Is left ventricular longitudinal strain a good prognostic factor in Friedreich ataxia?

Publication date: June 2019Source: Archives of Cardiovascular Diseases Supplements, Volume 11, Issue 3Author(s): C. Heuze, L. Legrand, A. Diallo, M.L. Monin, C. Ewenczyk, R. Isnard, E. Vicaut, A. Durr, F. PoussetIntroductionFriedreich ataxia (FRDA) is a rare genetic ataxia. The causal mutation is an expanded trinucleotide repeat (GAA) in the frataxin gene. Hypertrophic cardiomyopathy in FRDA is the major cause of early death. Patients with progressive decline of the left ventricular ejection fraction (LVEF) have the worst prognosis. The aim of the study was to evaluate the prognostic value of 2D global longitudinal strain (GLS) compared to LVEF in FRDA patients.MethodFrom 2003 to 2017 consecutive patients with genetically confirmed FRDA were included. GLS was retrospectively performed. News was obtained for all patients until April 2018.ResultsThe study included 156 patients of 35 ± 12 years (mean ± SD) with an age at disease onset of 17 ± 11 y and GAA repeat on the shorter allele of 590 ± 241 pb. The following echocardiographic parameters were studied: LVEF 64 ± 9%, GLS -19.8 ± 5% (n = 141), septal wall thickness (SWT) 11.4 ± 2.5 mm, posterior wall thickness (PWT) 10.4 ± 1.8 mm, LV end diastolic diameter (LVEDD) 44.4 ± 6 mm. Correlation between GLS and LVEF was 0.31 (P = 0.0002).After a mea...
Source: Archives of Cardiovascular Diseases Supplements - Category: Cardiology Source Type: research

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Strokes remain a leading cause of morbidity and mortality in patients with ventricular assist devices (VADs). Varying study populations, event definitions, and reporting methods make direct comparison of neurologic event risk across clinical trials and registries challenging. We aim to highlight important differences among major VAD studies and standardize rates of neurologic events to facilitate a comprehensive and objective comparison. We systematically identified and analyzed key clinical trials and registries evaluating the HeartMate II (HMII), HeartMate 3 (HM3), and HVAD devices. Reported neurologic events were nonexc...
Source: ASAIO Journal - Category: Medical Devices Tags: Adult Circulatory Support Source Type: research
Dysfunction of different components within continuous-flow (CF) left ventricular assist device (LVAD) systems may cause adverse cardiovascular and end-organ sequelae. Outflow graft obstruction is a recognized type of LVAD component dysfunction. Ten patients were admitted and treated for LVAD outflow graft obstruction. Two of these patients subsequently developed recurrent outflow graft obstruction requiring reintervention; however, each reoccurrence was at a different site than the original obstruction. Thus, a total of 12 cases of obstruction were analyzed. The most common reasons for hospital admission were low flow LVAD...
Source: ASAIO Journal - Category: Medical Devices Tags: Adult Circulatory Support Source Type: research
Ventricular assist devices (VADs) are an increasingly common therapy for end-stage heart failure across all ages as a bridge to recovery or transplant and more recently as destination therapy. With increasing experience and difficulties with establishing therapeutic heparin levels, we have begun to explore the effectiveness of direct thrombin inhibitors in this patient population. This is a retrospective review of all long-term VAD patients, both adult and pediatric, who were anticoagulated with bivalirudin between January 2009 and January 2016. The starting dose was 0.3 mg/kg/hr, and dose was titrated for a goal pa...
Source: ASAIO Journal - Category: Medical Devices Tags: Pediatric Circulatory Support Source Type: research
We present three cases that developed moderate to severe AI while on HeartMate II (Abbott Laboratories, Chicago, IL) LVAD support; hence, TAVR procedure was performed. Patients A and B (74 year old and 38 year old) developed severe AI and patient C (67 year old) developed moderate AI with cardiogenic shock, after 368, 1,288, and 342 days on LVAD support, respectively. Their aortic valve annulus sizes were 24.2, 24.6, and 23.3 mm, respectively. Oversized Edwards SAPIEN 3 valves (Edwards, Lifesciences, Irvine, CA) were implanted via a transfemoral approach. The patients were hemodynamically stable after the procedure ...
Source: ASAIO Journal - Category: Medical Devices Tags: Case Report Source Type: research
Abstract Cerebral venous thrombosis (CVT) is an uncommon cause of stroke that mainly affects young adults with known risk factors of prothrombotic conditions, pregnancy, infection, malignancy, and drugs. Dutasteride is a 5α-reductase inhibitor that is used for benign prostate hypertrophy and androgenetic alopecia. To date, CVT caused by dutasteride use has not been reported. A 25-year-old male presented with headache and diplopia. He had taken 0.5 mg of dutasteride every other day for 9 months to treat alopecia. A headache developed 7 months after he started taking medication, and horizontal diplopia occurre...
Source: Yonsei Medical Journal - Category: Universities & Medical Training Authors: Tags: Yonsei Med J Source Type: research
Publication date: Available online 29 May 2020Source: Journal of Dental SciencesAuthor(s): Min-Jeong Cho, Young-Seok Kim, Eun Young Park, Eun-Kyong Kim
Source: Journal of Dental Sciences - Category: Dentistry Source Type: research
Abstract With the popularization of rehabilitation robots, it is necessary to develop quantitative motor function assessment methods for patients with a stroke. To make the assessment equipment easier to use in clinics and combine the assessment methods with the rehabilitation training process, this paper proposes an anthropomorphic rehabilitation robot based on the basic movement patterns of the upper limb, point-to-point reaching and circle drawing movement. This paper analyzes patients' movement characteristics in aspects of movement range, movement accuracy, and movement smoothness and the output force charact...
Source: Biomed Res - Category: Research Authors: Tags: Biomed Res Int Source Type: research
Abstract The histone demethylase JMJD family is involved in various physiological and pathological functions. However, the roles of JMJD1A in the cardiovascular system remain unknown. Here, we studied the function of JMJD1A in cardiac hypertrophy. The mRNA and protein levels of JMJD1A were significantly downregulated in the hearts of human patients with hypertrophic cardiomyopathy and the hearts of C57BL/6 mice underwent cardiac hypertrophy induced by transverse aortic constriction (TAC) surgery or isoproterenol (ISO) infusion. In neonatal rat cardiomyocytes (NRCMs), siRNA-mediated JMJD1A knockdown facilitated ISO...
Source: Biomed Res - Category: Research Authors: Tags: Biomed Res Int Source Type: research
CONCLUSIONS: The prevalence of heart failure was less in 2017 than the 1.6% value for the year 2009. This can be due to the fact that this study collected patients treated or visited only this one year, while the other study in 2009 assessed patients diagnosed also in the previous 5 years but not appearing at any medical visit in 2009. Mortality rate data have also shown a remarkable improvement in comparison with the year 2009, but they still remained above the values reported for Western Europe. This improvement can be explained with the better up-titration of medications offering longer survival and the better adherence...
Source: Orvosi Hetilap - Category: General Medicine Authors: Tags: Orv Hetil Source Type: research
CONCLUSION: On the basis of a broadly based research and participatory development process, a set of indicators has been developed that enables comprehensive evaluation of the regional quality of care of cross-indication, integrated care models focusing on common diseases. In order to be able to increasingly evaluate aspects of care coordination and patient orientation, health promotion as well as nursing, palliative and emergency care in the future, it would be helpful if routine data were collected or made accessible in these areas as well. PMID: 32467041 [PubMed - as supplied by publisher]
Source: Zeitschrift fur Evidenz, Fortbildung und Qualitat im Gesundheitswesen - Category: Health Management Tags: Z Evid Fortbild Qual Gesundhwes Source Type: research
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