1,720,981 research outputs found
Hypersensitivity myocarditis following deferasirox administration
Full text linkWe describe a case of A 73-year-old woman, receiving regular blood transfusion for 2 years as affected by myelodysplasia and under treatment with deferasirox (14 mg/kg daily; 2 tablets of 360 mg/die) in the last 2 months due to the registration of high levels of ferritin (1000 μg/L) was admitted because of acute heart failure. She was on treatment only with deferasirox and previously the patient presented normal ECG and at echocardiogram normal cardiac parameters with a left ventricular (LV) ejection fraction of 64%
Downregulation of mannose-6-phosphate receptors in fabry dsease cardiomyopathy: a potential target for enzyme therapy enhancement
No full textBackground: The efficacy of enzyme replacement therapy (ERT) in mobilizing globotryaosylceramide (GB-3) from Fabry cardiomyocytes is limited. The mechanism involved is still obscure.
Methods: Assessment of M6Pr, M6Pr-mRNA, and Ubiquitin has been obtained by Western blot analysis and real-time PCR of frozen endomyocardial biopsy samples, from 17 pts with FD, various degree of left ventricular hypertrophy, and maximal wall thickening (MWT) from 11.5 and 20 mm. The diagnosis and severity of FDCM followed definitions of GLA mutation, α-galactosidase A enzyme activity, cardiac magnetic resonance, and left ventricular endomyocardial biopsy with the quantification of myocyte hypertrophy and the extent of Gb-3 accumulation. All patients have received alpha or beta agalsidase for ≥3 years without a reduction in LV mass nor an increase in T1 mapping at CMR. Controls were surgical biopsies from 15 patients undergoing mitral valve replacement.
Results: Protein analysis showed mean M6Pr in FDCM to be 5.4-fold lower than in a normal heart (4289 ± 6595 vs. 23,581 ± 4074, p = 0.0996) (p < 0.001): specifically, 9-fold lower in males, p = 0.009, (p < 0.001) and 3-fold lower in females, p = 0.5799, (p < 0.001) showing, at histology, a mosaic of normal and diseased cells. M6Pr-mRNA expression was normal, while ubiquitin showed an increase of 4.6 fold vs. controls (13,284 ± 1723 vs. 2870 ± 690, p = 0.001) suggesting that ubiquitin-dependent post-translational degradation is likely responsible for the reduction of M6Pr in FDCM.
Conclusion: M6Pr expression is remarkably reduced in FDCM as a likely result of post-translational degradation. This may explain the reduced efficacy of ERT and be a therapeutic target for the enhancement of ERT activity
Removal of cardiac AL-amyloid with positive remodeling of cardiomyocytes and of restrictive cardiomyopathy
Full text linkHerein, we describe histological mobilization of light chain cardiac amyloid documented by sequential left ventricular endomyocardial biopsies. These findings were associated with positive remodelling of cardiomyocytes and of restrictive cardiomyopathy resulting from 14 courses of chemotherapy over 17 years of time. Histological and ultrastructural findings of light
chain cardiac amyloid removal led to increase in cardiomyocyte dimension and electrocardiogram voltages, reduction of biventricular wall thickness with improvement of left ventricular diastolic function, and NYHA class shifting from III to I
Primary aldosteronism-associated cardiomyopathy: clinical-pathologic impact of aldosterone normalization
Full text linkBackground: Primary aldosteronism (PA) causes a cardiomyopathy (CM) which substrate and evolution after aldosterone normalization are unreported. Methods: Four male patients with aldosterone-secreting adrenal adenoma and cardiomyopathy (PACM, group A) were evaluated with 2D-echo, Magnetic Resonance (CMR), coronary angiography and left ventricular endomyocardial biopsy. Biopsy samples were processed for histology, electron microscopy, immunohistochemistry, and Western Blot analysis of myocardial aldosterone receptors and aquaporin 1 and 4. Results were compared with endomyocardial samples from 5 patients with hypertensive cardiomyopathy of equivalent severity and normal plasma aldosterone (group B) and surgical samples from 5 controls (group C). One PACM patient was re-examined with CMR and endomyocardial biopsy 12 months after adrenalectomy with aldosterone and cardiac normalization. Results: Coronary arteries were normal in all. Group A showed prominent myocardial hypertrophy and fibrosis, with water accumulation in the cytosol and organelles of cardiomyocytes and microvascular smooth muscle cells, associated to reduced myofibril concentration and 2.8-fold increase in myocardial aldosterone receptors and aquaporin 1. At CMR, LGE areas were diffusely present. After aldosterone normalization, cardiomyocyte diameter reduced with disappearance of intracellular vacuoles, recovery of electron-density of cytosol and cell organelles, and myofibrillar content, persisting fibrosis and down-regulation of aldosterone receptors and aquaporin 1 channels. At CMR, myocardial mass reduced with recovery of cardiac contractility. LGE signal remained unchanged. Conclusion: PACM is a reversible entity characterized by over-expression of aldosterone receptors and aquaporin 1. It induces a reversible intracellular water overloading causing impaired cardiomyocyte relaxation, contraction and ultrastructural integrity
Myocardial and microvascular inflammation/infection in patients with HIV-associated pulmonary artery hypertension
No full textRight ventricle compromise affects survival of patients with HIV-associated pulmonary artery hypertension (PAH)
A-V block as presentation of cardiac amyloid: prominent infiltration of conduction tissue revealed by endomyocardial biopsy
No full text
Heart failure from gouty myocarditis: a case report
No full textMyocarditis is an important cause of heart failure, and successful treatment depends on identifying the underlying cause and mechanism. Infectious and autoimmune diseases are the most common causes, although myocarditis can also be caused by endocrine disorders, such as pheochromocytoma; infiltrative disorders, such as amyloidosis; and metabolic disorders. Gout is a metabolic disorder characterized by increased uric acid in the blood and the deposition of amorphous urate crystals that cause a vigorous inflammatory reaction in the joints, kidneys, and soft tissues. Urate crystals have been found in coronary vessels and cardiac valves but, to our knowledge, not in the myocardium
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