Resultados de un primer procedimiento de crioablación como tratamiento de la fibrilación auricular paroxística

261 p.La crioablación es una de las opciones terapéuticas invasivas para el tratamiento de la fibrilación auricular (FA) paroxística. Se trata de una técnica de ablación relativamente ¿joven¿ para conseguir el aislamiento de las venas pulmonares (lo que se considera el objetivo principal de la ablación con catéter de la FA) y su objetivo final es mantener al paciente libre de recurrencias arrítmicas, por otro lado relativamente frecuentes en este tipo de arritmia. Presentamos un estudio observacional prospectivo que incluye 170 pacientes consecutivos con FA paroxística sometidos a crioablación por un único grupo y con un seguimiento de 43 ± 14 meses. Los hallazgos principales del estudio son los siguientes: 1) la crioablación constituye una técnica efectiva como primer tratamiento invasivo en pacientes con FA en nuestro medio, 2) el resultado de efectividad presenta un declinar progresivo con el paso de los años, por lo que el seguimiento prolongado de los pacientes resulta indispensable para valorar la efectividad de la técnica con propiedad, 3) la crioablación presenta una aceptable, aunque no menospreciable tasa de complicaciones, y 4) existe escasa correlación entre los síntomas y la recurrencias objetivable al final del seguimiento mediante la monitorización de larga duración. Asimismo, el haber presentado episodios persistentes de FA, el tamaño de la aurícula izquierda, el no aislar la vena pulmonar superior derecha y las recurrencias durante el periodo de blanking han mostrado ser los predictores más potentes de las recurrencias arrítmicas

Natural History of MYH7-Related Dilated Cardiomyopathy

BACKGROUND Variants in myosin heavy chain 7 (MYH7) are responsible for disease in 1% to 5% of patients with dilated cardiomyopathy (DCM); however, the clinical characteristics and natural history of MYH7-related DCM are poorly described. OBJECTIVES We sought to determine the phenotype and prognosis of MYH7-related DCM. We also evaluated the influence of variant location on phenotypic expression. METHODS We studied clinical data from 147 individuals with DCM-causing MYH7 variants (47.6% female; 35.6 +/- 19.2 years) recruited from 29 international centers. RESULTS At initial evaluation, 106 (72.1%) patients had DCM (left ventricular ejection fraction: 34.5% +/- 11.7%). Median follow-up was 4.5 years (IQR: 1.7-8.0 years), and 23.7% of carriers who were initially phenotype-negative developed DCM. Phenotypic expression by 40 and 60 years was 46% and 88%, respectively, with 18 patients (16%) first diagnosed at <18 years of age. Thirty-six percent of patients with DCM met imaging criteria for LV noncompaction. During follow-up, 28% showed left ventricular reverse remodeling. Incidence of adverse cardiac events among patients with DCM at 5 years was 11.6%, with 5 (4.6%) deaths caused by end-stage heart failure (ESHF) and 5 patients (4.6%) requiring heart transplantation. The major ventricular arrhythmia rate was low (1.0% and 2.1% at 5 years in patients with DCM and in those with LVEF of <= 35%, respectively). ESHF and major ventricular arrhythmia were significantly lower compared with LMNA-related DCM and similar to DCM caused by TTN truncating variants. CONCLUSIONS MYH7-related DCM is characterized by early age of onset, high phenotypic expression, low left ventricular reverse remodeling, and frequent progression to ESHF. Heart failure complications predominate over ventricular arrhythmias, which are rare. (C) 2022 The Authors. Published by Elsevier on behalf of the American College of Cardiology Foundation

Resultados de un primer procedimiento de crioablación como tratamiento de la fibrilación auricular paroxística

261 p.La crioablación es una de las opciones terapéuticas invasivas para el tratamiento de la fibrilación auricular (FA) paroxística. Se trata de una técnica de ablación relativamente ¿joven¿ para conseguir el aislamiento de las venas pulmonares (lo que se considera el objetivo principal de la ablación con catéter de la FA) y su objetivo final es mantener al paciente libre de recurrencias arrítmicas, por otro lado relativamente frecuentes en este tipo de arritmia. Presentamos un estudio observacional prospectivo que incluye 170 pacientes consecutivos con FA paroxística sometidos a crioablación por un único grupo y con un seguimiento de 43 ± 14 meses. Los hallazgos principales del estudio son los siguientes: 1) la crioablación constituye una técnica efectiva como primer tratamiento invasivo en pacientes con FA en nuestro medio, 2) el resultado de efectividad presenta un declinar progresivo con el paso de los años, por lo que el seguimiento prolongado de los pacientes resulta indispensable para valorar la efectividad de la técnica con propiedad, 3) la crioablación presenta una aceptable, aunque no menospreciable tasa de complicaciones, y 4) existe escasa correlación entre los síntomas y la recurrencias objetivable al final del seguimiento mediante la monitorización de larga duración. Asimismo, el haber presentado episodios persistentes de FA, el tamaño de la aurícula izquierda, el no aislar la vena pulmonar superior derecha y las recurrencias durante el periodo de blanking han mostrado ser los predictores más potentes de las recurrencias arrítmicas

Knowledge, preferences of post-explant management and opinions towards reuse of patients with cardiac implantable electronic devices.

The high cost of cardiac implantable electronic devices (CIEDs) is the most significant barrier to the lack of treatment in low- and middle-income countries (LMICs). The literature suggests that reusing CIEDs is safe and could therefore be a cost-effective alternative for patients who do not have access to new device

Patients’ opinions on cardiac implantable electronic device reuse in Bolivia

Tthe aim of our study was to assess the opinions of Bolivian patients on pacemaker reuse and to compare them depending on the ability to pay for a new device and other sociocultural factor

Association of Left Ventricular Systolic Dysfunction among Carriers of Truncating Variants in Filamin C with Frequent Ventricular Arrhythmia and End-stage Heart Failure

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