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1.
Eur Heart J ; 40(21): 1681-1687, 2019 06 01.
Artículo en Inglés | MEDLINE | ID: mdl-31152553

RESUMEN

AIMS: Based on European guidelines, alcohol septal ablation (ASA) for hypertrophic obstructive cardiomyopathy (HOCM) is indicated only in patients with interventricular septum (IVS) thickness >16 mm. The aim of this study was to evaluate the short- and long-term outcomes in ASA patients with mild hypertrophy (IVS ≤ 16 mm). METHODS AND RESULTS: We retrospectively evaluated 1505 consecutive ASA patients and used propensity score to match 172 pairs (344 patients) in groups IVS ≤ 16 mm or IVS > 16 mm. There was no occurrence of post-ASA ventriculoseptal defect in the whole cohort (n = 1505). Matched patients had 30-day mortality rate 0% in IVS ≤ 16 mm group and 0.6% in IVS > 16 mm group (P = 1). Patients in IVS ≤ 16 mm group had more ASA-attributable early complications (16% vs. 9%; P = 0.049), which was driven by higher need for pacemaker implantation (13% vs. 8%; P = 0.22). The mean follow-up was 5.4 ± 4.3 years and the annual all-cause mortality rate was 1.8 and 3.2 deaths per 100-patient-years in IVS ≤ 16 group and IVS > 16 group, respectively (log-rank test P = 0.04). There were no differences in symptom relief and left ventricular (LV) gradient reduction. Patients with IVS ≤ 16 mm had less repeated septal reduction procedures (log-rank test P = 0.03). CONCLUSION: Selected patients with HOCM and mild hypertrophy (IVS ≤ 16 mm) had more early post-ASA complications driven by need for pacemaker implantation, but their long-term survival is better than in patients with IVS >16 mm. While relief of symptoms and LV obstruction reduction is similar in both groups, a need for repeat septal reduction is higher in patients with IVS > 16 mm.


Asunto(s)
Técnicas de Ablación , Cardiomiopatía Hipertrófica , Hipertrofia Ventricular Izquierda , Técnicas de Ablación/efectos adversos , Técnicas de Ablación/métodos , Técnicas de Ablación/estadística & datos numéricos , Anciano , Cardiomiopatía Hipertrófica/mortalidad , Cardiomiopatía Hipertrófica/patología , Cardiomiopatía Hipertrófica/cirugía , Femenino , Tabiques Cardíacos/patología , Tabiques Cardíacos/cirugía , Humanos , Hipertrofia Ventricular Izquierda/complicaciones , Hipertrofia Ventricular Izquierda/mortalidad , Masculino , Persona de Mediana Edad , Complicaciones Posoperatorias/epidemiología , Puntaje de Propensión , Estudios Retrospectivos , Resultado del Tratamiento
2.
Europace ; 21(7): 1088-1095, 2019 Jul 01.
Artículo en Inglés | MEDLINE | ID: mdl-31121018

RESUMEN

AIMS: Stereotactic body radiotherapy (SBRT) for ventricular tachycardias (VTs) could be an option after failed catheter ablation. In this study, we analysed the long-term efficacy and toxicity of SBRT applied as a bail-out procedure. METHODS AND RESULTS: Patients with structural heart disease and unsuccessful catheter ablations for VTs underwent SBRT. The planning target volume (PTV) was accurately delineated using exported 3D electroanatomical maps with the delineated critical part of re-entry circuits. This was defined by detailed electroanatomic mapping and by pacing manoeuvres during the procedure. Using the implantable cardioverter-defibrillator lead as a surrogate contrast marker for respiratory movement compensation, 25 Gy was delivered to the PTV using CyberKnife. We evaluated occurrences of sustained VT, electrical storm, antitachycardia pacing, and shock; time to death; and radiation-induced events. From 2014 until March 2017, 10 patients underwent radiosurgical ablation (mean PTV, 22.15 mL; treatment duration, 68 min). After radiosurgery, four patients experienced nausea and one patient presented gradual progression of mitral regurgitation. During the follow-up (median 28 months), VT burden was reduced by 87.5% compared with baseline (P = 0.012) and three patients suffered non-arrhythmic deaths. After the blanking period, VT recurred in eight of 10 patients. The mean time to first antitachycardia pacing and shock were 6.5 and 21 months, respectively. CONCLUSION: Stereotactic body radiotherapy appears to show long-term safety and effectiveness for VT ablation in structural heart disease inaccessible to catheter ablation. We report one possible radiation-related toxicity and promising overall survival, warranting evaluation in a prospective multicentre clinical trial.


Asunto(s)
Radiocirugia/métodos , Taquicardia Ventricular/radioterapia , Anciano , Ablación por Catéter , Técnicas Electrofisiológicas Cardíacas , Femenino , Estudios de Seguimiento , Humanos , Masculino , Persona de Mediana Edad
3.
Europace ; 19(11): 1781-1789, 2017 Nov 01.
Artículo en Inglés | MEDLINE | ID: mdl-27707782

RESUMEN

AIMS: Identifying patients who benefit from restored sinus rhythm (SR) would optimize the selection of candidates for ablation of long-standing persistent atrial fibrillation (LSPAF). This prospective study sought to identify the hitherto unknown factors associated with global functional improvement after successful radiofrequency catheter ablation of LSPAF. METHODS AND RESULTS: In 171 LSPAF patients (84% of the total consecutive 203 patients) who were examined in SR 12 months after ablation, the individual per cent change from baseline value in maximum oxygen consumption at exercise test (VO2 max), left ventricular ejection fraction (LVEF), N-terminal prohormone of brain natriuretic peptide (NT-proBNP), and five-dimensional descriptive system (EQ-5D) of quality-of-life questionnaire were classified in quartiles by 0 (worse) to 3 (best) grades. The individual grades were summed into a composite score (SCORE, 0 … 12) reflecting global functional improvement. Significant improvement in VO2 max (3.4 ± 4.7 mL/kg/min), LVEF (7.5 ± 9.1%), NT-proBNP (-861 ± 809 pg/mL), and EQ-5D (0.7 ± 0.12) was observed (all P < 0.0001). On multivariable analysis, younger age (P = 0.001), male gender (P = 0.02), timely post-ablation left atrial appendage (LAA) outflow (P = 0.005) with improvement in outflow velocity (P = 0.0002), and withdrawal of Class I/III antiarrhythmic drugs (P < 0.05) were positively and independently correlated with the SCORE. CONCLUSIONS: Younger male patients benefited most from catheter ablation of LSPAF. Delayed or non-improved LAA outflow and inability to discontinue Class I/III antiarrhythmic medication reduced the post-ablation functional improvement.


Asunto(s)
Fibrilación Atrial/cirugía , Función del Atrio Izquierdo , Ablación por Catéter , Tolerancia al Ejercicio , Función Ventricular Izquierda , Adulto , Factores de Edad , Anciano , Antiarrítmicos/uso terapéutico , Fibrilación Atrial/diagnóstico , Fibrilación Atrial/fisiopatología , Biomarcadores/sangre , Ablación por Catéter/efectos adversos , Distribución de Chi-Cuadrado , Femenino , Humanos , Masculino , Persona de Mediana Edad , Análisis Multivariante , Péptido Natriurético Encefálico/sangre , Consumo de Oxígeno , Fragmentos de Péptidos/sangre , Estudios Prospectivos , Calidad de Vida , Recuperación de la Función , Sistema de Registros , Factores de Riesgo , Factores Sexuales , Volumen Sistólico , Encuestas y Cuestionarios , Factores de Tiempo , Resultado del Tratamiento
4.
Eur Heart J ; 37(19): 1517-23, 2016 05 14.
Artículo en Inglés | MEDLINE | ID: mdl-26746632

RESUMEN

AIMS: The first cases of alcohol septal ablation (ASA) for obstructive hypertrophic cardiomyopathy (HCM) were published two decades ago. Although the outcomes of single-centre and national ASA registries have been published, the long-term survival and clinical outcome of the procedure are still debated. METHODS AND RESULTS: We report long-term outcomes from the as yet largest multinational ASA registry (the Euro-ASA registry). A total of 1275 (58 ± 14 years, median follow-up 5.7 years) highly symptomatic patients treated with ASA were included. The 30-day post-ASA mortality was 1%. Overall, 171 (13%) patients died during follow-up, corresponding to a post-ASA all-cause mortality rate of 2.42 deaths per 100 patient-years. Survival rates at 1, 5, and 10 years after ASA were 98% (95% CI 96-98%), 89% (95% CI 87-91%), and 77% (95% CI 73-80%), respectively. In multivariable analysis, independent predictors of all-cause mortality were age at ASA (P < 0.01), septum thickness before ASA (P < 0.01), NYHA class before ASA (P = 0.047), and the left ventricular (LV) outflow tract gradient at the last clinical check-up (P = 0.048). Alcohol septal ablation reduced the LV outflow tract gradient from 67 ± 36 to 16 ± 21 mmHg (P < 0.01) and NYHA class from 2.9 ± 0.5 to 1.6 ± 0.7 (P < 0.01). At the last check-up, 89% of patients reported dyspnoea of NYHA class ≤2, which was independently associated with LV outflow tract gradient (P < 0.01). CONCLUSIONS: The Euro-ASA registry demonstrated low peri-procedural and long-term mortality after ASA. This intervention provided durable relief of symptoms and a reduction of LV outflow tract obstruction in selected and highly symptomatic patients with obstructive HCM. As the post-procedural obstruction seems to be associated with both worse functional status and prognosis, optimal therapy should be focused on the elimination of LV outflow tract gradient.


Asunto(s)
Cardiomiopatía Hipertrófica/terapia , Etanol/uso terapéutico , Solventes/uso terapéutico , Técnicas de Ablación/métodos , Técnicas de Ablación/mortalidad , Cardiomiopatía Hipertrófica/mortalidad , Supervivencia sin Enfermedad , Femenino , Tabiques Cardíacos , Humanos , Estimación de Kaplan-Meier , Masculino , Persona de Mediana Edad , Factores de Riesgo , Resultado del Tratamiento
5.
Vnitr Lek ; 63(4): 249-253, 2017.
Artículo en Checo | MEDLINE | ID: mdl-28520448

RESUMEN

Hypertrophic cardiomyopathy is currently understood as a group of diseases with left ventricular hypertrophy, which are not based on adaptive mechanisms. The first part of the review details the possibility of cardiac magnetic resonance in the diagnosis of sarcomeric forms of hypertrophic cardiomyopathy, the second part will focus on the possibilities of distinguishing the sarcomeric forms from their phenocopies.Key words: cardiac magnetic resonance - hypertrophic cardiomyopathy - phenocopies.


Asunto(s)
Cardiomiopatía Hipertrófica/diagnóstico por imagen , Hipertrofia Ventricular Izquierda/diagnóstico por imagen , Imagen por Resonancia Magnética , Humanos
6.
Vnitr Lek ; 63(3): 194-198, 2017.
Artículo en Checo | MEDLINE | ID: mdl-28379022

RESUMEN

Hypertrophic cardiomyopathy is the most common genetic cardiomyopathy in our population. The diagnosis of this disease is based on imaging methods, mainly echocardiography. Cardiac magnetic resonance offers more accurate and detailed information about the disease than echocardiography. Development of this method has become a standard part of the diagnostic algorithm. In the first part of review, the authors analyze the potential and significance of cardiac magnetic resonance in the diagnosis of hypertrophic cardiomyopathy.Key words: cardiac magnetic resonance - fibrosis - hypertrophic cardiomyopathy.


Asunto(s)
Cardiomiopatía Hipertrófica/diagnóstico por imagen , Imagen por Resonancia Magnética , Ecocardiografía , Humanos
7.
Catheter Cardiovasc Interv ; 84(1): 101-7, 2014 Jul 01.
Artículo en Inglés | MEDLINE | ID: mdl-24285605

RESUMEN

BACKGROUND: This study was designed to evaluate the outcomes of alcohol septal ablation (ASA) under multicenter and multinational conditions. METHODS: Data for 459 patients (age 57 ± 13 years) from nine European centers were prospectively collected and retrospectively analyzed. RESULTS: ASA led to a significant reduction in outflow gradient (PG) and dyspnea [median of PG from 88 (58-123) mm Hg to 21 (11-41) mm Hg; median of NYHA class from 3 (2-3) to 1 (1-2); P < 0.01]. The incidence of 3-month major adverse events (death, electrical cardioversion for tachyarrhythmias, resuscitation) and mortality was 2.8% and 0.7%, respectively. Permanent pacemakers for post-ASA complete heart block were implanted in 43 patients (9%). Multivariate analysis identified higher amount of alcohol (however, in generally low-dose procedures), higher baseline left ventricular ejection fraction and higher age as independent predictors of PG decrease ≥50%. CONCLUSIONS: The results of the first European multicenter and multinational study demonstrate that real-world early outcomes of ASA patients are better than was reported in observations from the first decade after ASA introduction.


Asunto(s)
Técnicas de Ablación/métodos , Procedimientos Quirúrgicos Cardíacos/métodos , Cardiomiopatía Hipertrófica/cirugía , Etanol/farmacología , Tabiques Cardíacos/cirugía , Adulto , Anciano , Anciano de 80 o más Años , Cardiomiopatía Hipertrófica/mortalidad , Cardiomiopatía Hipertrófica/fisiopatología , Europa (Continente)/epidemiología , Femenino , Estudios de Seguimiento , Humanos , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Tasa de Supervivencia/tendencias , Factores de Tiempo , Resultado del Tratamiento , Función Ventricular Izquierda/fisiología , Adulto Joven
8.
Europace ; 16(1): 15-25, 2014 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-23851514

RESUMEN

AIMS: Clinical benefit from ablation for long-standing persistent atrial fibrillation has remained unknown. We hypothesized that successful ablation of long-standing persistent atrial fibrillation would improve haemodynamics, functional status, and quality of life. METHODS AND RESULTS: A total of 160 patients (aged 59 ± 9 years, 23% females) undergoing ablation of long-standing (median of 28 months) persistent atrial were enrolled in this prospective study. Morphological and functional echocardiographic parameters, N-terminal prohormone of brain natriuretic peptide (NT-proBNP), maximum oxygen consumption during exercise test (VO2 max), and quality of life were assessed at baseline and 1 year after the ablation.  At the 1-year follow-up visit, 81% patients were examined in sinus rhythm (after repeat ablation in 38% patients). Left atrial appendage outflow velocity increased from 44 ± 20 to 58 ± 23 cm/s, left ventricular ejection fraction from 54 ± 9 to 59 ± 5%, and VO2 max from 20.4 ± 6.4 to 23.7 ± 8.1 mL/kg/min; NT-proBNP decreased from median 897 (interquartile range 603-1424) to 230 (interquartile range 120-420) pg/mL (all P < 0.0001). These beneficial effects of ablation were predominantly associated with the presence of sinus rhythm. Quality of life (range 0-100) increased significantly (EQ-5D index: from 68.8 ± 12.5 to 75.4 ± 14.4; EQ-VAS score: from 62.8 ± 13.2 to 70.6 ± 13.8; both P < 0.0001). CONCLUSION: Ablation of long-standing persistent atrial fibrillation was associated with significant recovery of haemodynamics and exercise capacity that projected onto the long-term improvement in quality of life.


Asunto(s)
Fibrilación Atrial/psicología , Fibrilación Atrial/cirugía , Ablación por Catéter/psicología , Depresión/psicología , Complicaciones Posoperatorias/psicología , Calidad de Vida/psicología , Recuperación de la Función , Actividades Cotidianas/psicología , Adulto , Anciano , Fibrilación Atrial/diagnóstico , Ablación por Catéter/efectos adversos , Enfermedad Crónica , Depresión/etiología , Femenino , Humanos , Estudios Longitudinales , Masculino , Persona de Mediana Edad , Complicaciones Posoperatorias/etiología , Estudios Prospectivos , Resultado del Tratamiento
9.
Can J Cardiol ; 39(11): 1622-1629, 2023 11.
Artículo en Inglés | MEDLINE | ID: mdl-37355228

RESUMEN

BACKGROUND: The current ACC/AHA guidelines on hypertrophic cardiomyopathy (HCM) caution that alcohol septal ablation (ASA) might be less effective in patients with left ventricular outflow tract obstruction (LVOTO) ≥ 100 mm Hg. METHODS: We used a multinational registry to evaluate the outcome of ASA patients according to baseline LVOTO. RESULTS: A total of 1346 ASA patients were enrolled and followed for 5.8 ± 4.7 years (7764 patient-years). The patients with baseline LVOTO ≥ 100 mm Hg were significantly older (61 ± 14 years vs 57 ± 13 years; P < 0.01), more often women (60% vs 45%; P < 0.01), and had a more pronounced HCM phenotype than those with baseline LVOTO < 100 mm Hg. There were no significant differences in the occurrences of 30-day major cardiovascular adverse events in the 2 groups. After propensity score matching (2 groups, 257 pairs of patients), the long-term survival was similar in both groups (P = 0.10), the relative reduction of LVOTO was higher in the group with baseline LVOTO ≥ 100 mm Hg (82 ± 21% vs 73 ± 26%; P < 0.01), but the residual resting LVOTO remained higher in this group (23 ± 29 mm Hg vs 13 ± 13 mm Hg; P < 0.01). Dyspnoea (NYHA functional class) at the most recent clinical check-up was similar in the 2 groups (1.7 ± 0.7 vs 1.7 ± 0.7; P = 0.85), and patients with baseline LVOTO ≥ 100 mm Hg underwent more reinterventions (P = 0.02). CONCLUSIONS: After propensity matching, ASA patients with baseline LVOTO ≥ 100 mm Hg had similar survival and dyspnoea as patients with baseline LVOTO < 100 mm Hg, but their residual LVOTO and risk of repeated procedures were higher.


Asunto(s)
Procedimientos Quirúrgicos Cardíacos , Cardiomiopatía Hipertrófica , Obstrucción del Flujo de Salida Ventricular Izquierda , Obstrucción del Flujo Ventricular Externo , Humanos , Femenino , Puntaje de Propensión , Cardiomiopatía Hipertrófica/complicaciones , Cardiomiopatía Hipertrófica/diagnóstico , Cardiomiopatía Hipertrófica/cirugía , Disnea/etiología , Obstrucción del Flujo Ventricular Externo/cirugía , Resultado del Tratamiento , Estudios Retrospectivos
10.
JACC Cardiovasc Imaging ; 15(2): 181-189, 2022 02.
Artículo en Inglés | MEDLINE | ID: mdl-34419390

RESUMEN

OBJECTIVES: This paper sought to evaluate the occurrence of decompression sickness (DCS) after the application of a patent foramen ovale (PFO) screening and risk stratification strategy. BACKGROUND: PFO is associated with an increased risk of DCS. Recently, transcatheter closure was reported to reduce DCS occurrence in divers with a high-grade shunt. However, to date, there are no data regarding the effectiveness of any PFO screening and risk stratification strategy for divers. METHODS: A total of 829 consecutive divers (age 35.4 ± 10.0 years, 81.5% men) were screened for PFO by means of transcranial color-coded sonography in the DIVE-PFO (Decompression Illness Prevention in Divers with a Patent Foramen Ovale) registry. Divers with a high-grade PFO were offered either catheter-based PFO closure (the closure group) or advised conservative diving (high grades). Divers with a low-grade shunt were advised conservative diving (low grades), whereas those with no PFO continued unrestricted diving (controls). A telephone follow-up was performed. To study the effect of the screening and risk stratification strategy, DCS occurrence before enrollment and during the follow-up was compared. RESULTS: Follow-up was available for 748 (90%) divers. Seven hundred and 2 divers continued diving and were included in the analysis (mean follow-up 6.5 ± 3.5 years). The DCS incidence decreased significantly in all groups, except the controls. During follow-up, there were no DCS events in the closure group; DCS incidence was similar to the controls in the low-grade group (HR: 3.965; 95% CI: 0.558-28.18; P = 0.169) but remained higher in the high-grade group (HR: 26.170; 95% CI: 5.797-118.160; P < 0.0001). CONCLUSIONS: The screening and risk stratification strategy using transcranial color-coded sonography was associated with a decrease in DCS occurrence in divers with PFO. Catheter-based PFO closure was associated with a DCS occurrence similar to the controls; the conservative strategy had a similar effect in the low-grade group, but in the high-grade group the DCS incidence remained higher than in all other groups.


Asunto(s)
Enfermedad de Descompresión , Foramen Oval Permeable , Adulto , Enfermedad de Descompresión/diagnóstico por imagen , Enfermedad de Descompresión/epidemiología , Enfermedad de Descompresión/etiología , Femenino , Foramen Oval Permeable/complicaciones , Foramen Oval Permeable/diagnóstico por imagen , Foramen Oval Permeable/epidemiología , Humanos , Masculino , Persona de Mediana Edad , Valor Predictivo de las Pruebas , Sistema de Registros , Medición de Riesgo
11.
JACC Cardiovasc Interv ; 15(19): 1910-1917, 2022 10 10.
Artículo en Inglés | MEDLINE | ID: mdl-36202559

RESUMEN

BACKGROUND: Atrioventricular block is a frequent major complication after alcohol septal ablation (ASA). OBJECTIVES: The aim of this study was to evaluate the outcomes of patients with implanted permanent pacemaker (PPM) related to a high-grade atrioventricular block after ASA for hypertrophic obstructive cardiomyopathy. METHODS: We used a multinational registry (the Euro-ASA registry) to evaluate the outcome of patients with PPM after ASA. RESULTS: A total of 1,814 patients were enrolled and followed up for 5.0 ± 4.3 years (median = 4.0 years). A total of 170 (9.4%) patients underwent PPM implantation during the first 30 days after ASA. Using propensity score matching, 139 pairs (n = 278) constituted the matched PPM and non-PPM groups. Between the matched groups, there were no long-term differences in New York Heart Association functional class (1.5 ± 0.7 vs 1.5 ± 0.9, P = 0.99) and survival (log-rank P = 0.47). Patients in the matched PPM group had lower long-term left ventricular (LV) outflow gradient (12 ± 12 mm Hg vs 17 ± 19 mm Hg, P < 0.01), more pronounced LV outflow gradient decrease (81% ± 17% vs 72% ± 35%, P < 0.01), and lower LV ejection fraction (64% ± 8% vs 66% ± 8%, P = 0.02) and were less likely to undergo reintervention (re-ASA or myectomy) (log-rank P = 0.02). CONCLUSIONS: Patients with hypertrophic obstructive cardiomyopathy treated with ASA have a 9% probability of PPM implantation within 30 days after ASA. In long-term follow-up, patients with PPM had similar long-term survival and New York Heart Association functional class but lower LV outflow gradient, a more pronounced LV outflow gradient decrease, a lower LV ejection fraction, and a lower likelihood of reintervention compared with patients without PPM.


Asunto(s)
Técnicas de Ablación , Bloqueo Atrioventricular , Cardiomiopatía Hipertrófica , Marcapaso Artificial , Técnicas de Ablación/efectos adversos , Bloqueo Atrioventricular/diagnóstico , Bloqueo Atrioventricular/etiología , Bloqueo Atrioventricular/terapia , Cardiomiopatía Hipertrófica/diagnóstico por imagen , Cardiomiopatía Hipertrófica/cirugía , Etanol/efectos adversos , Tabiques Cardíacos/diagnóstico por imagen , Tabiques Cardíacos/cirugía , Humanos , Estudios Retrospectivos , Resultado del Tratamiento
12.
ESC Heart Fail ; 9(6): 4160-4166, 2022 12.
Artículo en Inglés | MEDLINE | ID: mdl-36087038

RESUMEN

AIMS: Fabry disease (FD) is a rare X-linked genetic disorder caused by α-galactosidase A (AGALA) deficiency. Whereas 'classic' variant has multisystemic manifestation, the more recently described 'later-onset' variant is characterized by predominant cardiac involvement that often mimics hypertrophic cardiomyopathy (HCM). METHODS AND RESULTS: Consecutive unrelated patients with HCM were screened for FD in 16 (out of 17) cardiac centres in the Czech Republic covering specialized cardiology care from June 2017 to December 2018. AGALA activity and globotriaosylsphingosine (lyso-Gb3 ) levels were measured in all subjects using the dry blood spot method. FD was suspected in male patients with AGALA activity <1.2 µmol/h/L and in females with either low AGALA activity or lyso-Gb3  > 3.5 ng/mL. Positive screening results were confirmed by genetic testing. We evaluated 589 patients (390 males, 66%) with HCM (mean maximal myocardial thickness 19.1 ± 4.3 mm). The average age was 58.4 ± 14.7 years. In total, 17 patients (11 males, 6 females) had a positive screening result, and subsequently, six of them (four males and two females) had a genetically confirmed pathogenic GLA mutation (total prevalence of 1.02%). Five of these patients were carrying the p.N215S mutation known to cause a typical later-onset cardiac FD. CONCLUSIONS: We confirmed the prevalence of FD repeatedly reported in previous screening programmes (approximately 1% irrespective of gender) in a non-selected HCM population in Central Europe. Our findings advocate a routine screening for FD in all adult patients with HCM phenotype including both genders. The dry blood spot method used led to identification of clearly pathogenic variants.


Asunto(s)
Cardiomiopatía Hipertrófica , Enfermedad de Fabry , Femenino , Humanos , Masculino , alfa-Galactosidasa/genética , Cardiomiopatía Hipertrófica/diagnóstico , Cardiomiopatía Hipertrófica/epidemiología , Cardiomiopatía Hipertrófica/genética , República Checa/epidemiología , Enfermedad de Fabry/diagnóstico , Enfermedad de Fabry/epidemiología , Enfermedad de Fabry/genética , Pruebas Genéticas , Adulto , Persona de Mediana Edad , Anciano
13.
Am J Cardiol ; 184: 120-126, 2022 12 01.
Artículo en Inglés | MEDLINE | ID: mdl-36192196

RESUMEN

This study aimed to derive a new score, the Alcohol Septal Ablation-Sudden Cardiac ARREst (ASA-SCARRE) risk score, that can be easily used to evaluate the risk of sudden cardiac arrest events (sudden cardiac death, resuscitation, or appropriate implantable cardioverter-defibrillator discharge) after alcohol septal ablation (ASA) in patients with hypertrophic obstructive cardiomyopathy. We analyzed 1,834 patients from the Euro-ASA registry (49% men, mean age 57 ± 14 years) who were followed up for 5.0 ± 4.3 years (9,202 patient-years) after ASA. A total of 65 patients (3.5%) experienced sudden cardiac arrest events, translating to 0.72 events per 100 patient-years. The independent predictors of sudden cardiac arrest events were septum thickness before ASA (hazard ratio 1.09 per 1 mm, 95% confidence interval 1.04 to 1.14, p <0.001) and left ventricular outflow tract (LVOT) gradient at the last clinical checkup (hazard ratio 1.01 per 1 mm Hg, 95% confidence interval 1.01 to 1.02, p = 0.002). The following ASA-SCARRE risk scores were derived and independently predicted long-term risk of sudden cardiac arrest events: "0" for both LVOT gradient <30 mmHg and baseline septum thickness <20 mm; "1" for LVOT gradient ≥30 mm Hg or baseline septum thickness ≥20 mm; and "2" for both LVOT gradient ≥30 mm Hg and baseline septum thickness ≥20 mm. The C statistic of the ASA-SCARRE risk score was 0.684 (SE 0.030). In conclusion, the ASA-SCARRE risk score may be a useful and easily available clinical tool to predict risk of sudden cardiac arrest events after ASA in patients with hypertrophic obstructive cardiomyopathy.


Asunto(s)
Técnicas de Ablación , Procedimientos Quirúrgicos Cardíacos , Cardiomiopatía Hipertrófica , Masculino , Humanos , Adulto , Persona de Mediana Edad , Anciano , Femenino , Etanol/uso terapéutico , Cardiomiopatía Hipertrófica/complicaciones , Cardiomiopatía Hipertrófica/cirugía , Muerte Súbita Cardíaca/epidemiología , Muerte Súbita Cardíaca/etiología , Muerte Súbita Cardíaca/prevención & control , Factores de Riesgo , Resultado del Tratamiento
14.
Int J Cardiol ; 333: 127-132, 2021 06 15.
Artículo en Inglés | MEDLINE | ID: mdl-33647367

RESUMEN

BACKGROUND: The aim of this study was to evaluate short- and long-term outcomes related to dose of alcohol administered during alcohol septal ablation (ASA) in patients with hypertrophic obstructive cardiomyopathy (HOCM). Current guidelines recommend using 1-3 mL of alcohol administered in the target septal perforator artery, but this recommendation is based more on practical experience of interventionalists rather than on systematic evidence. METHODS: We included 1448 patients and used propensity score to match patients who received a low-dose (1.0-1.9 mL) versus a high-dose (2.0-3.8 mL) of alcohol during ASA. RESULTS: The matched cohort analysis comprised 770 patients (n = 385 in both groups). There was a similar occurrence of 30-day post-procedural adverse events (13% vs. 12%; p = 0.59), and similar all-cause mortality rates (0.8% vs. 0.5%; p = 1) in the low-dose group and the high-dose group, respectively. In the long-term follow-up (5.4 ± 4.5 years), a total of 110 (14%) patients died representing 2.58 deaths and 2.64 deaths per 100 patient-years in the low dose and the high dose group (logrank, p = 0.92), respectively. There were no significant differences in the long-term dyspnea and left ventricular outflow gradient between the two groups. Patients treated with a low-dose of alcohol underwent more subsequent septal reduction procedures (logrank, p = 0.04). CONCLUSIONS: Matched HOCM patients undergoing ASA with a low-dose (1.0-1.9 mL) or a high-dose (2.0-3.8 mL) of alcohol had similar short- and long-term outcomes. A higher rate of repeated septal reduction procedures was observed in the group treated with a low-dose of alcohol.


Asunto(s)
Técnicas de Ablación , Cardiomiopatía Hipertrófica , Ablación por Catéter , Cardiomiopatía Hipertrófica/diagnóstico por imagen , Cardiomiopatía Hipertrófica/cirugía , Etanol , Tabiques Cardíacos/diagnóstico por imagen , Tabiques Cardíacos/cirugía , Humanos , Estudios Retrospectivos , Resultado del Tratamiento
15.
J Cardiovasc Electrophysiol ; 21(6): 704-7, 2010 Jun 01.
Artículo en Inglés | MEDLINE | ID: mdl-20039988

RESUMEN

Knowledge on ventricular tachycardia (VT) in isolated ventricular noncompaction (IVNC) is limited. We report on a patient with IVNC who presented with cardiogenic shock due to an incessant drug-resistant VT that was cured by radiofrequency ablation. The VT had characteristics of a deep septal focal arrhythmia, which was distinctive by ablation-induced alternation of the rightward and leftward exits, and was difficult to ablate from either side of the ventricular septum.


Asunto(s)
Bloqueo de Rama/complicaciones , Bloqueo de Rama/fisiopatología , Taquicardia Ventricular/complicaciones , Taquicardia Ventricular/fisiopatología , Disfunción Ventricular/complicaciones , Disfunción Ventricular/fisiopatología , Adolescente , Amiodarona/uso terapéutico , Antiarrítmicos/uso terapéutico , Bloqueo de Rama/cirugía , Ablación por Catéter , Ecocardiografía , Electrocardiografía , Humanos , Hipertiroidismo/complicaciones , Masculino , Choque Cardiogénico/etiología , Choque Cardiogénico/cirugía , Taquicardia Ventricular/cirugía , Disfunción Ventricular/cirugía
16.
Pacing Clin Electrophysiol ; 33(5): 541-8, 2010 May.
Artículo en Inglés | MEDLINE | ID: mdl-20025720

RESUMEN

BACKGROUND: Left atrial (LA) endocardial voltage characteristics assessed during atrial fibrillation (AF) have not been previously compared in different AF types. This study was aimed at investigating the LA voltages and volumes in patients with paroxysmal and persistent AF. METHODS: LA electroanatomic voltage maps acquired during AF were compared between consecutive patients without major structural heart disease undergoing first catheter ablation for paroxysmal AF (n = 100) or persistent AF (n = 100). The groups were comparable in baseline clinical characteristics. RESULTS: Patients with persistent AF presented with lower median LA voltage (median 0.41, interquartile range [IQR] 0.31-0.51 mV versus median 0.99, IQR 0.47-1.56 mV; P < 0.001), and maximum LA voltage (4.07 +/- 1.76 vs 6.42 +/- 2.16 mV; P < 0.001). They also had a higher proportion of the LA points exhibiting voltage <0.2 mV (30 +/- 20 vs 12 +/- 11%; P < 0.001) and voltage 0.2-1.0 mV (55 +/- 15 vs 42 +/- 19%; P < 0.001). They further displayed higher LA volume/body surface area (75 +/- 16 vs 58 +/- 13 mL/m(2); P < 0.001). In the multivariate regression model, both LA voltage (P < 10(-9)) and LA volume (P < 10(-5)) were significant determinants of AF type. CONCLUSION: Patients with persistent AF had significantly lower LA voltage compared with patients with paroxysmal AF even after adjustment for differences in indexed LA volume. LA voltage represents an independent covariate of clinical manifestation of AF.


Asunto(s)
Fibrilación Atrial/fisiopatología , Atrios Cardíacos/fisiopatología , Adulto , Anciano , Fibrilación Atrial/cirugía , Mapeo del Potencial de Superficie Corporal , Ablación por Catéter , Fenómenos Electrofisiológicos , Femenino , Atrios Cardíacos/cirugía , Humanos , Persona de Mediana Edad , Resultado del Tratamiento , Adulto Joven
17.
Heart ; 106(6): 462-466, 2020 03.
Artículo en Inglés | MEDLINE | ID: mdl-31471463

RESUMEN

OBJECTIVE: The current guidelines suggest alcohol septal ablation (ASA) is less effective in hypertrophic obstructive cardiomyopathy (HOCM) patients with severe left ventricular hypertrophy, despite acknowledging that systematic data are lacking. Therefore, we analysed patients in the Euro-ASA registry to test this statement. METHODS: We compared the short-term and long-term outcomes of patients with basal interventricular septum (IVS) thickness <30 mm Hg to those with ≥30 mm Hg treated using ASA in nine European centres. RESULTS: A total of 1519 patients (57±14 years, 49% women) with symptomatic HOCM were treated, including 67 (4.4%) patients with IVS thickness ≥30 mm. The occurrence of short-term major adverse events were similar in both groups. The mean follow-up was 5.4±4.3 years and 5.1±4.1 years, and the all-cause mortality rate was 2.57 and 2.94 deaths per 100 person-years of follow-up in the IVS <30 mm group and the IVS ≥30 mm group (p=0.047), respectively. There were no differences in dyspnoea (New York Heart Association class III/IV 12% vs 16%), residual left ventricular outflow tract gradient (16±20 vs 16±16 mm Hg) and repeated septal reduction procedures (12% vs 18%) in the IVS <30 mm group and IVS ≥30 mm group, respectively (p=NS for all). CONCLUSIONS: The short-term results and the long-term relief of dyspnoea, residual left ventricular outflow obstruction and occurrence of repeated septal reduction procedures in patients with basal IVS ≥30 mm is similar to those with IVS <30mm. However, long-term all-cause and cardiac mortality rates are worse in the ≥30 mm group.


Asunto(s)
Cardiomiopatía Hipertrófica/terapia , Etanol , Hipertrofia Ventricular Izquierda/terapia , Tabique Interventricular/patología , Adulto , Anciano , Femenino , Humanos , Hipertrofia/terapia , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Índice de Severidad de la Enfermedad , Factores de Tiempo , Resultado del Tratamiento
18.
Arch Med Sci ; 15(3): 650-655, 2019 May.
Artículo en Inglés | MEDLINE | ID: mdl-31110530

RESUMEN

INTRODUCTION: The outcome of patients ≥ 60 years of age after alcohol septal ablation (ASA) for obstructive hypertrophic cardiomyopathy (HCM) remains unresolved. We sought to determine the long-term survival and the causes of death in this population. MATERIAL AND METHODS: We enrolled 156 consecutive patients (69 ±6 years, 69% women, follow-up: 4.8 ±3.5 years) who underwent ASA at ≥ 60 years of age. RESULTS: The 30-day mortality rate was 1.3%. At the last check-up, 81% of patients were in New York Heart Association class ≤ 2 and 76% had a left ventricular outflow tract gradient (LVOG) ≤ 30 mm Hg. A total of 39 patients died (51% of cardiovascular causes, 44% of non-cardiovascular causes, 5% of unknown causes) during the 734 patient-years. The annual sudden mortality, the sudden mortality and the all-cause mortality rates were 0.5%, 1.1%, and 4.8%, respectively. The all-cause mortality was higher compared to the age- and sex-matched general population (p = 0.002). CONCLUSIONS: Alcohol septal ablation was safe and effective in the long-term follow-up. We observed a reduced life expectancy compared to the age- and sex-matched general population. Mortality was almost equally due to cardiovascular and non-cardiovascular causes of death.

19.
Artículo en Inglés | MEDLINE | ID: mdl-29955186

RESUMEN

AIMS: Optimal ECG monitoring in detecting recurrences of atrial fibrillation (AF) or atrial tachycardia (AT) after catheter ablation has not been well established. The purpose of this prospective study was to compare the utility of daily ECG monitoring with episodic card recorder (ECR) vs. periodic monitoring with episodic loop recorder (ELR) for the detection of post-blanking AF/AT recurrences during early (Months 4-6) and late (Months 7-12) periods after catheter ablation for paroxysmal AF. METHODS: The study included 105 consecutive patients, who received ECR for 12 months and were instructed to send at least 2 random ECG recordings daily with extra-recordings during symptoms. The patients were simultaneously monitored for one week with ELR at the end of each period (Months 6 and 12). RESULTS: Thirty-one and 12 patients with AF/AT recurrence were identified by means of ECR and ELR, respectively. In patients with complete and valid data, ELR technology was inferior to ECR by detecting AF/AT in 5 (31%) of 16 and 5 (26%) of 19 patients with arrhythmia identified by ECR in the early and late period, respectively. Overall, ELR had a sensitivity of 8/23 (35%) for detecting AF/AT recurrence. There was no single patient with AF/AT recurrence on ELR that would not be known from ECR monitoring. Only 2 patients with arrhythmia recurrence were completely asymptomatic throughout the study period. CONCLUSION: Daily ECG monitoring with ECR was better than periodic monitoring with ELR in detecting AF/AT recurrences during the follow-up periods. Entirely asymptomatic patients with AF/AT recurrences were rare.


Asunto(s)
Fibrilación Atrial/fisiopatología , Fibrilación Atrial/cirugía , Ablación por Catéter , Electrocardiografía Ambulatoria , Complicaciones Posoperatorias/fisiopatología , Anciano , Antiarrítmicos , Fibrilación Atrial/diagnóstico , Ablación por Catéter/efectos adversos , Femenino , Humanos , Masculino , Persona de Mediana Edad , Complicaciones Posoperatorias/diagnóstico , Estudios Prospectivos , Recurrencia , Factores de Tiempo , Resultado del Tratamiento
20.
J Cardiol ; 74(6): 519-523, 2019 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-31255461

RESUMEN

BACKGROUND: Patent foramen ovale (PFO), male sex, age, and body mass index (BMI) were all identified as potential risk factors of decompression sickness (DCS). It has been debated whether PFO might cause unprovoked DCS (i.e. without violation of decompression procedure) due to paradoxical embolization of venous gas emboli. To date, there are no data on the incidence or risk factors of unprovoked DCS. This study sought to evaluate the risk factors of unprovoked DCS in recreational divers. METHODS: A total of 489 consecutive divers were screened for PFO between January 2006 and January 2014 by means of transcranial Doppler. All patients were prospectively included in the study registry. Survival analysis techniques were used to assess for risk factors for unprovoked DCS. Age, sex, BMI, PFO presence, and grade were analyzed. The total sum of dives was used as a measure of time. RESULTS: The group performed a total of 169,411 dives (mean 346±636). Thirty-six (7%) of the divers suffered from an unprovoked DCS. The frequency of PFO was 97.2% in divers with a history of unprovoked DCS and 35.5% in controls (p<0.001). There was no difference in sex, age, BMI, or total number of dives between the respective groups. In the adjusted Cox proportional hazards model, PFO grade 3 was a major risk factor for unprovoked DCS; there was a slight protective effect of increasing age. CONCLUSIONS: We demonstrated that a high-grade PFO was a major risk factor for unprovoked DCS in recreational scuba divers.


Asunto(s)
Enfermedad de Descompresión/etiología , Buceo/efectos adversos , Foramen Oval Permeable/complicaciones , Adulto , Femenino , Humanos , Masculino , Persona de Mediana Edad , Modelos de Riesgos Proporcionales , Estudios Prospectivos , Sistema de Registros , Factores de Riesgo
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