BACKGROUND: Anderson-Fabry disease (AFD) is an X-linked inherited lysosomal disease caused by a defect in the gene encoding lysosomal enzyme α-galactosidase A (GLA). Atrio-ventricular (AV) nodal conduction defects and sinus node dysfunction are common complications of the disease. It is not fully elucidated how frequently AFD is responsible for acquired AV block or sinus node dysfunction and if some AFD patients could manifest primarily with spontaneous bradycardia in general population. The purpose of study was to evaluate the prevalence of AFD in male patients with implanted permanent pacemaker (PM). METHODS: The prospective multicentric screening in consecutive male patients between 35 and 65 years with implanted PM for acquired third- or second- degree type 2 AV block or symptomatic second- degree type 1 AV block or sinus node dysfunction was performed. RESULTS: A total of 484 patients (mean age 54 ± 12 years at time of PM implantation) were enrolled to the screening in 12 local sites in Czech Republic. Out of all patients, negative result was found in 481 (99%) subjects. In 3 cases, a GLA variant was found, classified as benign: p.Asp313Tyr, p.D313Y). Pathogenic GLA variants (classical or non-classical form) or variants of unclear significance were not detected. CONCLUSION: The prevalence of pathogenic variants causing AFD in a general population sample with implanted permanent PM for AV conduction defects or sinus node dysfunction seems to be low. Our findings do not advocate a routine screening for AFD in all adult males with clinically significant bradycardia.

• Keywords
• Bradycardia, Fabry disease, Permanent pacing, Screening,
• MeSH
• Atrioventricular Block * diagnosis   epidemiology   therapy   MeSH
• Bradycardia complications   therapy   MeSH
• Adult MeSH
• Fabry Disease * diagnosis   epidemiology   genetics   MeSH
• Pacemaker, Artificial * adverse effects   MeSH
• Middle Aged MeSH
• Humans MeSH
• Prospective Studies MeSH
• Aged MeSH
• Sick Sinus Syndrome diagnosis   epidemiology   therapy   MeSH
• Check Tag
• Adult MeSH
• Middle Aged MeSH
• Humans MeSH
• Male MeSH
• Aged MeSH
• Publication type
• Journal Article MeSH

A 91-year-old woman, who had a pacemaker implanted in 1977, underwent replacement of a pulse generator and lead in 1995 because of recurrent syncope. The new lead dislodged the next day and migrated to the pulmonary artery. Because of her dependence on continued pacing, repeated resuscitation was required. Considering her advanced age and the impact of cardiopulmonary resuscitation on her general condition, we attempted catheter aided repositioning of the pacemaker lead. The procedure was technically successful; lead position was stable and optimal pacing parameters were attained. She was discharged in good condition.

• MeSH
• Pacemaker, Artificial * MeSH
• Humans MeSH
• Foreign-Body Migration therapy   MeSH
• Aged, 80 and over MeSH
• Aged MeSH
• Cardiac Catheterization * MeSH
• Check Tag
• Humans MeSH
• Aged, 80 and over MeSH
• Aged MeSH
• Female MeSH
• Publication type
• Journal Article MeSH
• Case Reports MeSH

From various points of view abandoned or displaced permanent pacemaker leads should be extracted in indicated cases using a broad spectrum of catheter-based techniques. We describe a relatively easy extraction procedure of almost the whole pacemaker lead, which broke near the pulse generator and migrated into right-heart chambers; so both ends were indwelling. The described procedure was undertaken using a simple homemade nitinol-based extractor.

• MeSH
• Foreign Bodies therapy   MeSH
• Electrodes, Implanted * MeSH
• Pacemaker, Artificial * MeSH
• Humans MeSH
• Device Removal MeSH
• Equipment Failure MeSH
• Aged, 80 and over MeSH
• Aged MeSH
• Alloys MeSH
• Sick Sinus Syndrome therapy   MeSH
• Check Tag
• Humans MeSH
• Male MeSH
• Aged, 80 and over MeSH
• Aged MeSH
• Publication type
• Journal Article MeSH
• Case Reports MeSH
• Names of Substances
• nitinol MeSH Browser
• Alloys MeSH

AIMS: Drug-related atrioventricular block (DR-AVB) may develop in patients with underlying latent degenerative conduction disorders, especially with antiarrhythmics and antihypertensives. Although, according to the current guidelines, reversal is achieved with cessation of the inducing agent, this is not the case for nearly half of the patients. The pathophysiological processes of DR-AVB and myringosclerosis include systemic inflammation and degeneration. This study investigated the role of myringosclerosis in predicting irreversible high-grade DR-AVB despite drug cessation. METHODS: This observational, non-randomized, prospective study involved 152 patients with high-grade DR-AVB, 72 of whom had reversible DR-AVB and 80 had irreversible DR-AVB and required permanent pacemakers. The patients' demographic, clinical, echocardiographic, and laboratory characteristics were recorded. Otoscopic tympanic membrane examinations for myringosclerosis were performed. RESULTS: There were no major differences in demographic, echocardiographic or laboratory characteristics between the two groups or previous medications. The median monitoring time with a temporary pacemaker was significantly longer in the irreversible than in the reversible group (5 [4-7] days vs. 2 [1-5] days; P<0.001). The incidence of myringosclerosis was significantly higher in the irreversible than in the reversible group (61.3% vs. 22.2%; P=0.001). Multivariate logistic regression analysis showed that myringosclerosis was an independent predictor of irreversible DR-AVB (odds ratio: 1.703, 95% confidence interval: 1.194-3.058; P=0.01). CONCLUSION: Myringosclerosis is a readily available, inexpensive, and non-invasive assessment and is a marker of inflammation and degeneration that can predict irreversible DR-AVB.

• Keywords
• atrioventricular block, myringosclerosis, pacemaker, tympanosclerosis,
• MeSH
• Atrioventricular Block * etiology   therapy   MeSH
• Pacemaker, Artificial * adverse effects   MeSH
• Humans MeSH
• Myringosclerosis * complications   MeSH
• Prospective Studies MeSH
• Risk Factors MeSH
• Treatment Outcome MeSH
• Inflammation MeSH
• Check Tag
• Humans MeSH
• Publication type
• Journal Article MeSH
• Observational Study MeSH

OBJECTIVE: The onset of new conduction abnormalities requiring permanent pacemaker implantation (PPI) after transcatheter aortic valve implantation (TAVI) is still a relevant adverse event. The main objective of this registry was to identify modifiable procedural risk factors for an improved outcome (lower rate of PPI) after TAVI in patients at high risk of PPI. METHODS: Patients from four European centres receiving a balloon-expandable TAVI (Edwards SAPIEN 3/3 Ultra) and considered at high risk of PPI (pre-existing conduction disturbance, heavily calcified left ventricular outflow tract or short membranous septum) were prospectively enrolled into registry. RESULTS: A total of 300 patients were included: 42 (14.0%) required PPI after TAVI and 258 (86.0%) did not. Patients with PPI had a longer intensive care unit plus intermediate care stay (65.7 vs 16.3 hours, p<0.001), general ward care stay (6.9 vs 5.3 days, p=0.004) and later discharge (8.6 vs 5.0 days, p<0.001). Of the baseline variables, only pre-existing right bundle branch block at baseline (OR 6.8, 95% CI 2.5 to 18.1) was significantly associated with PPI in the multivariable analysis. Among procedure-related variables, oversizing had the highest impact on the rate of PPI: higher than manufacturer-recommended sizing, mean area oversizing as well as the use of the 29 mm valve (OR 3.4, 95% CI 1.4 to 8.5, p=0.008) all were significantly associated with PPI. Rates were higher with the SAPIEN 3 (16.1%) vs SAPIEN 3 Ultra (8.5%), although not statistically significant but potentially associated with valve sizing. Implantation depth and postdelivery balloon dilatation also tended to affect PPI rates but without a statistical significance. CONCLUSION: Valve oversizing is a strong procedure-related risk factor for PPI following TAVI. The clinical impact of the valve type (SAPIEN 3), implantation depth, and postdelivery balloon dilatation did not reach significance and may reflect already refined procedures in the participating centres, giving attention to these avoidable risk factors. TRIAL REGISTRATION NUMBER: NCT03497611.

• Keywords
• aortic valve stenosis, pacemaker, artificial, transcatheter aortic valve replacement,
• MeSH
• Aortic Valve Stenosis * surgery   MeSH
• Cardiac Pacing, Artificial adverse effects   methods   MeSH
• Pacemaker, Artificial * MeSH
• Humans MeSH
• Registries MeSH
• Risk Factors MeSH
• Heart Valve Prosthesis * adverse effects   MeSH
• Transcatheter Aortic Valve Replacement * adverse effects   MeSH
• Check Tag
• Humans MeSH
• Publication type
• Journal Article MeSH
• Clinical Trial MeSH
• Research Support, Non-U.S. Gov't MeSH

INTRODUCTION: Use of the mechanically expandable transcatheter aortic valve (MEV) has been recently linked to increased risks of valve dysfunction and cardiovascular mortality. The risk of developing conduction disturbance with the MEV valve is well known, and the negative prognostic impact of permanent pacemaker implantation (PPI) after transcatheter aortic valve implantation is another consideration. AIM: This study aimed to compare the mid-term survival of patients with MEV and self-expandable valves (SEV), and to examine survival of both groups according to the presence or absence of PPI. METHODS: This single-centre, retrospective, observational study examined data from MEV and SEV groups comprising 92 and 373 patients, respectively. The mean clinical follow-up was 2.5±1.7 years. Mortality information was obtained from the National Institutes of Health Information and Statistics. RESULTS: Baseline characteristics were comparable between the groups. The log-rank test showed higher cardiovascular mortality in the MEV group (p=0.042; the relative risk (RR) 1.594 (95% CI 1.013 to 2.508)). The Cox proportional hazards model identified MEV implantation as an independent predictor of cardiovascular mortality. The rate of PPI was twice as high in the MEV vs SEV group (33.7% vs 16.1%; p<0.001). We compared the survival of both groups according to the presence or absence of PPI and found higher mortality in the MEV group without PPI versus the SEV group without PPI (p=0.007; RR 2.156 (95% CI 1.213 to 3.831)). Survival did not differ in the groups with PPI. CONCLUSIONS: A higher mid-term cardiovascular mortality rate was observed with MEV versus SEV implants. Comparing both groups according to the presence or absence of PPI, we observed a higher mortality risk in patients with MEV without PPI than in SEV without PPI. In contrast, mortality did not differ between the groups when PPI was implanted.

• Keywords
• Aortic Valve Stenosis, Death, Sudden, Cardiac, Pacemaker, Artificial, Transcatheter Aortic Valve Replacement,
• MeSH
• Aortic Valve diagnostic imaging   surgery   MeSH
• Aortic Valve Stenosis * diagnostic imaging   surgery   MeSH
• Pacemaker, Artificial * MeSH
• Humans MeSH
• Retrospective Studies MeSH
• Risk Factors MeSH
• Treatment Outcome MeSH
• Check Tag
• Humans MeSH
• Publication type
• Journal Article MeSH
• Observational Study MeSH

OBJECTIVES: The occurrence of new conduction abnormalities necessitating permanent pacemaker implantation (PPI) is a complication of transcatheter aortic valve replacement (TAVR). Previous studies have shown inconsistent results about the clinical impact of new PPI after TAVR. METHODS: CONDUCT was a prospective observational registry that enrolled 295 patients undergoing TAVR at four European centres. The primary goal of this registry was to compare 1-year clinical outcomes in TAVR patients with or without PPI, using one-to-four propensity score matched (PSM) analysis. It also assessed major adverse cardiac events (MACE) in patients undergoing right ventricular pacing after PPI. RESULTS: Out of 160 PSM patients, 36 underwent PPI and the other 124 had no PPI within 30 days post-TAVR. The median age of the patients was 80 years, with more men (80.6% and 84.7% in patients with and without PPI, respectively) and similar EuroSCORE II and Society of Thoracic Surgeons scores. Patients with PPI had higher diabetes prevalence (p=0.055) and lower left ventricular ejection fraction percentages (p=0.034), but higher systolic pulmonary artery pressure (p=0.013) than those without PPI. However, these differences diminished after PSM. At 1 year, PPI patients had a nonsignificant but slightly higher incidence of MACE (22.2% versus 13.7%; p=0.216) (HR 1.63; 95% CI [0.72-3.71]) driven by increased heart failure (11.1% versus 2.4%; p=0.046) (HR 5.05; 95% CI [1.09-23.4]). Freedom from all-cause mortality, cardiovascular death, stroke and endocarditis at 1-year follow-up was comparable between groups. CONCLUSION: Despite a higher incidence of congestive heart failure rehospitalisation in patients undergoing PPI, 1-year clinical outcomes were similar in both groups.

• Keywords
• Aortic stenosis, permanent pacemaker implantation, transcatheter aortic valve replacement,
• Publication type
• Journal Article MeSH

BACKGROUND: His-Bundle pacing (HBP) is an emerging technique for physiological pacing. However, its effects on right ventricle (RV) performance are still unknown. METHODS: We enrolled consecutive patients with an indication for pacemaker (PM) implantation to compare HBP versus RV pacing (RVP) effects on RV performance. Patients were evaluated before implantation and after 6 months by a transthoracic echocardiogram. RESULTS: A total of 84 patients (age 75.1±7.9 years, 64% male) were enrolled, 42 patients (50%) underwent successful HBP, and 42 patients (50%) apical RVP. At follow up, we found a significant improvement in RV-FAC (Fractional Area Change)% [baseline: HBP 34 IQR (31-37) vs. RVP 33 IQR (29.7-37.2),p = .602; 6-months: HBP 37 IQR (33-39) vs. RVP 30 IQR (27.7-35), p < .0001] and RV-GLS (Global Longitudinal Strain)% [baseline: HBP -18 IQR (-20.2 to -15) vs. RVP -16 IQR (-18.7 to -14), p = .150; 6-months: HBP -20 IQR(-23 to -17) vs. RVP -13.5 IQR (-16 to -11), p < .0001] with HBP whereas RVP was associated with a significant decline in both parameters. RVP was also associated with a significant worsening of tricuspid annular plane systolic excursion (TAPSE) (p < .0001) and S wave velocity (p < .0001) at follow up. Conversely from RVP, HBP significantly improved pulmonary artery systolic pressure (PASP) [baseline: HBP 38 IQR (32-42) mmHg vs. RVP 34 IQR (31.5-37) mmHg,p = .060; 6-months: HBP 32 IQR (26-38) mmHg vs. RVP 39 IQR (36-41) mmHg, p < .0001] and tricuspid regurgitation (p = .005) irrespectively from lead position above or below the tricuspid valve. CONCLUSIONS: In patients undergoing PM implantation, HBP ensues a beneficial and protective impact on RV performance compared with RVP.

• Keywords
• His-Bundle pacing, conduction system pacing, echocardiogram, pacemaker, right ventricle,
• MeSH
• Ventricular Dysfunction, Right physiopathology   therapy   MeSH
• Bundle of His physiopathology   MeSH
• Cardiac Pacing, Artificial methods   MeSH
• Pacemaker, Artificial * MeSH
• Humans MeSH
• Aged MeSH
• Stroke Volume MeSH
• Check Tag
• Humans MeSH
• Male MeSH
• Aged MeSH
• Female MeSH
• Publication type
• Journal Article MeSH

Permanent pacemaker implantation (PPI) is a widely recognized complication associated with TAVI (incidence up to 20%). Smaller registries have identified several variables associated with PPI. The objective was to validate patient- and transcatheter aortic valve implantation (TAVI)-related procedural variables associated with PPI. We performed a retrospective analysis of patients from six European centers undergoing TAVI with the Edwards SAPIEN 3 prosthesis. Baseline variables and pre-procedural ECG characteristics and CT-scans were taken into account. Data for 1745 patients were collected; 191 (10.9%) required PPI after TAVI. The baseline variables pulmonary hypertension (OR 1.64; 95% CI 1.01-2.59), QRS duration > 117 ms (OR 2.58; 95% CI 1.73-3.84), right bundle branch block (RBBB; OR 5.14; 95% CI 3.39-7.72), left anterior hemi block (OR 1.92; 95% CI 1.19-3.02) and first-degree atrioventricular block (AVB, OR 1.63; 95%CI 1.05-2.46) were significantly associated with PPI. RBBB (OR 8.11; 95% CI 3.19-21.86) and first-degree AVB (OR 2.39; 95% CI 1.18-4.66) remained significantly associated in a multivariate analysis. Procedure-related variables included access site (TF; OR 1.97; 95% CI 1.07-4.05), implanted valve size (29 mm; OR 1.88; 95% CI 1.35-2.59), mean TAVI valve implantation depth below the annulus > 30% (OR 3.75; 95% CI 2.01-6.98). Patients receiving PPI had longer ICU stays and later discharges. Acute kidney injury stage 2/3 was more common in patients with PPI until discharge (15.2 vs. 3.1%; p = 0.007), but was not statistically significant thereafter. Further differences in outcomes at 30 days did not reach significance. The data will aid pre- and post-procedural patient management and prevent adverse long-term outcomes.Clinical Trial: NCT03497611.

• Keywords
• Aortic stenosis, Pacemaker, Transcatheter aortic valve implantation,
• MeSH
• Aortic Valve diagnostic imaging   physiopathology   surgery   MeSH
• Aortic Valve Stenosis diagnostic imaging   physiopathology   surgery   MeSH
• Balloon Valvuloplasty adverse effects   MeSH
• Time Factors MeSH
• Risk Assessment MeSH
• Cardiac Pacing, Artificial * MeSH
• Pacemaker, Artificial * MeSH
• Humans MeSH
• Registries MeSH
• Retrospective Studies MeSH
• Risk Factors MeSH
• Aged, 80 and over MeSH
• Aged MeSH
• Heart Block diagnosis   etiology   physiopathology   therapy   MeSH
• Heart Valve Prosthesis * MeSH
• Heart Rate * MeSH
• Transcatheter Aortic Valve Replacement adverse effects   instrumentation   MeSH
• Treatment Outcome MeSH
• Check Tag
• Humans MeSH
• Male MeSH
• Aged, 80 and over MeSH
• Aged MeSH
• Female MeSH
• Publication type
• Journal Article MeSH
• Multicenter Study MeSH
• Geographicals
• Europe MeSH

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.

• Keywords
• alcohol septal ablation, permanent pacemaker, prognosis,
• MeSH
• Ablation Techniques * adverse effects   MeSH
• Atrioventricular Block * diagnosis   etiology   therapy   MeSH
• Ethanol adverse effects   MeSH
• Cardiomyopathy, Hypertrophic * diagnostic imaging   surgery   MeSH
• Pacemaker, Artificial * MeSH
• Humans MeSH
• Retrospective Studies MeSH
• Heart Septum diagnostic imaging   surgery   MeSH
• Treatment Outcome MeSH
• Check Tag
• Humans MeSH
• Publication type
• Journal Article MeSH
• Names of Substances
• Ethanol MeSH