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1.
J Interv Card Electrophysiol ; 66(5): 1095-1101, 2023 Aug.
Article in English | MEDLINE | ID: mdl-35835889

ABSTRACT

BACKGROUND: Postmortem explanted cardiac implantable electronic devices (CIEDs) from developed countries could provide patients unable to afford new devices in low- and middle-income countries (LMIC) a treatment they lack. This study describes the preferences of electrophysiologists and device implanting cardiologists from Spain on the management of explanted CIEDs and opinions and concerns regarding reuse in LMIC. METHODS: A nationwide self-administered questionnaire was sent to members of the Spanish Rhythm Association (n = 1110), between December 2020 and January 2021. RESULTS: Forty-two physician responses were obtained (response rate 5%). There was a strong preference to donate explanted devices for reuse in humans (61.9%) or animals (31%). The vast majority of the participants thought device reutilization was safe, ethical, and a reasonable alternative if a new device is not accessible. Moreover, they indicated they would be comfortable asking patients to consider post-mortem donation, and willing to implant post-mortem explanted and resterilized devices if they were unable to obtain new ones. 57.1% of respondents considered it would be beneficial for patients to have a document so they could reflect their wishes regarding device handling after their death. The most mentioned concerns regarding device reuse were malfunction (57.1%) and infection (54.8%). CONCLUSIONS: The majority of respondents support reusable CIED donation to LMIC. It would be interesting to study the feasibility of a nationwide device reutilization program.


Subject(s)
Defibrillators, Implantable , Pacemaker, Artificial , Humans , Developing Countries , Autopsy , Surveys and Questionnaires
3.
JAMA Cardiol ; 6(8): 891-901, 2021 08 01.
Article in English | MEDLINE | ID: mdl-33978673

ABSTRACT

Importance: Truncating variants in the gene encoding filamin C (FLNCtv) are associated with arrhythmogenic and dilated cardiomyopathies with a reportedly high risk of ventricular arrhythmia. Objective: To determine the frequency of and risk factors associated with adverse events among FLNCtv carriers compared with individuals carrying TTN truncating variants (TTNtv). Design, Setting, and Participants: This cohort study recruited 167 consecutive FLNCtv carriers and a control cohort of 244 patients with TTNtv matched for left ventricular ejection fraction (LVEF) from 19 European cardiomyopathy referral units between 1990 and 2018. Data analyses were conducted between June and October, 2020. Main Outcomes and Measures: The primary end point was a composite of malignant ventricular arrhythmia (MVA) (sudden cardiac death, aborted sudden cardiac death, appropriate implantable cardioverter-defibrillator shock, and sustained ventricular tachycardia) and end-stage heart failure (heart transplant or mortality associated with end-stage heart failure). The secondary end point comprised MVA events only. Results: In total, 167 patients with FLNCtv were studied (55 probands [33%]; 89 men [53%]; mean [SD] age at baseline evaluation, 43 [18] years). For a median follow-up of 20 months (interquartile range, 7-60 months), 29 patients (17.4%) reached the primary end point (19 patients with MVA and 10 patients with end-stage heart failure). Eight (44%) arrhythmic events occurred among individuals with baseline mild to moderate left ventricular systolic dysfunction (LVSD) (LVEF = 36%-49%). Univariable risk factors associated with the primary end point included proband status, LVEF decrement per 10%, ventricular ectopy (≥500 in 24 hours) and myocardial fibrosis detected on cardiac magnetic resonance imaging. The LVEF decrement (hazard ratio [HR] per 10%, 1.83 [95% CI, 1.30-2.57]; P < .001) and proband status (HR, 3.18 [95% CI, 1.12-9.04]; P = .03) remained independent risk factors on multivariable analysis (excluding myocardial fibrosis and ventricular ectopy owing to case censoring). There was no difference in freedom from MVA between FLNCtv carriers with mild to moderate or severe (LVEF ≤35%) LVSD (HR, 1.29 [95% CI, 0.45-3.72]; P = .64). Carriers of FLNCtv with impaired LVEF at baseline evaluation (n = 69) had reduced freedom from MVA compared with 244 TTNtv carriers with similar baseline LVEF (for mild to moderate LVSD: HR, 16.41 [95% CI, 3.45-78.11]; P < .001; for severe LVSD: HR, 2.47 [95% CI, 1.04-5.87]; P = .03). Conclusions and Relevance: The high frequency of MVA among patients with FLNCtv with mild to moderate LVSD suggests that higher LVEF values than those currently recommended should be considered for prophylactic implantable cardioverter-defibrillator therapy in FLNCtv carriers.


Subject(s)
Cardiomyopathy, Dilated/genetics , Death, Sudden, Cardiac/prevention & control , Filamins/genetics , Heart Failure/genetics , Tachycardia, Ventricular/genetics , Ventricular Dysfunction, Left/genetics , Adult , Cardiomyopathy, Dilated/mortality , Cardiomyopathy, Dilated/physiopathology , Cardiomyopathy, Dilated/therapy , Codon, Nonsense , Connectin/genetics , Defibrillators, Implantable , Female , Heart Failure/mortality , Heart Failure/physiopathology , Heart Failure/therapy , Heart Transplantation/statistics & numerical data , Humans , Male , Middle Aged , Mutation , Stroke Volume , Tachycardia, Ventricular/epidemiology , Tachycardia, Ventricular/physiopathology , Ventricular Dysfunction, Left/physiopathology
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