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Cardiac amyloidosis (CA) is an increasingly recognized cause of heart failure, characterized by extracellular deposition of insoluble protein fibrils leading to progressive myocardial dysfunction. The most common types of cardiac amyloidosis are immunoglobin light-chain (AL) and transthyretin (ATTR). Conduction abnormalities are commonly encountered among patients with cardiac amyloidosis and are an important cause of morbidity and mortality. Abnormalities range from infra-Hisian intraventricular conduction delay and bundle branch block to complete atrioventricular block. Pacemaker placement in CA patients follows established guidelines, similar to those for patients without CA, with generally good efficacy. The role and appropriate timing of pacemakers for primary prevention of brady-arrhythmias in CA remains uncertain. While biventricular (BiV) pacing has been shown to improve clinical outcomes in patients with systolic heart failure without CA, there are few data examining the utility of BiV pacing in patients with CA. With the advent of effective treatments for AL and ATTR, appropriate application of pacing is important to support patients with CA and conduction disease through therapeutic trials. This systematic review summarizes the current literature examining the utility of pacing in CA. This article is protected by copyright. All rights reserved.

We report a case report of a 68-year-old man with chemotherapy-induced cardiomyopathy and uncontrolled permanent atrial fibrillation. Cardiac resynchronization therapy implantation and atrioventricular junction ablation were planned. DF-1 single chamber defibrillator was connected to lead's defibrillation and a lead destinated to left bundle branch area pacing. This system leads to reduce costs by one-third, improve battery longevity, and provide a more physiological pacing.

INTRODUCTION Pacing-induced cardiomyopathy (PICM) is a potential complication of chronic right ventricular (RV) pacing, but its characterization in adult patients is often complicated by pre-existing cardiomyopathy. This study investigated the incidence of PICM in patients with congenital heart block (cHB) who have conduction disease from birth without confounding pre-existing cardiac conditions. METHODS AND RESULTS This retrospective cohort analysis included 42 patients with cHB and baseline left ventricular ejection fraction (LVEF) ≥50%. Kaplan-Meier analysis was used to assess freedom from cardiomyopathy (defined as LVEF

A 26-year-old patient with prior surgery for Ebstein's anomaly and a pacemaker (placed for post-surgical heart block and poor underlying rhythm) underwent SICD was placement. During defibrillation testing, device-device interaction led to undersensing of ventricular fibrillation with failure to shock. Increasing the pacemaker sensitivity resolved the problem but post shock pacing was unable to capture the heart after both shocks. The patient underwent removal of both the pacemaker and the SICD and placement of a transvenous ICD. Complex device-device interactions can occur in patients who are pacemaker dependent and undergo placement of a SICD.

Giant cell myocarditis (GCM) is a rare condition. Its association with SARS‐CoV‐2 has not been described before. The 46‐year‐old female patient was admitted to the clinic on September 2020. She had 7 year adrenal insufficiency history and infarct‐like debut of myocardial disease in November 2019. After COVID‐19 in April 2020, cardiac disease progressed. The examination showed low QRS voltage, QS complexes in V1–V5 leads, atrial standstill, left ventricular systolic and restrictive dysfunction, elevated anti‐heart antibodies, and subepicardial late gadolinium enhancement by magnetic resonance imaging. Endomyocardial biopsy and pacemaker implantation were performed, but the patient died suddenly due to ventricular tachycardia or ventricular fibrillation (the resuscitation was ineffective). The autopsy revealed GCM, SARS‐CoV‐2, and Parvovirus B19 were detected in the myocardium. The role of SARS‐CoV‐2 in the pathogenesis of autoimmune myocarditis is discussed.

A 65 year-old-gentleman underwent dual chamber pacemaker implantation (DDDR, St Jude Medical) 7 years back for infra-hisian complete heart block. He was completely asymptomatic and came for his annual routine check-up. After undergoing ECG with and without magnet, he was prepared for device evaluation. After placing the programmer wand over the chest as soon as the 'interrogate' button on the programmer screen was pressed, the patient immediately experienced pre-syncope but recovered instantly as the wand was promptly withdrawn. After taking him to the casualty room with all resuscitation measures in hand, a repeat attempt of interrogation was made after connecting ECG, which revealed reproducible loss of capture (LOC), exclusively during wand placement. A differential diagnosis of lead failure, battery depletion or wand related issues were considered. However, serial ECGs recorded without wand raised the possibility of AutoCapture malfunction. With all precautions, the device was programmed to fixed ventricular output mode after which interrogation could be performed safely. There was a remaining battery longevity of 2 years with acceptable lead parameters and stable threshold. He continues to be asymptomatic at 10 months of follow up. This article is protected by copyright. All rights reserved.

We report on an unusual case with asymptomatic bilateral external iliac vein non-thrombotic obstruction causing difficulty in delivery of the leadless permanent pacemaker and discuss on the strategies to overcome the problem.

We report isolated extramedullary relapse in a 14-year-old boy, sequentially presenting with intestinal and cardiac myeloid sarcoma (MS). Acute myeloblastic leukemia M5 was diagnosed 41 months ago. On the 14th month of the second HSCT, he presented with ileus and underwent surgical treatment. After 2 weeks, arrhythmia, bradycardia, complete heart block, and atrial flutter developed and echocardiography revealed multiple cardiac masses. There was no bone marrow relapse but pathology of the intestinal biopsy showed leukemic infiltration. Patient was successfully treated with a permanent pacemaker and salvage chemotherapy. To the best of our knowledge, this is the first pediatric cardiac MS developed after HSCT.

Background Exercise capacity is an important aspect of quality of life in patients undergoing pacemaker implantation. Device algorithms for ventricular pacing avoidance have been developed to avoid unnecessary and potentially harmful effects of right ventricular pacing. However, little data exists on the immediate response of these algorithms to sudden AV block during exercise.Methods The ventricular pacing avoidance algorithms of four pacemaker manufacturers were tested in an ex-vivo model. The RSIM-1500-USB Device-Interactive Heart Simulator (Rivertek Medical Systems, Inc.) was used to simulate three different scenarios: the first one starting with an initially conducted atrial pacing rate of 60 min(-1), the second one starting with an atrial rate of 120 min(-1) and finally a scenario starting with an atrial rate of 150 min(-1). In all three scenarios, the initially conducted atrial rate was followed by a sudden, long lasting episode of third-degree AV-block. The response to those scenarios was recorded for each of the (brand-specific) ventricular pacing avoidance algorithms.Results In the first scenario, the simulation resulted in a ventricular pause of 1333 ms (Boston Scientific), 2000 ms (Medtronic and Microport), and 2340 ms (Biotronik). In the second and third scenario, different results were observed across devices. All simulations of the second and third scenario resulted in repetitive 2:1 block response (during eight cycles) in Boston Scientific and Biotronik devices. These scenarios were confirmed in patient cases.Conclusion Simulator based observations of unanticipated pacemaker-induced 2:1 block response during exercise may explain clinical symptoms experienced by some patients having a two-chamber pacemaker.

Background Temporary cardiac pacing, conventionally achieved using a passive transvenous pacing wire, can be life-saving for unstable arrhythmias. However, they run the risk of complications, the longer they remain in-situ. Externalized prolonged temporary pacing (EPTP), using active-fixation lead and an externalized pulse generator; may be an alternative for transient pacing indications, concurrent illness or sepsis that precludes permanent pacing. Methods Sixty-seven patients (mean age 69 ± 14 years; 82% male) underwent EPTP between November 2011 and April 2019. EPTP was performed in a sterile facility, under fluoroscopy, using active-fixation leads anchored to the right ventricle septum. Externalized lead was connected to a re-sterilized pulse generator and secured to anterior chest wall with transparent dressings. EPTP indications and patient outcomes were evaluated. Results Pacing indications were high-grade atrio-ventricular (AV) block (73.2%), sinus arrest (14.9%), overdrive suppression of VT (5.9%) and pause-dependent VT (4.5%). Reasons for ETPT rather than permanent pacing included: sepsis (38.8%), CIED-related infection (8.9%), transient pacing indication (25%), to allow further investigations prior to decision on CIED type (22%), and over-drive arrhythmia suppression (6%). Sixty three percent patients were severely ill in an ICU. Mean duration of pacing was 16 ± 12 days. Sixty seven percent patients subsequently received a CIED and had no evidence of device-related infection at 1-year post-implant. There were three non-fatal complications during EPTP while no deaths were attributed to EPTP. Conclusion EPTP is a safe and useful method of prolonged temporary pacing for patients who require chronotropic support, but in whom immediate permanent pacemaker implantation is contraindicated.

INTRODUCTION It has not been clarified how to identify the electrophysiological junction between right atrium (RA) and superior vena cava (SVC). The aim of this study was to identify the electrophysiological RA-SVC junction according to slow conduction or conduction bock and to examine the electrophysiological SVC isolation procedure. METHODS Seventy-three consecutive atrial fibrillation patients who underwent SVC mapping using a CARTO 3 system were enrolled in this study. Slow conduction or conduction block between the RA and SVC was identified by adjusting the lower threshold criteria of the early meets late function and was described as a white line. The SVC isolation was performed along the white line and with pacing maneuvers to confirm direct SVC capture. RESULTS Activation mapping (1296 ± 631 points) was obtained in 66 patients (90%) in 4.6 ± 1.8 min. Slow conduction or conduction block was observed in all patients. The threshold for detecting slow conduction or conduction block was 24 ± 8 ms. The location of the electrophysiological RA-SVC junction was higher in the anterior portion (anterior-septal, anterior, and anterior-lateral) than in the posterior portion (posterior-septal, posterior, and posterior-lateral) (-2.3 ± 6.2 mm vs. 7.1 ± 6.3 mm, p

Transcatheter aortic valve replacement (TAVR) is a rapidly growing procedure. Conduction disease post-TAVR is frequent and routinely monitored for periprocedurally. Permanent pacemaker placement is relatively common and usually associated with worse outcomes post-TAVR. We report a case of very late presenting complete heart block post-TAVR treated with His-bundle pacing. Our case underscores the need for larger studies to further evaluate the utility of long-term cardiac monitoring post-TAVR and outcomes of His-bundle pacing in this population.

We present the case of a worker with occupational exposure to a pyrethroid insecticide who acutely developed nontransient third-degree heart block. In 2000, a 57-year-old male truck driver on his delivery route was accidentally exposed to pyrethroid insecticide being sprayed for West Nile virus containment. Both the driver and his vehicle were coated with the spray. The exposure was prolonged because he did not change his clothes until after his shift ended and he used the same contaminated truck for a week. Within days, he presented with a third-degree heart block, for which he was emergently treated, and a pacemaker was placed. He had no past history of arrhythmias. In the weeks thereafter, he also developed reactive airway dysfunction syndrome (RADS). In the second decade following the exposure, the patient replaced his pacemaker, confirming the permanent nature of his heart block. In addition to the persistence of his exposure-related RADS, he developed restrictive lung disease and was diagnosed with pulmonary interstitial fibrosis in the absence of established risk factors. The patient died in October 2019 from respiratory illness. Most previous reports of pyrethroid-related disorders are limited to acute exposures, in which transient symptoms predominate. To our knowledge, this is the first report of an exposed worker experiencing permanent third-degree heart block, as well as persistent respiratory findings, as possible short- and long-term sequelae of pyrethroid exposure.

Desmin‐related myopathy (DRM) is a rare heritable cardiac and skeletal muscle disease caused by mutations in the desmin gene (DES). DRM is generally characterized by skeletal muscle weakness, conduction disturbance, and dilated cardiomyopathy. However, the clinical cardiac phenotypes of DRM are not yet fully understood. Herein, we report the first case of DRM with the de novo missense DES mutation, R454W, that is characterized by left ventricular non‐compaction cardiomyopathy, progressive cardiac conduction defect, spontaneous coronary artery dissection, and no skeletal muscle weakness. Our case findings suggest that clinicians should genetically test patients who have cardiomyopathy, progressive cardiac conduction defect, and coronary artery dissection, even if the patient has neither family history of DRM nor skeletal muscle symptoms.

Background Although radiofrequency ablation is widely used in the treatment of arrhythmias, its role in septal reduction therapy of hypertrophic obstructive cardiomyopathy (HOCM) is unclear. This meta‐analysis aimed to assess the efficacy and safety of radiofrequency septal ablation for HOCM. Hypothesis Radiofrequency septal ablation is effective and safe for relieving obstruction and improving exercise capacity in patients with HOCM. Methods A systematic review of eligible studies that reported outcomes of patients with HOCM who underwent radiofrequency septal ablation was performed using PubMed, Embase, Cochrane, ProQuest, Scopus, ScienceDirect, and Web of Science database. Pooled estimates were calculated using random‐effects meta‐analysis. Methodological quality was assessed using the Newcastle‐Ottawa scale. Publication bias and sensitivity analyses were also performed. Results Eight studies with 91 patients (mean follow‐up 11.6 months) were included. The left ventricular outflow tract (LVOT) gradient at rest decreased significantly after radiofrequency septal ablation (pooled reduction: −58.8 mmHg; 95% confidence interval [CI] −64.3 to −53.5). A reduction was also found in the provoked LVOT gradient with a pooled reduction of −97.6 mmHg (95% CI: −124.4 to −87.1). An improvement of the New York Heart Association classification (mean: −1.4; 95% CI: −1.6 to −1.2) was found during follow‐up. The change in septal thickness was minimal and not statistically significant. Two procedure‐related deaths were documented, and complete heart block occurred in eight patients. Conclusions Radiofrequency septal ablation is effective and safe for relieving LVOT obstruction and improving exercise capacity in patients with HOCM.

A 30-year-old female patient with 12 weeks of gestation was admitted to our hospital due to dizziness and amaurosis fugax. Moreover, 24 h Holter monitoring showed paroxysmal atrioventricular block (P-AVB) and ventricular arrest. The heart block in the patient was likely a vagally mediated heart block based on the "vagal score." She was not given a pacemaker, and the symptoms and AV nodal conduction were improved following the delivery.

The clinical presentation of cardiac sarcoidosis is variable. We report two cases of cardiac sarcoidosis to highlight the varied clinical presentations and diagnostic challenges in our setting, and encourage the consideration of sarcoidosis as a differential in unexplained arrhythmias and heart failure., Cardiac sarcoidosis is a major cause of death and disability in sarcoid patients. It can be benign and asymptomatic or life‐threatening. Early diagnosis is lifesaving since prompt therapy can lead to the resolution of cardiac dysfunction.

A 59-year-old female underwent a dual-chamber pacemaker implantation for intermittent complete heart block. A baseline electrocardiogram showed normal sinus rhythm with first-degree atrioventricular (AV) block and right bundle branch block. A His bundle lead placement was attempted. An intracardiac electrogram from the His bundle lead demonstrated atrial-His, and His-ventricular intervals were 186 and 110 ms, respectively. Pacing was performed from the His bundle lead with a decremental pacing output to assess for the His bundle capture threshold. However, there were no significant QRS morphology changes during the pacing. Is the His bundle captured? The tracing evaluation demonstrated the fascinating physiology of activation wavefront in His Purkinje system that could be applied in the use of conducting system pacing technologies.

There is limited known safety and efficacy of leadless pacemaker device use in patients with durable left ventricular assist devices (LVADs). We present a case of a pacemaker-dependent LVAD patient with infection of permanent transvenous pacemaker who underwent successful implantation of Micra transcatheter pacing system (Medtronic).

This case concerns a 24‐year‐old female who developed malignant ventricular tachyarrhythmia a few weeks after pacemaker implantation for complete heart block. Apparently, right ventricular pacing caused significant repolarization abnormalities in both native and paced rhythms with marked QT prolongation and substantial electrical instability. This case highlights other intriguing phenomena in the puzzle of cardiac repolarization and how pacing therapy may alter this complex process providing arrhythmic substrate in vulnerable subjects. Though such arrhythmic events are clinically rare, vulnerable patients or with suspected myocardial disease that may cause QT prolongation should be carefully followed in the course of pacing therapy.

BACKGROUND Conduction disturbances are the most frequent complication of transcatheter aortic valve replacement (TAVR). However, no data exists regarding the outcomes of intraprocedural high-degree atrioventricular block (HAVB) or complete heart block (CHB) in patients without previous conduction disturbances. OBJECTIVES The aim of this study was to evaluate the outcomes of intraprocedural-HAVB/CHB in patients without previous intraventricular conduction disturbances. METHODS The occurrence of intraprocedural-HAVB/CHB was assessed in 676 consecutive patients undergoing TAVR, and two groups were established according to its duration: persistent-HAVB/CHB (PHAVB/CHB) and transient-HAVB/CHB (THAVB/CHB), not present at the end of the procedure. RESULTS Intraprocedural-HAVB/CHB occurred in 50 patients (7.4%), being persistent in 32 (64.0%), and transient in 18 (36.0%). The use of Medtronic Corevalve Revalving System (MCRS) and a greater oversizing of the valve increased the risk of intraprocedural-HAVB/CHB (p

Background Heart block requiring a pacemaker is common after self-expandable transcatheter aortic valve replacement (SE-TAVR); however, conduction abnormalities may improve over time. Optimal device management in these patients is unknown. Objective To evaluate the long-term, natural history of conduction disturbances in patients undergoing pacemaker implantation following SE-TAVR. Methods All patients who underwent new cardiac implantable electronic device (CIED) implantation at Michigan Medicine following SE-TAVR placement between January 1, 2012 and September 25, 2017 were identified. Electrocardiogram and device interrogation data were examined during follow-up to identify patients with recovery of conduction. Logistic regression analysis was used to compare clinical and procedural variables to predict conduction recovery. Results Following SE-TAVR, 17.5% of patients underwent device placement for new atrioventricular (AV) block. Among 40 patients with an average follow-up time of 17.1 ± 8.1 months, 20 (50%) patients had durable recovery of AV conduction. Among 20 patients without long-term recovery, four (20%) had transient recovery. The time to transient conduction recovery was 2.2 ± 0.2 months with repeat loss of conduction at 8.2 ± 0.9 months. On multivariate analysis, larger aortic annular size (odds ratio: 0.53 [0.28-0.86]/mm, P = 0.02) predicted lack of conduction recovery. Conclusions Half of the patients undergoing CIED placement for heart block following SE-TAVR recovered AV conduction within several months and maintained this over an extended follow-up period. Some patients demonstrated transient recovery of conduction before recurrence of conduction loss. Larger aortic annulus diameter was negatively associated with conduction recovery.

The need for creation or enlargement of a ventricular septal defect (VSD) is a rare occurrence. It is most frequently required in patients with double-outlet right ventricle who develop restriction of a remote muscular VSD or obstruction of a perimembranous VSD secondary to atrioventricular (AV) valve attachments to the interventricular septum. Surgical and transcatheter options for VSD creation or enlargement are associated with several risks including heart block, AV valve injury, and perforation. We report the first description of a hybrid approach to VSD creation and enlargement in two patients.

An infant with hypoplastic left heart syndrome (HLHS) presented with complete heart block and severe myocardial dysfunction requiring ECMO support due to complete left main coronary artery (LMCA) thrombosis. Current guidelines for managing coronary artery thrombosis in infants with single ventricle physiology are inadequate. We describe successful LMCA and branch recanalization via intra coronary infusion of recombinant tissue plasminogen activator and discuss management of acute coronary thrombosis in children with single ventricle physiology.

BACKGROUND Transcatheter aortic valve replacement (TAVR) can be an effective option for high-risk Aortic Regurgitation (AR) patients. Although international experiences of TAVR for AR are published, U.S. data are limited. This study sought to report the short-term outcomes of TAVR in AR in the U.S. POPULATION METHODS Study cohorts were derived from the Nationwide Inpatient Sample (NIS) and Nationwide Readmissions Database (NRD) 2016-17. TAVR and AR were identified using ICD-10-CM-codes. The key outcomes were all-cause mortality, disabling stroke, valvular complications, complete heart block (CHB)/permanent pacemaker placement (PPM), open-heart surgery, acute kidney injury (AKI) requiring dialysis, and vascular complications. Multivariate logistic regression was used to adjust for confounders. RESULTS 915 patients from the NIS (male-71%, age ≥65-84.2%) and 822 patients from the NRD (male-69.3%, age ≥65-80.5%) underwent TAVR for AR. The median length of stay (LOS) was 4 days for both cohorts. In-hospital mortality was 2.7%, and 30-day mortality was 3.3%. Disabling strokes were noted in 0.6% peri-procedurally and 1.8% at 30-days. Valve-related complications were 18-19% with paravalvular leak (4-7%) being the most common. Approximately 11% of patients developed CHB and/or needed PPM in both cohorts. In NRD, 2.2% of patients required dialysis for AKI, 1.5% developed vascular complications, and 0.6% required open-heart surgery within 30-days post-procedure. Anemia was predictive of increased overall complications and valvular complications, whereas peripheral vascular disease was a predictor of increased valvular complications and CHB/PPM. CONCLUSION TAVR is a promising option in AR. Further studies are necessary for the expansion of TAVR as the standard treatment in AR.

BACKGROUND: Despite descriptions of various cardiovascular manifestations in patients with coronavirus disease 2019 (COVID-19), there is a paucity of reports of new onset bradyarrhythmias, and the clinical implications of these events are unknown. METHODS: Seven patients presented with or developed severe bradyarrhythmias requiring pacing support during the course of their COVID-19 illness over a 6-week period of peak COVID-19 incidence. A retrospective review of their presentations and clinical course was performed. RESULTS: Symptomatic high-degree heart block was present on initial presentation in three of seven patients (43%), and four patients developed sinus arrest or paroxysmal high-degree atrioventricular block. No patients in this series demonstrated left ventricular systolic dysfunction or acute cardiac injury, whereas all patients had elevated inflammatory markers. In some patients, bradyarrhythmias occurred prior to the onset of respiratory symptoms. Death from complications of COVID-19 infection occurred in 57% (4/7) patients during the initial hospitalization and in 71% (5/7) patients within 3 months of presentation. CONCLUSIONS: Despite management of bradycardia with temporary (3/7) or permanent leadless pacemakers (4/7), there was a high rate of short-term morbidity and death due to complications of COVID-19. The association between new-onset bradyarrhythmias and poor outcomes may influence management strategies for acutely ill patients with COVID-19.

Background The significance of endo‐epicardial asynchrony (EEA) and atrial conduction block (CB), which play an important role in the pathophysiology of atrial fibrillation (AF) during sinus rhythm is poorly understood. The aim of our study was therefore to examine 3‐dimensional activation of the human right atrium (RA). Methods and Results Eighty patients (79% men, 39% history of AF) underwent simultaneous endo‐epicardial sinus rhythm mapping of the inferior, middle and superior RA. Areas of CB were defined as conduction delays of ≥12 ms, EEA as activation time differences of opposite electrodes of ≥15 ms and transmural CB as CB at similar endo‐epicardial sites. CB was more pronounced at the endocardium (all locations P P =0.03). EEA occurred up to 84 ms and was more pronounced at the superior RA (superior: 27 ms [interquartile range, 18.3–39.3], versus mid‐RA: 20.3 ms [interquartile range, 0–29.9], and inferior RA: 0 ms [interquartile range, 0–21], P P =0.009), diabetes mellitus ( P =0.018), and hypercholesterolemia ( P =0.015) were associated with a higher degree of EEA. CB ( P =0.007) and EEA ( P =0.037) were more pronounced in patients with a history of persistent AF compared with patients without AF history. Conclusions This study provides important insights into complex atrial endo‐epicardial excitation. Significant differences in conduction disorders between the endo‐ and epicardium and a significant degree of EEA are already present during sinus rhythm and are more pronounced in patients with cardiovascular risk factors or a history of persistent AF.

Aims Cardiac sarcoidosis (CS) often presents with ventricular arrhythmias, heart block, and cardiomyopathy. The prognosis of CS with contemporary management is uncertain. We estimated the impact of baseline and treatment variables on left ventricular ejection fraction (LVEF), ventricular assist device placement, heart transplant, and death. Methods and results We identified patients with CS seen from 1994-2014 at two large academic medical centres. All met the 2014 Heart Rhythm Society expert consensus criteria for diagnosis. From the 574 patients identified, 91 met inclusion criteria. Twenty-two (24.2%) were diagnosed by endomyocardial biopsy. Cardiomyopathy was the primary presentation in 47 patients (51.6%). Within 90 days of diagnosis, 41 patients (45.0%) received prednisone alone, 29 (31.9%) received alternative immunosuppression with or without prednisone, and 21 (23.1%) received no immunosuppression. During follow-up, 31 of 47 cardiomyopathy patients experienced improvement in LVEF, while 23 experienced decline in LVEF or clinical exacerbation, and 15 of 22 patients presenting with ventricular arrhythmia had recurrence. These results did not differ by treatment group. During a median follow-up of 44 months for our cohort, 14 patients reached the composite endpoint of ventricular assist device placement, heart transplant, or death. Survival without the composite outcome did not differ by treatment group, but was worse among patients presenting with cardiomyopathy (log-rank = 0.005). Conclusion In a large series of CS subjects, rates of ventricular arrhythmia and heart failure events remain high with no treatment regimen clearly associated with better outcome. Patients with cardiomyopathy at diagnosis were more likely to reach the composite endpoint.

The interventricular septal dissection is an uncommon, and is occasionally associated with sinus of Valsalva aneurysm. A spontaneous dissection with a normal sinus of Valsalva is extremely rare and is mostly a result of a congenital defect. We report the case of a 23-year-old male admitted for complete heart block and presenting as an incidental finding during the TTE multiple and huge cystic-like mass in the interventricular septum (IVS) which is the TTE characteristic of IVS dissection. Most patients remain asymptomatic until the anatomical and hemodynamic changes lead to complications like: severe aortic regurgitation with heart failure and conduction abnormalities. Although transthoracic echocardiogram (TTE) has been the first-line imaging modality for such findings, cardiac computed tomography (CT) and magnetic resonance imaging have been increasingly used as supplemental or confirmatory tests. The imaging modalities showed a perforation of right sinus of Valsalva causing the expansion of the dissection to the apex. The patient underwent surgical repair with satisfying results. In conclusion, we report an extremely rare clinical case of spontaneous dissection of the interventricular septum using multiple imaging techniques and with a successful surgery.

Item does not contain fulltext OBJECTIVE: We sought to evaluate the feasibility, technical aspects, and outcome of transcatheter perimembranous ventricular septal defect (pmVSD) closure using duct occluder devices with a single retention disc. BACKGROUND: Use of duct occluder devices to close pmVSD seems a promising alternative therapy. However, limited data exist on this technique. METHODS: From 2010 to 2016, 222 patients (female 47.7%) were identified from databases of five participating institutions in whom pmVSD closure was attempted using an Amplatzer Duct Occluder I or Lifetech duct occluder device. RESULTS: Patients ranged in age from 0.7 to 52 years (median, 7.0 years) and in weight from 4.0 to 70 kg (median, 18.0 kg). The mean size of the VSD was 6.8 +/- 2.2 mm. A large defect (> 6 mm) was present in 137 patients (61.7%). Device closure was successful in 218 patients (98.2%). The 10/8 mm device was used in most patients (n = 85, 38.3%), and the vascular approach was from the femoral vein in 169 patients (76.1%). There were 18 early complications in 17/218 patients (7.8%). Three patients (1.4%) developed complete heart block (transient n = 2; requiring permanent pacing n = 1). Median follow-up was 6 months (6 months-6 years). A mild residual shunt was seen in 10 patients at 6 months follow-up. CONCLUSIONS: The immediate results of transcatheter pmVSD closure using a duct occluder device with a single retention disc are promising. It is an effective technique with a lower rate of complications than for other currently available devices.

Henoch-Schonlein purpura (HSP) is a rare, typically self-limited, multi-organ vasculitis. Cardiac involvement with HSP carries high morbidity and mortality, thus requiring early aggressive immunosuppressive therapy. We report a case of HSP complicated with acute systolic left ventricular (LV) dysfunction, symptomatic sinus bradycardia and high-grade atrio-ventricular (AV) heart block. Cyclophosphamide, a commonly used agent in HSP, was contraindicated due to the patient's presentation with acute renal failure. Treatment with monoclonal antibody rituximab and corticosteroids was initiated with an improvement in and resolution of LV systolic dysfunction, sinus bradycardia and AV block. We believe this is the first published report on rituximab treatment in HSP with cardiac involvement manifesting with severe LV systolic dysfunction, sinus bradycardia and high-grade AV block.

OBJECTIVE To establish nomograms for fetal atrioventricular (AV) time intervals assessed by 3 different pulsed-wave Doppler techniques: left ventricular inflow and outflow tracts (LV in/out), superior vena cava and ascending aorta (SVC/AA), and pulmonary artery and pulmonary vein (PA/PV). METHODS A cross-sectional study was performed in 311 normal fetuses divided into 5 groups between 16 and 38 weeks. Pulsed-wave Doppler-derived AV intervals were measured by interrogation of flow in LV in/out, SVC/AA, and PA/PV. Linear regression analyses were performed to examine correlations with gestational age (GA) and fetal heart rate (FHR). Intraclass correlation coefficients for reproducibility of each method were compared. RESULTS Pulmonary artery and pulmonary vein revealed the longest mean AV time intervals (P

The accuracy of photoplethysmography (PPG) for heart rate (HR) estimation in cardiac arrhythmia is unknown. PPG-HR was evaluated in 112 hospitalised inpatients (cardiac arrhythmias (n = 60), sinus rhythm (n = 52)) using a continuous electrocardiogram monitoring as a reference standard. Strong agreement was observed in sinus rhythm HR < 100 and atrial flutter (bias 1 beat), modest agreement in sinus tachycardia (bias 24 beats) and complete heart block (bias -6 beats) and weak agreement with significant HR underestimation was seen in atrial fibrillation (bias 23 beats). Routine utilisation of PPG for HR estimation may delay early recognition of clinical deterioration in certain arrhythmias and sinus tachycardia.