1/29. Outpatient management of heart failure.The outpatient treatment of heart failure can be divided into 2 broad categories: older therapies, which improve hemodynamics, and newer therapies, which increase survival and improve function. Hemodynamic "triple" therapy includes digoxin to increase cardiac inotropy, antihypertensives to lower systolic pressure, and diuretics to remove fluid and decrease filling pressures. Disease-modifying therapy requires the use of specific agents to lower blood pressure (angiotensin-converting enzyme inhibitors, angiotensin ii blockers, spironolactone, or hydralazine and nitrates) and beta-adrenergic blockade with carvedilol. The success of these newer therapies suggests that the standard triple therapy for heart failure should be expanded to "quadruple" therapy that includes carvedilol.- - - - - - - - - - ranking = 1keywords = iron (Clic here for more details about this article) |
2/29. Cardiac transplantation in a patient with hereditary hemochromatosis: role of adjunctive phlebotomy and erythropoietin.We describe the case of a 36-year-old woman with hereditary hemochromatosis (HH) resulting in end-stage cardiomyopathy and treated successfully with orthotopic cardiac transplantation. Before and after transplantation, the patient underwent aggressive treatment with frequent phlebotomy. We used erythropoietin concomitantly to maintain adequate hematocrit to support continued phlebotomy. We believe that aggressive use of phlebotomy provided the patient hemodynamic benefit and hastened the return of endocrine function post-transplantation. We also believe that the patient's history of high-dose vitamin C usage may have accelerated iron deposition in the heart and other vital organs.- - - - - - - - - - ranking = 1keywords = iron (Clic here for more details about this article) |
3/29. An aviator with cardiomyopathy and genetic susceptibility to hereditary hemochromatosis: a case report.A 44-yr-old male pilot was diagnosed with non-ischemic cardiomyopathy, possibly as a complication of hereditary hemochromatosis, 8 yr after an acquired left bundle branch block was discovered on a routine ECG. Biochemical testing returned high levels of iron and percentage transferrin saturation, and genetic testing for hemochromatosis was remarkable for a heterozygous H63D mutation in the HFE gene on chromosome 6. Hereditary hemochromatosis should be considered in the differential diagnosis when a patient presents with cardiomyopathy and genetic testing for HFE gene variants influencing iron overload is now available as a clinical adjunct for diagnosis and patient management issues. Cardiomyopathy and symptomatic hemochromatosis are aeromedically disqualifying conditions in the U.S. Air Force; however, early identification of hereditary hemochromatosis susceptibility with biochemical or genetic diagnostic tests, followed by education in primary and secondary prevention, will prevent a significant proportion of the possible sequelae.- - - - - - - - - - ranking = 126.5361833264keywords = iron overload, overload, iron (Clic here for more details about this article) |
4/29. Treatment of cardiomyopathy and rhabdomyolysis in long-chain fat oxidation disorders using an anaplerotic odd-chain triglyceride.The current dietary treatment of long-chain fatty acid oxidation defects (high carbohydrate with medium-even-chain triglycerides and reduced amounts of long-chain fats) fails, in many cases, to prevent cardiomyopathy, rhabdomyolysis, and muscle weakness. We hypothesized that the apparent defect in energy production results from a depletion of the catalytic intermediates of the citric acid cycle via leakage through cell membranes (cataplerosis). We further hypothesized that replacing dietary medium-even-chain fatty acids (precursors of acetyl-CoA) by medium-odd-chain fatty acids (precursors of acetyl-CoA and anaplerotic propionyl-CoA) would restore energy production and improve cardiac and skeletal muscle function. We fed subjects with long-chain defects a controlled diet in which the fat component was switched from medium-even-chain triglycerides to triheptanoin. In three patients with very-long-chain acyl-coa dehydrogenase deficiency, this treatment led rapidly to clinical improvement that included the permanent disappearance of chronic cardiomyopathy, rhabdomyolysis, and muscle weakness (for more than 2 years in one child), and of rhabdomyolysis and weakness in the others. There was no evidence of propionyl overload in these patients. The treatment has been well tolerated for up to 26 months and opens new avenues for the management of patients with mitochondrial fat oxidation disorders.- - - - - - - - - - ranking = 26.556133267323keywords = overload (Clic here for more details about this article) |
5/29. Bilateral pleural effusion and pulmonary edema caused by a fistulous aneurysm of the ductus arteriosus.We report an unusual case of pulmonary edema and rapid collection of bilateral pleural effusion caused by a fistulous large aneurysm of the ductus arteriosus (DAA). The diagnosis was performed by contrast CT and aortography. The cause of pulmonary edema and effusion was thought to be both elevated capillary pressure due to overload of the pulmonary circulation and decreased water clearance due to compression of the lymphatic system by the large DAA itself. Therefore, fistulous DAA should be considered when a continuous heart murmur and swelling in the aortic window are recognized. Once DAA is diagnosed, surgery should be performed without delay.- - - - - - - - - - ranking = 26.556133267323keywords = overload (Clic here for more details about this article) |
6/29. Successful combined heart-liver transplantation in a patient with hemochromatosis.hemochromatosis is a disorder of excess iron deposition in tissues that may cause multiorgan dysfunction. Because the early symptoms of hemochromatosis are nonspecific, the diagnosis is frequently overlooked until significant organ failure has developed. The primary cause of death in these patients is usually liver cancer related to cirrhosis. patients who are candidates for liver transplantation should be referred for evaluation. For patients with severe cardiomyopathy, in addition to end-stage liver disease, combined transplantation may be performed. Although there is a limited number of combined heart-liver transplantations that have been performed, successful outcomes can be achieved with close monitoring and a multidisciplinary team approach. In this case report, we will discuss the prevalence, pathophysiology, and treatment of hemochromatosis and potential complications of combined heart-liver transplantation.- - - - - - - - - - ranking = 1keywords = iron (Clic here for more details about this article) |
7/29. Rapidly advancing invasive endomyocardial aspergillosis.The exposure to aspergillus organisms/spores is likely common, but disease caused by tissue invasion with these fungi is uncommon and occurs primarily in the setting of immunosuppression. We report a case of rapidly advancing invasive endomyocardial aspergillosis secondary to prolonged usage of multiple broad-spectrum antibiotics in a nonimmunocompromised host. A 36-year-old cotton textile worker presented to our institution with a 3-month history of weight loss and fatigue. He reported receiving prolonged use of multiple broad-spectrum antibiotic treatment. The echocardiogram demonstrated multiple endomyocardial vegetations and a mass in the left atrium. Myocardial biopsy specimen revealed an invasive endomyocardial aspergillosis. The patient was investigated for immune deficiency including hiv, and this workup was negative. Treatment was started with amphotericin b and heparin for presumed left atrial thrombus. The patient died because of a rupture of mycotic aneurysm that resulted in cerebral hemorrhage. This case illustrates the risk of an invasive fungal infection in a nonimmunocompromised host who is a prolonged user of antibiotics in the setting of environmental exposure of opportunistic invasive fungal infections.- - - - - - - - - - ranking = 1keywords = iron (Clic here for more details about this article) |
8/29. Cardiomyopathy associated with celiac disease.celiac disease or celiac sprue is predominantly a disease of the small intestine characterized by chronic malabsorption in genetically susceptible individuals who ingest grains containing gluten, such as wheat, barley, and rye. Although previously believed to be uncommon, celiac disease may be present in up to 1% of the general population. celiac disease is associated frequently with iron deficiency anemia, dermatitis herpetiformis, selective iga deficiency, thyroid disorders, diabetes mellitus, and various connective tissue disorders but is rarely associated with cardiomyopathy. We describe a patient with celiac disease associated with cardiomyopathy whose cardiac function improved substantially after treatment with a gluten-free diet. Cardiomyopathy associated with celiac disease is a serious and potentially lethal condition. However, with early diagnosis and treatment with a gluten-free diet, cardiomyopathy in patients with celiac disease may be completely reversible.- - - - - - - - - - ranking = 1keywords = iron (Clic here for more details about this article) |
9/29. early diagnosis of hemochromatosis-related cardiomyopathy with magnetic resonance imaging.The hallmark of hemochromatosis is the deposition of iron in multiple tissue types, most notably the skin, liver, pancreas, thyroid, and heart. Definitive diagnosis of iron deposition generally requires invasive methods, such as direct tissue biopsy. We describe a 40 year-old woman with end-stage liver disease secondary to hereditary hemochromatosis and alcohol abuse, who was referred to the cardiology service as part of an evaluation for orthotopic liver transplant. The patient had no cardiac history but a dobutamine stress echocardiogram, performed as a portion of the pre-operative cardiac evaluation, could not be completed due to intermittent, supraventricular tachycardia. Additional cardiac testing, including electrocardiography and resting echocardiography, raised suspicion for cardiomyopathy related to hemochromatosis but was non-diagnostic. Cardiac magnetic resonance (MR) of this patient revealed deposition of iron in the myocardium and established the diagnosis of hemachromatosis-related cardiomyopathy. These findings suggest that cardiac MR may be more sensitive than other non-invasive, diagnostic tools in the initial evaluation of hemochromatosis-related cardiomyopathy and may be used as an alternative to myocardial biopsy. We propose that conventional T1- and T2-weighted spin echo MR sequences can be used routinely as non-invasive modalities to assess the presence of iron deposition in the tissues of patients with hemochromatosis.- - - - - - - - - - ranking = 4keywords = iron (Clic here for more details about this article) |
10/29. cobalt cardiomyopathy. A report of two cases from mineral assay laboratories and a review of the literature.Two young men employed in the mineral assay industry developed non-inflammatory cardiomyopathy. By review of clinical findings, elicitation of occupational and environmental histories, work-site evaluations, and ascertainment of tissue cobalt levels, nevada public health authorities confirmed these cases to be due to occupational cobalt exposure. hair and heart cobalt levels were elevated for the cases, but control samples had no detectable cobalt. Excess ischemic heart disease mortality among cobalt-exposed workers may reflect misdiagnosis of cardiomyopathy.- - - - - - - - - - ranking = 1keywords = iron (Clic here for more details about this article) |
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