Refractory Right Ventricular Failure in a Patient with Emery-Dreifuss Muscular Dystrophy.

Refractory Right Ventricular Failure in a Patient with Emery-Dreifuss Muscular Dystrophy. Intern Med. 2020 Feb 19;: Authors: Chiba Y, Fukushima A, Nakao M, Kobayashi Y, Ishigaki T, Tenma T, Kamiya K, Shingu Y, Ooka T, Matsui Y, Anzai T Abstract A 23-year-old man had progressive muscle weakness and Emery-Dreifuss muscular dystrophy (EDMD) due to a LMNA (lamin A/C) mutation. Congestive heart failure diagnosed at 19 years of age. Maximal drug treatment/cardiac resynchronization failed to improve the cardiac function. He was therefore hospitalized due to heart failure. Despite extracorporeal membrane oxygenation, he developed severe right heart dysfunction and died (multiple organ failure). A cardiac lesion's presence determines the prognosis of EDMD. While there are many arrhythmia reports, few reports on heart failure (particularly severe heart failure requiring cardiac transplantation) have been published. Right heart function monitoring and early ventricular-assist device use plus right heart support considering heart transplantation are important. PMID: 32074578 [PubMed - as supplied by publisher]
Source: Internal Medicine - Category: Internal Medicine Tags: Intern Med Source Type: research

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No abstract available
Source: ASAIO Journal - Category: Medical Devices Tags: Invited Commentary Source Type: research
This study aimed to assess the hemodynamic effects of concomitant MVS. Of all 73 enrolled patients, 44 patients had undergone concomitant MVS and 29 patients had not. Before LVAD implantation, MVS group had higher pulmonary capillary wedge pressure (p = 0.04). After LVAD implantation, MVS group had higher mean pulmonary artery pressure and cardiac output (CO). During the hemodynamic ramp study, MVS group had steeper CO slopes (0.18 [0.13 0.28] vs. 0.15 [0.08, 0.20] L/min/step; p = 0.04) at incremental LVAD speed and achieved a higher CO at the optimized set speed (5.5 [4.7, 6.9] vs. 4.9 [4.0, 5.7] L/min; p = 0.03). One-yea...
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Continuous-flow left ventricular assist devices (CF-LVADs) are increasingly used in advanced heart failure patients. Recent studies suggest that low socioeconomic status (SES) predicts worst survival after heart transplantation. Both individual-level and neighborhood-level SES (nSES) have been linked to cardiovascular health; however, the impact of SES in CF-LVAD patients remains unknown. We hypothesized that SES is a major determinant of CF-LVAD candidacy and postimplantation outcomes. A retrospective chart review was conducted on 362 patients between February 2009 and May 2016. Neighborhood-level SES was measured using t...
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Source: ASAIO Journal - Category: Medical Devices Tags: Invited Commentary Source Type: research
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Source: ASAIO Journal - Category: Medical Devices Tags: Adult Circulatory Support Source Type: research
More News: Arrhythmia | Cardiology | Congestive Heart Failure | Heart | Heart Failure | Heart Transplant | Internal Medicine | Muscular Dystrophy | Reflex Sympathetic Dystrophy | Transplants