Transseptal puncture for left atrial ablation: Risk factors for cardiac tamponade and a proposed causative classification system.

AIMS: Cardiac tamponade is a high morbidity complication of transseptal puncture (TSP). We examined the associations of TSP-related cardiac tamponade (TRCT) for all patients undergoing left atrial ablation at our center from 2016 to 2020. METHODS AND RESULTS: Patient and procedural variables were extracted retrospectively. Cases of cardiac tamponade were scrutinized to adjudicate TSP culpability. Adjusted multivariate analysis examined predictors of TRCT. A total of 3239 consecutive TSPs were performed; cardiac tamponade occurred in 51 patients (incidence: 1.6%) and was adjudicated as TSP-related in 35 (incidence: 1.1%; 68.6% of all tamponades). Patients of above-median age [odds ratio (OR): 2.4 (1.19-4.2), p = .006] and those undergoing re-do procedures [OR: 1.95 (1.29-3.43, p = .042] were at higher risk of TRCT. Of the operator-dependent variables, choice of transseptal needle (Endrys vs. Brockenbrough, p > .1) or puncture sheath (Swartz vs. Mullins vs. Agilis vs. Vizigo vs. Cryosheath, all p > .1) did not predict TRCT. Adjusting for operator, equipment and demographics, failure to cross the septum first pass increased TRCT risk [OR: 4.42 (2.45-8.2), p = .001], whilst top quartile operator experience [OR: 0.4 (0.17-0.85), p = .002], transoesophageal echocardiogram [TOE prevalence: 26%, OR: 0.51 (0.11-0.94), p = .023], and use of the SafeSept transseptal guidewire [OR: 0.22 (0.08-0.62), p = .001] reduced TRCT risk. An increase in transseptal guidewire use over time (2016: 15.6%, 2020: 60.2%) correlated with an annual reduction in TRCT (R2 = 0.72, p < .001) and was associated with a relative risk reduction of 70%. CONCLUSIONS: During left atrial ablation, the risk of TRCT was reduced by operator experience, TOE-guidance, and use of a transseptal guidewire, and was increased by patient age, re-do procedures, and failure to cross the septum first pass.

A multi-center experience of ablation index for evaluating lesion delivery in typical atrial flutter.

BACKGROUND: Anatomical studies demonstrate significant variation in cavotricuspid isthmus (CTI) architecture. METHODS: Thirty-eight patients underwent CTI ablation at two tertiary centers. Operators delivered 682 lesions with a target ablation index (AI) of 600 Wgs. Ablation parameters were recorded every 10-20 ms. Post hoc, Visitags were trisected according to CTI position: inferior vena cava (IVC), middle (Mid), or ventricular (V) lesions. RESULTS: There were no complications. 92.1% of patients (n = 35) remained in sinus rhythm after 14.6 ± 3.4 months. For the whole CTI, peak AI correlated with mean impedance drop (ID) (R2  = 0.89, p < .0001). However, analysis by anatomical site demonstrated a non-linear relationship Mid CTI (R2  = 0.15, p = .21). Accordingly, while mean AI was highest Mid CTI (IVC: 473.1 ± 122.1 Wgs, Mid: 539.6 ± 103.5 Wgs, V: 486.2 ± 111.8 Wgs, ANOVA p < .0001), mean ID was lower (IVC: 10.7 ± 7.5Ω, Mid: 9.0 ± 6.5Ω, V: 10.9 ± 7.3Ω, p = .011), and rate of ID was slower (IVC: 0.37 ± 0.05 Ω/s, Mid: 0.18 ± 0.08 Ω/s, V: 0.29 ± 0.06 Ω/s, p < .0001). Mean contact force was similar at all sites; however, temporal fluctuations in contact force (IVC: 19.3 ± 12.0 mg/s, Mid: 188.8 ± 92.1 mg/s, V: 102.8 ± 32.3 mg/s, p < .0001) and catheter angle (IVC: 0.42°/s, Mid: 3.4°/s, V: 0.28°/s, p < .0001) were greatest Mid CTI. Use of a long sheath attenuated these fluctuations and improved energy delivery. CONCLUSIONS: Ablation characteristics vary across the CTI. At the Mid CTI, higher AI values do not necessarily deliver more effective ablation; this may reflect localized fluctuations in catheter angle and contact force.

Correction: Seed germination ecology of Conyza stricta Willd. and implications for management.

[This corrects the article DOI: 10.1371/journal.pone.0244059.].

First experience of POLARx™ versus Arctic Front Advance™: An early technology comparison.

INTRODUCTION: Cryoballoon ablation is an established technique to achieve pulmonary vein isolation in patients with atrial fibrillation (AF). Recently, a new manufacturer of cryoballoon achieved regulatory CE marking (POLARx™; Boston Scientific). We describe our early experience of using this new market entrant of the technology and describe procedural aspects in comparison to the incumbent Medtronic Arctic Front Advance™. METHODS: We assessed the first 40 AF ablations performed with the POLARx catheter at the Barts Heart Centre. These patients were compared with a contemporaneous series of patients undergoing ablation by the same operators using the Arctic Front Advance. Procedural metrics were prospectively recorded. RESULTS: A total of four operators undertook 40 cases using the POLARx catheter, compared with 40 cases using the Arctic Front Advance. Procedure times (60.0 vs. 60.0 min) were similar between the two technologies, however left atrial dwell time (35.0 vs 39.0 min) and fluoroscopy times (3.3 vs. 5.2 min) were higher with the POLARx. Measured nadir and isolation balloon temperatures were significantly lower with POLARx. Almost all veins were isolated with a median freezing time of 16.0 (POLARx) versus 15.0 (Arctic Front Advance) min. The rate of procedural complications was low in both groups. CONCLUSION: The POLARx cryoballoon is effective for pulmonary vein isolation. Measured isolation and nadir temperatures are lower compared with the predicate Arctic Front Advance catheter. The technology appears similar in acute efficacy and has a short learning curve, but formal dosing studies may be required to prove equivalence of efficacy.

Seed germination ecology of Conyza stricta Willd. and implications for management.

Numerous cropping systems of the world are experiencing the emergence of new weed species in response to conservation agriculture. Conyza stricta Willd. is being a newly emerging weed of barley-based cropping systems in response to conservational tillage practices. Seed germination ecology of four populations (irrigated, rainfed, abandoned and ruderal habitats) was studied in laboratory and greenhouse experiments. The presence/absence of seed dormancy was inferred first, which indicated seeds were non-dormant. Seed germination was then recorded under various photoperiods, constant and alternating day/night temperatures, and pH, salinity and osmotic potential levels. Seedling emergence was observed from various seed burial depths. Seeds of all populations proved photoblastic and required 12-hour light/dark period for germination. Seeds of all populations germinated under 5-30°C constant temperature; however, peak germination was recorded under 17.22-18.11°C. Nonetheless, the highest germination was noted under 20/15°C alternating day/night temperature. Ruderal and irrigated populations better tolerated salinity and germinated under 0-500 mM salinity. Similarly, rainfed population proved more tolerant to osmotic potential than other populations. Seeds of all populations required neutral pH for the highest germination, whereas decline was noted in germination under basic and alkaline pH. Seedling emergence was retarded for seeds buried >2 cm depth and no emergence was recorded from >4 cm depth. These results add valuable information towards our understanding of seed germination ecology of C. stricta. Seed germination ability of different populations under diverse environmental conditions suspects that the species can present severe challenges in future if not managed. Deep seed burial along with effective management of the emerging seedlings seems a pragmatic option to manage the species in cultivated fields. However, immediate management strategies are needed for rest of the habitats.

Non-vitamin K oral anticoagulants at the time of cardiac rhythm device surgery: A systematic review and meta-analysis.

INTRODUCTION: Use of non-vitamin K oral anticoagulants (NOACs) has rapidly increased worldwide. We aimed to systematically assess the available evidence regarding the safety and efficacy of NOACs in patients undergoing cardiac implantable electronic device (CIED) surgery. METHODS: We performed a systematic literature search. Eligible randomised controlled trials and cohort studies were included. The primary outcome measures were clinically significant device-pocket haematoma and thromboembolic events. RESULTS: A total of 12 studies were included, equating to 2120 patients. The separate pooling of rate of events showed a low incidence of clinically significant device-pocket haematoma, although numerically higher in patients on continued (1.5%; CI95%0.8-3.0) versus interrupted NOAC (0.9%; CI95%0.5-1.7). The rate of any device-pocket haematoma was numerically higher in the continued versus interrupted NOAC group (5.4%; CI95%3.8-7.7 versus 2.4%; CI95%1.8-3.3). The incidence of thromboembolic events (0.4%; CI95%0.2-0.8) was low and comparable. From a meta-analysis of 3 studies (equating to 773 subjects) allowing for a comparison of continued versus interrupted NOAC, we found no significant difference between the 2 strategies in terms of clinically significant pocket haematoma (RR1.14; CI95%0.43-3.06, p = 0.79), thromboembolic complications (RR1.03; CI95%0.06-16.37, p = 0.98), and any pocket haematoma (RR1.19; CI95%0.65-2.20, p = 0.57). CONCLUSION: Use of NOACs at the time of CIEDs surgery appears to be safe, and either strategy of peri-procedure continuation or interruption might be reasonable. However, continuation of NOAC seems to be associated with a numerically higher rate of bleeding complications. Certainty of the evidence is low, and further studies are required to confirm these findings.

Full blood count as potential predictor of outcomes in patients undergoing cardiac resynchronization therapy.

Almost a third of patients fulfilling current guidelines criteria have suboptimal responses following cardiac resynchronization therapy (CRT). Circulating biomarkers may help identify these patients. We aimed to assess the predictive role of full blood count (FBC) parameters in prognosis of heart failure (HF) patients undergoing CRT device implantation. We enrolled 612 consecutive CRT patients and FBC was measured within 24 hours prior to implantation. The follow-up period was a median of 1652 days (IQR: 837-2612). The study endpoints were i) composite of all-cause mortality or transplant, and ii) reverse left ventricular (LV) remodeling. On multivariate analysis [hazard ratio (HR), 95% confidence interval (CI)] only red cell count (RCC) (p = 0.004), red cell distribution width (RDW) (p < 0.001), percentage of lymphocytes (p = 0.03) and platelet count (p < 0.001) predicted all-cause mortality. Interestingly, RDW (p = 0.004) and platelet count (p = 0.008) were independent predictors of reverse LV remodeling. This is the first powered single-centre study to demonstrate that RDW and platelet count are independent predictors of long-term all-cause mortality and/or heart transplant in CRT patients. Further studies, on the role of these parameters in enhancing patient selection for CRT implantation should be conducted to confirm our findings.

Anticoagulation and the risk of complications in ventricular tachycardia and premature ventricular complex ablation.

BACKGROUND: Many patients undergoing ventricular tachycardia (VT) or premature ventricular complex (PVC) ablation receive antithrombotic medications.  Their uninterrupted use has the potential to affect complication rates. We assessed the incidence of complications in a large cohort of patients undergoing these procedures, according to antithrombotic medication use. METHODS: From June 2014 to June 2016, 201 VT and PVC ablations were performed at a single center. We allocated patients to three groups: (A) anticoagulation group (international normalized ratio ≥ 1.5 or non-vitamin K anticoagulant or full-dose low-molecular-weight (LMW) heparin on day of procedure); (B) antithrombotic group (antiplatelet therapy and/or prophylactic LMW heparin on day of procedure); and (C) no antithrombotics group.  We assessed periprocedural complication rates in each group.  Multivariable analysis was performed. RESULTS: Group A (47 patients) had 8.5% procedural complication rate: one stroke, one pseudoaneurysm, one femoral artery occlusion, and one access site hematoma. In this group, 37 patients had femoral arterial and 18 had epicardial access. In Group B (46 patients), the complication rate was 6.5%: two cardiac tamponades and one pericardial effusion without compromise. Group C (108 patients) had a 5.6% complication rate: three cardiac tamponades (with one periprocedural death and one concomitant gastric vessel injury), one pericardial effusion without compromise, one stomach perforation, and two access site hematomas. Multivariable analysis did not show any significant predictors of complications, though age approached significance. CONCLUSIONS: Complication rates were not significantly different between groups. These findings suggest that VT and PVC ablation can be performed safely in patients with uninterrupted antithrombotic medications.

Radiofrequency ablation of atrial tachyarrhythmias in adults with tetralogy of Fallot - predictors of success and outcome.

BACKGROUND: Adults with tetralogy of Fallot experience atrial tachyarrhythmias; however, there are a few data on the outcomes of radiofrequency ablation. We examined the characteristics, outcome, and predictors of recurrence of atrial tachyarrhythmias after radiofrequency ablation in tetralogy of Fallot patients. Methods/results Retrospective data were collected from 2004 to 2013. In total, 56 ablations were performed on 37 patients. We identified two matched controls per case: patients with tetralogy of Fallot but no radiofrequency ablation and not known to have atrial tachyarrhythmias. Acute success was 98%. Left atrial arrhythmias increased in frequency over time. The mean follow-up was 41 months; 78% were arrhythmia-free. Number of cardiac surgeries, age, and presence of atrial fibrillation were predictors of recurrence. Lone cavo-tricuspid isthmus-dependent flutter reduced the likelihood of atrial fibrillation. Right and left atria in patients with tetralogy of Fallot were larger in ablated cases than controls. NYHA class was worse in cases and improved after ablation; baseline status predicted death. Of matched non-ablated controls, a number of them had atrial fibrillation. These patients were excluded from the case-control study but analysed separately. Most of them had died during follow-up, whereas of the matched ablated cases all were alive and the majority in sinus rhythm. CONCLUSION: Patients with tetralogy of Fallot and atrial tachyarrhythmias have more dilated atria than those without atrial tachyarrhythmias. Radiofrequency ablation improves functional status. Left atrial ablation is more commonly required with repeat procedures. There is a high prevalence of atrial tachyarrhythmias, particularly atrial fibrillation, in patients with tetralogy of Fallot; early radiofrequency ablation may have a protective effect against this.

Comparison Of Efficacy Of Chloroquine And Artemether/Lumafantrine In Treating Vivax Malaria In Thall And Surrounding Area.

BACKGROUND: Fever is the main complaint in patients reporting to our hospital and the most common cause of fever in our set up is malaria. The aim of this study was to know about the clinical response, efficacy and resistance of vivax malaria to chloroquine in patients reporting to Thall Scouts Hospital. METHODS: All the adult male patients reporting to Thall Scouts Hospital with fever and other symptoms of malaria having slide positive vivax malaria were included in the study. Both thick and thin slide were used for the diagnosis and species determination of malaria. Age group of the patients was from 18-40 years old. The study was conducted for the period of two years. RESULTS: Total number of patients included in the study was 518. Of the 518 patients, 374 (72.2%) responded to chloroquine and the remaining 144 (27.8%) were given Arthemether/Lumafantrine combination. Having positive symptoms of malaria total 374 patients treated with chloroquine 171 (45.72%) were asymptomatic after 24 hours, 98 (26.2%) after 48 hours, 78 (20.86%), after 72 hours of treatment while 27 (7.22%) were found to be resistant to chloroquine. Of the 144 patients having positive malaria treated with Artemether/Lumafantrine 62 (43.06%) were asymptomatic after 24 hours, 65 (45.14%) after 48 hours, 13 (9.03%) after 72 hours while 4 (2.78%) had still positive symptoms of malaria. CONCLUSIONS: Vivax malaria in our set up is sensitive to both Chloroquine and Arthemether/Lumafantrine. As Chloroquine is a cheap and easily available drug, so it can be safely given to patients with vivax malaria. It will also decrease the total cost of the disease.

Ventricular Arrhythmia Burden in Patients With Heart Failure and Cardiac Resynchronization Devices: The Importance of Renal Function.

BACKGROUND: Chronic kidney disease (CKD) is a risk factor for arrhythmias in patients with heart failure (HF). However, the effects of CKD on ventricular arrhythmia (VA) burden in patients with cardiac resynchronization therapy and defibrillator (CRT-D) devices in a primary prevention setting are unknown. OBJECTIVE: To determine whether baseline CKD is associated with increased risk of VA in patients implanted with primary prevention CRT-D devices. METHODS AND RESULTS: In this retrospective study, 199 consecutive primary prevention CRT-D recipients (2005-2010) were stratified by estimated glomerular filtration rate (eGFR) levels prior to device implantation with 106 (53.2%) ≥CKD III (eGFR < 60 mL/min/1.73 m2 ) (CKD group). CKD group patients were significantly older (70.0 ± 10 years vs. 61.3 ± 12 years, P < 0.05) with higher prevalence of ischemic cardiomyopathy (56.2% vs. 40.2%, P < 0.05). Detected ventricular tachycardia (VT)/ventricular fibrillation (VF) episodes resulting in device therapy occurred significantly more frequently in the CKD group [40/106(37.8%)] than controls [24/93(25.8%)], (odd ratio [OR] = 1.74, 95% confidence interval [CI] = 1.01-3.2, P = 0.05). At 5-year follow-up, interval censored data analysis showed 41% VT/VF incidence in the CKD group compared to 24% incidence in controls (P < 0.05). Cox proportional hazards model identified CKD > III as the only predictor of sustained VA in this group (adjusted hazard ratio [HR] 2.92, CI = 1.39-6.1, P = 0.004). CONCLUSION: Baseline CKD is a strong independent risk factor for VA in primary prevention CRT-D recipients. Further understanding of the underlying arrhythmogenic mechanisms relating to CKD may be of interest to allow appropriate correction and prevention. Device programming in this cohort may need to reflect this increased risk.

Ventricular stimulus site influences dynamic dispersion of repolarization in the intact human heart.

The spatial variation in restitution properties in relation to varying stimulus site is poorly defined. This study aimed to investigate the effect of varying stimulus site on apicobasal and transmural activation time (AT), action potential duration (APD) and repolarization time (RT) during restitution studies in the intact human heart. Ten patients with structurally normal hearts, undergoing clinical electrophysiology studies, were enrolled. Decapolar catheters were placed apex to base in the endocardial right ventricle (RVendo) and left ventricle (LVendo), and an LV branch of the coronary sinus (LVepi) for transmural recording. S1-S2 restitution protocols were performed pacing RVendo apex, LVendo base, and LVepi base. Overall, 725 restitution curves were analyzed, 74% of slopes had a maximum slope of activation recovery interval (ARI) restitution (Smax) > 1 (P < 0.001); mean Smax = 1.76. APD was shorter in the LVepi compared with LVendo, regardless of pacing site (30-ms difference during RVendo pacing, 25-ms during LVendo, and 48-ms during LVepi; 50th quantile, P < 0.01). Basal LVepi pacing resulted in a significant transmural gradient of RT (77 ms, 50th quantile: P < 0.01), due to loss of negative transmural AT-APD coupling (mean slope 0.63 ± 0.3). No significant transmural gradient in RT was demonstrated during endocardial RV or LV pacing, with preserved negative transmural AT-APD coupling (mean slope -1.36 ± 1.9 and -0.71 ± 0.4, respectively). Steep ARI restitution slopes predominate in the normal ventricle and dynamic ARI; RT gradients exist that are modulated by the site of activation. Epicardial stimulation to initiate ventricular activation promotes significant transmural gradients of repolarization that could be proarrhythmic.

Applicability of a risk score for prediction of the long-term benefit of the implantable cardioverter defibrillator in patients receiving cardiac resynchronization therapy.

AIMS: The Goldenberg risk score, comprising five clinical risk factors (New York Heart Association class >2, atrial fibrillation, QRS duration >120 ms, age >70 years, and urea >26 mg/dL), may help identify patients in whom the survival benefit of the defibrillator may be limited. We aim at assessing whether this score can accurately predict the long-term all-cause mortality risk of patients receiving cardiac resynchronization therapy (CRT) and identify those who are more likely to benefit from the defibrillator. METHODS AND RESULTS: In this retrospective observational cohort study, 638 patients with ischaemic or non-ischaemic dilated cardiomyopathy who had CRT-defibrillator (CRT-D) (n = 224) vs. CRT-pacemaker (CRT-P) (n = 414) implantation were prospectively followed up for survival outcomes. The long-term outcome of patients with CRT-D vs. CRT-P was compared within risk score categories and in patients with severe renal dysfunction. Mean follow-up in surviving and deceased patients was 62.7 and 32.5 months, respectively. This score showed higher discriminative performance in all-cause mortality prediction in CRT-D vs. CRT-P patients (area under the curve 0.718 ± 0.041 vs. 0.650 ± 0.032, respectively, P = 0.001). In those with scores 0-2, a CRT-D device decreased mortality rates in the first 4 years of follow-up compared with CRT-P (11.3 vs. 24.7%, P = 0.041), but this effect attenuated with longer follow-up duration (21.2 vs. 32.7%, P = 0.078). In this group, the benefit of CRT-D during the follow-up was seen after adjusting for traditional mortality predictors (hazard ratio 0.339, P = 0.001). No significant differences in mortality rates were seen in patients with score ≥3 (57.9% with CRT-D vs. 56.9%, P = 0.8) and those with severe renal dysfunction (92.9% in CRT-D vs. 76.2%, P = 0.17). Similar results were seen following propensity score matching. CONCLUSION: A simple risk stratification score comprising five clinical risk factors may help identify CRT patients who are more likely to benefit from the presence of the defibrillator.

Decrement Evoked Potential Mapping: Basis of a Mechanistic Strategy for Ventricular Tachycardia Ablation.

BACKGROUND: Substrate-based mapping for ventricular tachycardia (VT) ablation is hampered by its inability to determine critical sites of the VT circuit. We hypothesized that those potentials, which delay with a decremental extrastimulus (decrement evoked potentials or DEEPs), are more likely to colocalize with the diastolic pathways of VT circuits. METHODS AND RESULTS: DEEPs were identified in intraoperative left ventricular maps from 6 patients with ischemic cardiomyopathy (total 9 VTs) and were compared with late potential (LP) and activation maps of the diastolic pathway for each VT. Mathematical modeling was also used to further validate and elucidate the mechanisms of DEEP mapping. All patients demonstrated regions of DEEPs and LPs. The mean endocardial surface area of these potentials was 18±4% and 21±6%, respectively (P=0.13). The mean sensitivity for identifying the diastolic pathway in VT was 50±23% for DEEPs and 36±32% for LPs (P=0.31). The mean specificity was 43±23% versus 20±8% for DEEP and LP mapping, respectively (P=0.031). The electrograms that displayed the greatest decrement in each case had a sensitivity and specificity for the VT isthmus of 29±10% and 95±1%, respectively. Mathematical modeling studies recapitulated DEEPs at the VT isthmus and demonstrated their role in VT initiation with a critical degree of decrement. CONCLUSIONS: In this preliminary study, DEEP mapping was more specific than LP mapping for identifying the critical targets of VT ablation. The mechanism of DEEPs relates to conduction velocity restitution magnified by zigzag conduction within scar channels.

Is There Still a Role for Complex Fractionated Atrial Electrogram Ablation in Addition to Pulmonary Vein Isolation in Patients With Paroxysmal and Persistent Atrial Fibrillation? Meta-Analysis of 1415 Patients.

BACKGROUND: Ablation of complex fractionated atrial electrograms (CFAEs) has been proposed as a strategy to improve outcomes in atrial fibrillation (AF) catheter ablation, but the use of this technique remains contentious. We aimed to assess the impact of CFAE ablation in addition to pulmonary vein isolation (PVI) in patients undergoing ablation for AF. METHODS AND RESULTS: We performed a random effects meta-analysis of studies comparing PVI versus PVI+CFAE ablation. The outcomes of freedom from AF/atrial tachycardia after 1 or several ablation procedures and acute procedural-related complications were assessed. Studies were searched on MEDLINE, EMBASE, COCHRANE, and clinicaltrials.gov, and sensitivity analyses were performed. Thirteen studies including a total of 1415 patients were considered eligible. Additional ablation of CFAEs resulted in no improvement in mid-term procedural outcome or freedom from AF or atrial tachycardia (odds ratio [OR], 0.80; 95% confidence interval [CI], 0.58-1.10; P=0.17). Sensitivity analysis of 398 paroxysmal AF ablation procedures showed no incremental benefit of CFAE ablation (OR, 0.80; 95% CI, 0.46-1.38; P=0.42). PVI+CFAE ablation versus PVI alone did not improve the overall rate of freedom from AF or atrial tachycardia in patients with persistent AF (OR, 1.01; 95% CI, 0.63-1.64; P=0.96) or longstanding persistent AF (OR, 0.84; 95% CI, 0.24-2.96; P=0.79). There was no increase in procedural-related adverse events (OR, 1.06; 95% CI, 0.41-2.75; P=0.91). CONCLUSIONS: Despite the apparent safety of this technique, CFAE ablation did not improve freedom from AF/atrial tachycardia in patients with paroxysmal or persistent AF. The role of CFAE ablation in addition to PVI should be questioned and other alternatives assessed to improve the outcome of AF ablation.

A study of implanted cardiac rhythm recorders in advanced cardiac AL amyloidosis.

AIMS: AL amyloidosis may respond to chemotherapy but two-thirds of patients with severe cardiac involvement die within a year of diagnosis, purportedly from tachyarrhythmias or electromechanical dissociation. We sought to characterize the nature of cardiac arrhythmias in severe cardiac AL amyloidosis using implanted cardiac rhythm recorders. METHODS AND RESULTS: Implantable loop recorders (ILRs) were inserted within 24 h of baseline evaluation at the UK National Amyloidosis Centre, into 20 consecutive patients with newly diagnosed severe cardiac AL amyloidosis and symptoms of syncope or pre-syncope. Weekly ILR recordings and additional recordings at the time of symptoms were obtained. Median (range) follow-up from baseline was 308 (10-399) days. Thirteen patients died, and median survival in the whole cohort was 61 days from device insertion. In each of eight evaluable cases, death was heralded by bradycardia, usually associated with complete atrioventricular block (CAVB), followed shortly thereafter by pulseless electrical activity. Four patients received pacemakers, a median (range) of 7 (3-38) h after development of symptomatic CAVB, but these did not prevent rapid cardiac decompensation and death in three cases. Despite 272 loop recordings, there was only one episode of non-sustained ventricular tachycardia, which was preceded by severe bradycardia. Patients who died had significantly worse global left ventricular strain on echocardiography (P = 0.029) and reduced 6 min walk distance (P = 0.048) at baseline compared with survivors. CONCLUSIONS: The discovery that bradyarrhythmias heralded terminal cardiac decompensation in most patients with severe cardiac AL amyloidosis supports a study of prophylactic pacemaker insertion in this patient population.