patients. The nature of the relationship is complex, involving multiple relationships with other clinical parameters.
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PMID:Autonomic neuropathy in asymptomatic subjects with non-insulin-dependent diabetes mellitus and microalbuminuria. 980 45

Even though autonomic diabetic neuropathy is highly prevalent and a noted risk factor for cardiovascular morbidity and mortality, very little is known about factors associated with it. We carried out standard autonomic nervous system function tests by means of a computerized portable system on 55 diabetic patients (22 with type 1 diabetes, 33 with type 2 diabetes) who had no signs or symptoms of autonomic diabetic neuropathy and on 10 age- and sex-matched healthy control subjects. Test results of patients with type 1 diabetes did not differ significantly from those with type 2 diabetes. Of the clinical, metabolic, and anthropometric variables considered, only the duration of diabetes was inversely and independently correlated to deep breathing test scores (E:I ratio value of deep breathing 1.38-0.009. years of diabetes; R2 = 0.25). The duration of diabetes was inversely correlated to variations in orthostatic systolic blood pressure (r = -0.37, p < 0.01). The prevalence of diabetic retinopathy (score: 1 = no; 2 = yes) was significantly higher in the diabetic group with lower deep breathing values (1.8 +/- 0.3 vs 1.0 +/- 0.0; p < 0.01). The prevalence of ischemic electrocardiographic alterations (score: 1 = no; 2 = yes) was significantly higher in the diabetic group with a poorer orthostatic systolic blood pressure response (1.4 +/- 0.1 vs 1.2 +/- 0.1; p < 0.01). This study suggests that 1) autonomic neuropathy is correlated to disease duration; 2) type of diabetes, present level of metabolic compensation, and anthropometric characteristics do not seem correlated to this complication; 3) diabetic retinopathy and ischemic cardiopathy may be correlated to autonomic neuropathy.
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PMID:Factors associated with autonomic nervous function in type 1 and type 2 diabetic subjects free of clinical manifestations of autonomic neuropathy. 1034 1

Diabetic autonomic neuropathy can cause heart disease, gastrointestinal symptoms, genitourinary disorders, and metabolic disease. Strict glycemic control can slow the onset of diabetic autonomic neuropathy and sometimes reverse it. Pharmacologic and nonpharmacologic therapies are available to treat symptoms.
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PMID:Recognizing and treating diabetic autonomic neuropathy. 1171 32

The causes of accelerated atherogenesis in diabetes are unclear but the consequences in terms of cardiovascular morbidity and mortality are profound. Thus diabetes not only increases the risk of coronary heart disease but also increases the case fatality rate, ensuring that the majority of patients die of cardiovascular causes, often before the age of 50 years. The problem is compounded by autonomic neuropathy which alters the perception of cardiac pain, attenuating symptoms which are often atypical or absent. This may delay presentation or lead to inappropriate triage decisions such that access to defibrillators and specific treatment is denied. Central to the cardiovascular management of diabetes is vigorous risk factor modification although clear evidence that this leads to extra protection against coronary heart disease beyond that achieved in non-diabetic individuals has not been forthcoming. In other respects too, the management of diabetic patients with heart disease is underpinned by the same evidence-base as applies to non-diabetic patients, and it is noteworthy that 15-20% of the patients in most of the landmark clinical trials have been diabetic. Recently, however, trials such as the United Kingdom Prospective Diabetes Study (UKPDS), the Heart Outcomes Prevention Evaluation (HOPE) study, and the Diabetes Mellitus, Insulin Glucose Infusion in Acute Myocardial Infarction (DIGAMI) study have identified novel strategies for reducing cardiovascular risk in diabetes. These trials have already had a major impact on cardiological practice, emphasising the prime importance of blood pressure control and converting enzyme inhibition for reducing cardiovascular risk in diabetes as well as the value of insulin therapy for reducing mortality in diabetic myocardial infarction. Additional trials, already in progress, are expected to refine further the cardiovascular management of patients with diabetes in order to provide an effective challenge for a problem that shows no signs of going away.
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PMID:Diabetes. 1175 9

This study used noninvasive methods to assess disturbance in cardiovascular function in insulin-dependent diabetes mellitus (IDDM) patients. Cardiovascular function and the autonomic nervous system of 38 IDDM patients were assessed, and the presence or absence of left ventricular dysfunction was determined. Fifty-six percent of the patients were found to have autonomic neuropathy. Twelve percent had left ventricular diastolic dysfunction; none had left ventricular systolic dysfunction. Heart disease in IDDM patients was found to constitute a separate entity, termed diabetic cardiomyopathy. All IDDM patients with left ventricular diastolic dysfunction had evidence of autonomic neuropathy. However, there was no correlation with left ventricular systolic dysfunction. Also, there was no correlation between left ventricular dysfunction and microvascular complications of diabetes mellitus.
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PMID:Cardiovascular function in patients with insulin-dependent diabetes mellitus: a study using noninvasive methods. 1202 Nov 55

Both type 1 and type 2 diabetic patients have an increased incidence of ischemic heart disease and congestive heart failure. Cardiovascular disease accounts for up to 80% of the excess mortality in patients with type 2 diabetes. The burden of cardiovascular disease is especially pronounced in diabetic women. Factors that underlie diabetic heart disease include multiple vessel coronary artery disease, long-standing hypertension, metabolic derangements such as hyperglycemia and dyslipidemia, microvascular disease, and autonomic neuropathy. There is also increased sudden death associated with diabetes, which is due, in part, to the underlying autonomic neuropathy. This article reviews diabetic cardiac disease, with an emphasis on type 2 diabetes.
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PMID:Heart disease in diabetic patients. 1276 70

The detection of preclinical heart disease is a new direction in diabetes care. This comment describes the study by Vinereanu and co-workers in this issue of Clinical Science in which tissue Doppler echocardiography has been employed to demonstrate subtle systolic and diastolic dysfunction in Type II diabetic patients who had normal global systolic function and were free of coronary artery disease. The aetiology of early ventricular dysfunction in diabetes relates to complex intramyocardial and extramyocardial mechanisms. The initiating event may be due to insulin resistance, and involves abnormal myocardial substrate utilization and uncoupling of mitochondrial oxidative phosphorylation. Dysglycaemia plays an important role via the effects of oxidative stress, protein kinase C activation and advanced glycosylation end-products on inflammatory signalling, collagen metabolism and fibrosis. Extramyocardial mechanisms involve peripheral endothelial dysfunction, arterial stiffening and autonomic neuropathy. The clinical significance of the ventricular abnormalities described is unknown. Confirmation of their prognostic importance for cardiac disease in diabetes would justify routine screening for presymptomatic ventricular dysfunction, as well as clinical trials of novel agents for correcting causal mechanisms. These considerations could also have implications for patients with obesity and the metabolic syndrome.
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PMID:Ventricular dysfunction in early diabetic heart disease: detection, mechanisms and significance. 1283 96

The presence of a diabetic cardiomyopathy, independent of hypertension and coronary artery disease, is still controversial. This systematic review seeks to evaluate the evidence for the existence of this condition, to clarify the possible mechanisms responsible, and to consider possible therapeutic implications. The existence of a diabetic cardiomyopathy is supported by epidemiological findings showing the association of diabetes with heart failure; clinical studies confirming the association of diabetes with left ventricular dysfunction independent of hypertension, coronary artery disease, and other heart disease; and experimental evidence of myocardial structural and functional changes. The most important mechanisms of diabetic cardiomyopathy are metabolic disturbances (depletion of glucose transporter 4, increased free fatty acids, carnitine deficiency, changes in calcium homeostasis), myocardial fibrosis (association with increases in angiotensin II, IGF-I, and inflammatory cytokines), small vessel disease (microangiopathy, impaired coronary flow reserve, and endothelial dysfunction), cardiac autonomic neuropathy (denervation and alterations in myocardial catecholamine levels), and insulin resistance (hyperinsulinemia and reduced insulin sensitivity). This review presents evidence that diabetes is associated with a cardiomyopathy, independent of comorbid conditions, and that metabolic disturbances, myocardial fibrosis, small vessel disease, cardiac autonomic neuropathy, and insulin resistance may all contribute to the development of diabetic heart disease.
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PMID:Diabetic cardiomyopathy: evidence, mechanisms, and therapeutic implications. 1529 81

Patients with diabetes experience cardiac autonomic neuropathy that may affect the way they perceive the symptoms of unstable angina (UA). The purpose of this study was to examine symptom differences in patients with and without diabetes during an episode of UA. A convenience sample of 50 women and 50 men were recruited. Patients with diabetes were more likely to have a history of hypercholesterolemia (83% vs. 60%), prior history of heart disease (85% vs. 65%), and prior angiogram (85% vs. 67%). Patients with diabetes reported having less nausea (20% vs. 40%), less squeezing (25% vs. 48%) and less aching (25% vs. 45%) type pain, and more hyperventilation (27.5% vs. 11.7%). Other cardiac symptoms were similar between the groups. Further study of symptom presentation in patients with diabetes is warranted given their high levels of morbidity and mortality from cardiac disease.
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PMID:Symptoms of unstable angina in patients with and without diabetes. 1577 56


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