Enzyme Replacement Therapy Decreases Left Ventricular Mass Index in Patients with Hunter Syndrome?

AbstractAlthough enzyme replacement therapy (ERT) has shown benefit in improving cardiac systolic function in a murine model of cardiomyopathy associated with Hunter syndrome, few studies have analyzed its effect in humans. We evaluated the effect of ERT on patients with Hunter syndrome-related cardiomyopathy. We performed a retrospective analysis of serial transthoracic echocardiograms performed before and over the first 5  years after treatment initiation, in 14 patients with Hunter syndrome. An important cardiac remodeling occurred in all patients in this study. There was a significant reduction in left ventricular mass index from 70.88 to 26.75 g/m2.7 (p = 0.003), with a trend towards a decrease in relative wall thickness from 0.515 at baseline to 0.370 after 5 years of enzyme therapy (p = 0.140). No differences were observed in aortic root diameter, left atrial diameter, left ventricular diastolic and systolic diameters, left ventricular ejection fraction, or myocardial performance index. Our findings demonstrate that ERT contributes to reducing left ventricular mass index in patients with Hunter syndrome.
Source: Mammalian Genome - Category: Genetics & Stem Cells Source Type: research

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Authors: Lizardo K, Ayyappan JP, Ganapathi U, Dutra WO, Qiu Y, Weiss LM, Nagajyothi JF Abstract Chagas disease is caused by Trypanosoma cruzi which is endemic in Latin America. T. cruzi infection results in a latent infection with approximately a third of latently infected patients developing chronic Chagas cardiomyopathy (CCM). CCM is a common cause of cardiomyopathy in endemic regions and has a poor prognosis compared to other cardiomyopathies. The factors responsible for the transition from the asymptomatic indeterminate latent stage of infection to CCM are poorly understood. Our previous studies demonstrated th...
Source: Disease Markers - Category: Laboratory Medicine Tags: Dis Markers Source Type: research
Publication date: Available online 17 January 2020Source: Canadian Journal of CardiologyAuthor(s): Sarah AM. Cuddy, Rodney H. FalkAbstractThe systemic amyloidoses are a group of diseases, all characterized by the deposition of amyloid, a material formed from misfolding of proteins, in one or more organs. The 2 commonest forms of amyloidosis, transthyretin amyloidosis (derived from wild-type {ATTRwt} or mutant transthyretin {ATTRm}) and light chain (AL) amyloidosis, derived from abnormal circulating light chains produced by a plasma cell dyscrasia. Both frequently involve the heart, producing an infiltrative cardiomyopathy ...
Source: Canadian Journal of Cardiology - Category: Cardiology Source Type: research
ConclusionBerberine could impede the development of dementia via multiple mechanisms: preventing brain damages and enhancing cognition directly in the brain, and indirectly through alleviating risk factors such as metabolic dysfunction, and cardiovascular, kidney and liver diseases. This study provided evidence to support the value of berberine in the prevention of dementia associated with MetS.
Source: Journal of Integrative Medicine - Category: Complementary Medicine Source Type: research
EXCEL, amyloid cardiomyopathy, drug prices, palliative care in cardiology, and gender bias are the topics covered by Dr John Mandrola in this week ’ s podcast.theheart.org on Medscape
Source: Medscape Cardiology Headlines - Category: Cardiology Tags: Cardiology Commentary Source Type: news
CONCLUSIONS: The degree of reverse remodelling was larger in patients with leads situated in a scar-free LCR. In patients with leads situated within scar there was a neutral effect on reverse remodelling, which can be caused both by higher scar burden or lead position. These findings demonstrate the feasibility of a CMR work-up and potential benefit in ICM patients undergoing CRT. PMID: 31953775 [PubMed - as supplied by publisher]
Source: Netherlands Heart Journal - Category: Cardiology Authors: Tags: Neth Heart J Source Type: research
In this study, we examined the mechanisms of SRV2-mediated mitochondrial fission in septic cardiomyopathy. Western blotting, ELISA, and immunofluorescence were used to evaluate mitochondrial function, oxidative balance, energy metabolism and caspase-related death, and siRNA and adenoviruses were used to perform loss- and gain-of-function assays. Our results demonstrated that increased SRV2 expression promotes, while SRV2 knockdown attenuates, cardiomyocyte death in LPS-induced septic cardiomyopathy. Mechanistically, SRV2 activation promoted mitochondrial fission and physiological abnormalities by upregulating oxidative inj...
Source: Aging - Category: Biomedical Science Authors: Tags: Aging (Albany NY) Source Type: research
Aims: One-third of DCM patients experience ventricular tachycardia (VT), but a clear biological basis for this has not been established. The purpose of this study was to identify transcriptome signatures and enriched pathways in the hearts of dilated cardiomyopathy (DCM) patients with VT.Methods and Results: We used RNA-sequencing in explanted heart tissue from 49 samples: 19 DCM patients with VT, 16 DCM patients without VT, and 14 non-failing controls. We compared each DCM cohort to the controls and identified the genes that were differentially expressed in DCM patients with VT but not without VT.
Source: Journal of Molecular and Cellular Cardiology - Category: Cytology Authors: Source Type: research
Condition:   Hypertrophic Cardiomyopathy Interventions:   Other: Asynchronous dual chamber pacing (DOO) with paced ventricular activation;   Other: Asynchronous atrial pacing (AOO) with intrinsic ventricular activation Sponsor:   Barts & The London NHS Trust Not yet recruiting
Source: ClinicalTrials.gov - Category: Research Source Type: clinical trials
Patients with nonischemic cardiomyopathy (NICM) often require an epicardial ventricular tachycardia (VT) ablation procedures via subxiphoid access. The coronary venous system (CVS) provides limited access to the epicardial space
Source: Heart Rhythm - Category: Cardiology Authors: Source Type: research
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Source: Current Cardiology Reports - Category: Cardiology Source Type: research
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