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

Cardiac involvement is well known in a number of skeletomuscular diseases but not in facio-scapulohumeral muscular dystrophy (FSHD). We report on a 71 year old woman with progressive cardiac insufficiency in FSHD, which was also confirmed by molecular analysis in one of the two daughters affected by the disease. Autopsy of the deceased patient showed the typical changes in skeletal muscles including focal inflammatory infiltrates in the diaphragm and, in addition, cardiac muscular involvement. The histological changes resembled those seen in primary cardiomyopathy despite the normal muscle mass volume. Both clinically and morphologically, the cardiac disease was the cause of death in this patient with FSHD.
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PMID:[Facioscapulohumeral muscle dystrophy and heart disease]. 1586 64

Non-compaction of the ventricular myocardium is an anomaly of myocardial morphogenesis that leads to persistence of the embryonic myocardium with an excessively prominent trabecular meshwork and deep intertrabecular recesses. This report describes 3 cases of isolated left ventricular non-compaction in adults leading to terminal heart failure. We describe their distinctive myocardial histopathology and prospective diagnosis by cardiac magnetic resonance imaging (CMR). Heart transplantation was the only treatment option for all 3 of these severely ill patients. Isolated ventricular non-compaction should be considered in patients with severe idiopathic cardiomyopathy whose symptoms appear in early adulthood, and consideration given for early transplantation. Non-invasive imaging with CMR can confirm the diagnosis.
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PMID:Isolated ventricular non-compaction in adults with idiopathic cardiomyopathy: cardiac magnetic resonance and pathologic characterization of the anomaly. 1594 42

Twelve patients with dilated cardiomyopathy and heart failure subjected to cardiac surgery received the CorCap Cardiac Support Device (CSD), a polyester fabric with bi-directional compliance placed around the dilated, failing heart. Patients with ischemic cardiomyopathy (n = 6) additionally underwent coronary artery bypass surgery receiving 1-3 bypass grafts. In the idiopathic cardiomyopathy group (n = 6) mitral valve plasty was performed in four patients in combination with the CSD, while two patients received the CSD only. Twelve months follow-up revealed a gradual, sustained improvement in cardiac dimensions and functional improvement as well as in quality of life. We conclude that addition of the CSD to conventional cardiac surgery, or applied alone, seems to reverse ventricular dilatation and improve functional capacity and well being of heart failure patients with dilated cardiomyopathy. Further studies will delineate what patient population will best benefit from passive containment surgery using the CSD.
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PMID:[Passive volume-reducing surgery in heart failure and dilated cardiomyopathy. Results from a pilot study account for careful optimism]. 1616 26

Although mechanical circulatory support (MCS) can improve myocardial function in patients with advanced heart failure, its effects on relative myocardial perfusion are unclear. Using positron emission tomographic imaging techniques, the authors assessed relative myocardial perfusion in patients with ischemic or idiopathic cardiomyopathy who were receiving chronic MCS with a left ventricular assist device (pulsatile HeartMate [n = 2] [Thoratec Corporation, Pleasanton, CA] or nonpulsatile Jarvik 2000 [n = 4] [Jarvik Heart, Inc., New York, NY]). Relative myocardial perfusion was compared at lower and higher levels of MCS (50 vs. 100 - 110 ejections/min for the HeartMate and 8000 vs. 12,000 rpm for the Jarvik 2000). The size and severity of perfusion defects at rest and after dipyridamole stress were measured objectively and subjectively by computer algorithms and visual inspection, respectively. Relative myocardial perfusion increased > 5% from baseline in only one of six patients when MCS was increased. No change in relative myocardial perfusion of > 5% was seen in any of the other five patients, even after subsequent dipyridamole stress positron emission tomographic imaging. These pilot study findings suggest that the decreased metabolic requirements induced by ventricular unloading correspondingly decreased blood flow requirements to physiologically inactive myocardium.
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PMID:Myocardial perfusion as assessed by positron emission tomography during long-term mechanical circulatory support. 1659 39

Endothelin-1 (ET-1) exerts multiple biological effects, including vasoconstriction and the stimulation of cell proliferation in tissues both within and outside of the cardiovascular system. ET-1 is synthesized by ET-converting enzymes (ECE), chymases (CMAs), and non-ECE metalloproteases through a process regulated in an autocrine fashion in vascular and nonvascular cells. ET-1 acts through the activation of G(i)protein-coupled receptors. ET(A) receptors mediate vasoconstriction and cell proliferation, whereas ET(B) receptors are important for aldosterone secretion, endothelial cell (EC) migration, the release of nitric oxide (NO) and prostacyclin, the clearance of ET-1, and the inhibition of ECE-1. ET is activated in scleroderma, hypertension, atherosclerosis, restenosis, heart failure, idiopathic cardiomyopathy, and renal failure. Tissue concentrations more reliably reflect the activation of the ET system because of the predominantly abluminal secretion of the peptide. Experimental studies and clinical trials have demonstrated that ET-1 plays a major role in normal cardiovascular homeostasis and in the functional and structural changes observed in arterial and pulmonary hypertension, glomerulosclerosis, atherosclerosis, and heart failure. Accordingly, ET antagonists are promising new agents in the treatment of cardiovascular diseases. Single nucleotide polymorphisms (SNPs) of the genes of preproET-1, ECE-1, CMA, ET(A) and ET(B) receptors have been identified and can be important for their functional regulation. However, for most of them the association with disease conditions and the evidence for a functional role remain controversial. Thus, even though ET antagonists are being used for the treatment of pulmonary hypertension, there is no convincing evidence for a role of SNPs in affecting the therapeutic strategies.
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PMID:Genetic variation in the endothelin system: do polymorphisms affect the therapeutic strategies? 1685 33

Potential regulation of two factors linked to physiological outcomes with left ventricular (LV) hypertrophy, resistance to apoptosis, and matching of metabolic capacity, by the transcription factor cyclic-nucleotide regulatory element binding protein (CREB), was examined in the two models of physiological LV hypertrophy: involuntary treadmill running of female Sprague-Dawley rats and voluntary exercise wheel running in female C57Bl/6 mice. Comparative studies were performed in the models of pathological LV hypertrophy and failure: the spontaneously hypertension heart failure (SHHF) rat and the hypertrophic cardiomyopathy (HCM) transgenic mouse, a model of familial idiopathic cardiomyopathy. Activating CREB serine-133 phosphorylation was decreased early in remodeling in response to both physiological (decreased 50-80%) and pathological (decreased 60-80%) hypertrophic stimuli. Restoration of LV CREB phosphorylation occurred concurrent with completion of physiological hypertrophy (94% of sedentary control), but remained decreased (by 90%) during pathological hypertrophy. In all models of hypertrophy, CREB phosphorylation/activation demonstrated strong positive correlations with 1) expression of the anti-apoptotic protein bcl-2 (a CREB-dependent gene) and subsequent reductions in the activation of caspase 9 and caspase 3; 2) expression of peroxisome proliferator-activated receptor-gamma coactivator-1 (PGC-1; a major regulator of mitochondrial content and respiratory capacity), and 3) LV mitochondrial respiratory rates and mitochondrial protein content. Exercise-induced increases in LV mitochondrial respiratory capacity were commensurate with increases observed in LV mass, as previously reported in the literature. Exercise training of SHHF rats and HCM mice in LV failure improved cardiac phenotype, increased CREB activation (31 and 118%, respectively), increased bcl-2 content, improved apoptotic status, and enhanced PGC-1 content and mitochondrial gene expression. Adenovirus-mediated expression of constitutively active CREB in neonatal rat cardiac recapitulated exercise-induced upregulation of PGC-1 content and mitochondrial oxidative gene expression. These data support a model wherein CREB contributes to physiological hypertrophy by enhancing expression of genes important for efficient oxidative capacity and resistance to apoptosis.
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PMID:Restoration of CREB function is linked to completion and stabilization of adaptive cardiac hypertrophy in response to exercise. 1733 97

The study was designed to evaluate the prognostic value of the 6-minute walk test in stable outpatients with heart failure. We prospectively studied 43 patients (6 women and 37 men) who had chronic heart failure secondary to ischemic heart disease or idiopathic cardiomyopathy. All patients had left ventricular systolic dysfunction (ejection fraction, < or = 0.40), and they were in stable New York Heart Association functional class II or III heart failure. All patients were evaluated by M-mode and 2-dimensional echocardiography. At the outset, walking distances of all the patients were evaluated by the 6-minute walk test. The patients were divided into 2 groups: Group I, patients with a 6-minute walk test distance of < or = 300 m; and Group II, patients with a 6-minute walk test distance of > 300 m. The patients were then monitored for a period of 2 years in regard to cardiac death. The mortality rate was significantly higher in patients with a 6-minute walk test distance of < or = 300 m than in patients with a 6-minute walk test distance of > 300 m (79% vs 7%; P <0.001). The death risk was found to be significantly higher in patients with a distance of < or = 300 m (P=0.005). The death risk was also higher in patients whose left ventricular ejection fraction was < or = 0.30 (P=0.02). We conclude that a 6-minute walk test distance of < or = 300 m is a simple and useful prognostic marker of subsequent cardiac death in patients with mild-to-moderate heart failure.
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PMID:Prognostic value of 6-minute walk test in stable outpatients with heart failure. 1762 62

A 17-year-old male patient presented to his primary care provider with heart failure symptoms and was transferred to our hospital with the diagnosis of idiopathic cardiomyopathy. His workup identified a large mediastinal mass with right ventricular outflow obstruction, which was resected. The pathology of the mass was a low-grade chondrosarcoma. The patient currently remains disease free at 4 years.
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PMID:Chondrosarcoma masquerading as cardiomyopathy. 1857 45

To evaluate the influence on circulating plasma levels of natriuretic peptides following passive containment surgery in heart failure patients with dilated cardiomyopathy, thirteen patients with dilated cardiomyopathy subjected to cardiac surgery received the Acorn Cardiac Support Device. Patients with ischemic cardiomyopathy (n=7) underwent coronary artery bypass surgery receiving 2-3 bypass grafts. In the idiopathic cardiomyopathy group (n=6), mitral valve plasty was performed in five patients while one patients received the Cardiac Support Device only. Circulating plasma atrial natriuretic peptide, brain natriuretic peptide and C-type natriuretic peptide were measured in all patients before surgery and 12 months postoperatively. Following surgery there was a significant decrease in circulating plasma levels of brain natriuretic peptide (0.14+/-0.04 ng/ml vs. 0.06+/-0.03 ng/ml, P<0.05). No significant changes were seen in circulating plasma levels of atrial natriuretic peptide or C-type natriuretic peptide. NYHA functional class improved (2.7+/-0.1 vs. 1.8+/-0.2, P<0.001). The 6-min-walk increased (354+/-35 m vs. 473+/-31 m, P<0.01). There was a decrease in left ventricular end diastolic diameter (73+/-2 mm vs. 65+/-2 mm, P<0.001) and left ventricular end systolic diameter (65+/-2 mm vs. 56+/-3 mm, P<0.01). Following passive containment surgery using the ACORN Cardiac Support Device functional improvement and reversed remodelling is accompanied by decreased BNP levels.
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PMID:Changes in natriuretic peptides following passive containment surgery in heart failure patients with dilated cardiomyopathy. 1900 26

Hypertrophic cardiomyopathy (HCM) is classified as a primary cardiomyopathy. HCM is a clinically heterogeneous but relatively common autosomal dominant genetic heart disease that probably is the most frequently occurring cardiomyopathy. HCM is characterized morphologically and defined by a hypertrophied, nondilated left ventriculum (LV) in the absence of another systemic or cardiac disease that is capable of producing the magnitude of wall thickening evident (e.g., systemic hypertension, aortic valve stenosis). Most HCM patients have the propensity to develop dynamic obstruction to LV outflow under resting or physiologically provocable conditions, produced by systolic anterior motion of the mitral valve with ventricular septal contact. The phenotypic features of HCM may develop at any age from infancy to adulthood, and are characterized by a great heterogeneity in the extent, magnitude, and distribution of left ventricular hypertrophy. Hypertrophic obstructive cardiomyopathy (HOCM) often leads to heart failure, severe ischemia, severe symptoms and death. Determination of the exact site of the hypertrophy and of the obstruction of the left ventricular outflow tract, in asymmetric septal hypertrophy, establishes which is the best treatment strategy. In the treatment of HOCM, drug therapy with negatively inotropic drugs, percutaneous transluminal septal myocardial ablation by alcohol-induced septal branch occlusion, surgical myectomy and DDD pacemaker therapy are considered the therapeutical options. We present a case of an obstructive hypertrophic cardiomyopathy in an 84-year-old Italian woman with a left ventricular outflow tract (LVOT) peak gradient with the Valsalva maneuver of 188 mm Hg and with a history of first episode of syncope.
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PMID:Revelation of an obstructive hypertrophic cardiomyopathy in an elderly patient. 1918 3


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