Dataset Information

Left Ventricular Assist Device Support for Fabry Cardiomyopathy: A Case Series.

ABSTRACT: Patients with restrictive cardiomyopathy due to Fabry disease are often deemed ineligible for left ventricular assist device (LVAD) support due to the risk of suction events with a small LV cavity. We describe the first case series of LVAD support for Fabry disease. LVAD therapy can improve survival, quality of life, and provide clinical stability to start enzyme replacement therapy. Precautions at the time of surgery include rapid treatment of fever to avoid Fabry crises, involvement of a multidisciplinary team, and early initiation of rehabilitation. We describe LVAD support for both bridging and destination therapy.

SUBMITTER: Omid Kiamanesh MD

PROVIDER: S-EPMC7893197 | biostudies-literature | 2021 Feb

REPOSITORIES: biostudies-literature

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Left Ventricular Assist Device Support for Fabry Cardiomyopathy: A Case Series.

Omid Kiamanesh M D MD Moayedi Yasbanoo Y Aleksova Natasha N Posada Juan Duero JD Gershon Ariel A Seidman Michael A MA Rao Vivek V Billia Filio F

CJC open 20201006 2

Patients with restrictive cardiomyopathy due to Fabry disease are often deemed ineligible for left ventricular assist device (LVAD) support due to the risk of suction events with a small LV cavity. We describe the first case series of LVAD support for Fabry disease. LVAD therapy can improve survival, quality of life, and provide clinical stability to start enzyme replacement therapy. Precautions at the time of surgery include rapid treatment of fever to avoid Fabry crises, involvement of a multi ...[more]

PMID: 33644734

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Project description:BackgroundChronic cerebral hypoperfusion is a potential mechanism that causes cognitive impairment in patients with heart failure. Cognitive impairment in this population is associated with an increased mortality and poorer quality of life. Understanding the etiopathogenesis of cognitive impairment is crucial to developing effective treatment. A left ventricular assist device (LVAD) is a durable mechanical circulatory support device that restores systemic perfusion in patients with heart failure, potentially reversing cerebral hypoperfusion and cognitive impairment.ObjectiveThis case series and systematic review examines the effect of LVAD implantation on cognition in patients with heart failure.MethodsWe report a case series of 4 LVAD recipients at a tertiary academic center who underwent preimplant and postimplant cognitive testing. We also conducted a systematic review of studies with adult recipients of a continuous-flow LVAD whose cognition was measured before and after implantation. We searched Medline, EMBASE, SCOPUS, and the Cochrane library (start of database to July 16, 2021) for longitudinal, peer-reviewed studies written in English.ResultsCognitive improvement after LVAD implantation was observed in the case series, with improvement on phonemic fluency and digit symbol coding assessments. Two out of 4 cases in the case series improved on Clinical Dementia Rating: one from moderate dementia to mild cognitive impairment and another from mild cognitive impairment to unimpaired. Seven studies were included in the systematic review and were heterogeneous regarding cognitive tests employed, follow-up period, and measured outcomes. Montreal Cognitive Assessment and Trail-Making Test Part B were used most commonly. Cognitive improvement was reported in all 7 studies with at least 1 study reporting statistically significant improvements in each the following cognitive domains: delayed and immediate recall, executive function, visuospatial function, verbal function, attention, and processing speed. Most studies had small sample sizes and lacked a control group.ConclusionsLVAD implantation appears to be associated with improved cognition. Adequately powered, prospective studies are needed to examine the effect of LVAD on cognitive function in patients with heart failure. Additionally, studies that directly examine cerebral blood flow in conjunction with cognitive assessment are needed to establish the relationship between the reversal of cerebral hypoperfusion and improved cognition.

Dataset Information

Left Ventricular Assist Device Support for Fabry Cardiomyopathy: A Case Series.

Publications

Left Ventricular Assist Device Support for Fabry Cardiomyopathy: A Case Series.

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