Search

The most common cause of congenital heart block (CHB) is neonatal lupus, an acquired autoimmune disease caused by transplacental transfer of maternal antibodies to the fetus. A full-term female neonate was admitted to neonatal intensive care unit for severe bradycardia with stable haemodynamics. The mother, showing no clinical symptoms or any particular history, was transferred to our tertiary centre for profound fetal bradycardia. At birth, the infant’s ECG showed a third-degree atrioventricular block and echocardiography was normal. Cardiac neonatal lupus was confirmed with positive maternal anti-Ro antibodies. Under close monitoring, the infant tolerated the bradycardia well (median 67 beats per minute (bpm)) and was discharged on day 6 of life. There was no indication for pacemaker, but she would be on regular follow-up with a paediatric cardiologist. This article holds an important insight as it is the first confirmed case of autoimmune CHB in Cambodia in which the mother’s antibody was found only after diagnosis on the neonate.

Performance of the leadless pacemaker capable of atrioventricular (AV) synchronous pacing in de novo patients warrants further investigation.The aims of this study were to assess what programming changes are needed to achieve proper atrial tracking and to study the percentage of AV synchrony (AVS) the device can provide under real-world conditions.Consecutive patients undergoing Micra AV implantation between June 2020 and November 2021 were studied. Reprogramming of atrial sensing parameters during follow-up was performed by following device counters. AVS was studied with an ambulatory 24-hour Holter monitor and automatically analyzed by an electrocardiogram delineation system. The primary end point was AVS ≥85% of total cardiac cycles during 24-hour Holter electrocardiogram monitoring.Thirty-one patients who remained in VDD mode were studied, and all of them required manual reprogramming. The automatic A3 window end was deactivated, and a fixed and short value was set in all patients throughout follow-up. AVS significantly increased from 68.7% ± 14.7% at 24-hour follow-up to 83.9% ± 7.4% at 1-month visit (P = .001). At 1-month visit, shorter A3 window end time (P = .019), higher A4 threshold (P = .011), and deactivation of the automatic A3 window (P = .054) were independently related to higher AVS. A total of 2,291,953 Holter-recorded cardiac cycles were analyzed. Median AVS during 24-hour daily activities was 87.6% (interquartile range 84.5%-90.6%). Twenty of 26 patients (79.6%) reached AVS ≥85% of cardiac cycles.High rates of AVS can be achieved in real-world patients undergoing leadless pacing. Manual reprogramming of the atrial sensing parameters is essential to optimize mechanically sensed atrial tracking.

Left ventricular outflow tract obstruction is common among symptomatic patients with hypertrophic cardiomyopathy, yet septal reduction by surgical myectomy (septal myectomy [SM]) is performed infrequently in many centers. This study examined the possible relationship between institutional case volume and early outcomes of SM.The Society of Thoracic Surgeons Adult Cardiac Surgery Database was queried for patients with hypertrophic cardiomyopathy who underwent SM from January 2012 to December 2019. The study defined center case volume categories as1, 1 to 4.99, 5 to 9.99, and ≥10 cases performed on average per year.The study population included 5935 patients at 481 centers with 933 surgeons. The range of average center volume was1 to 138 cases per year. Overall early mortality was 2.6%, ventricular septal defect (VSD) occurred in 1.9%, and complete heart block occurred in 9.0%. Concomitant mitral valve (MV) repair was performed in 28.7%, and MV replacement was performed in 17.1%. In multivariable analysis, the lowest annual case volume (average1 case/y) was consistently associated with greater early mortality (odds ratio [OR], 5.4; CI, 3.0-9.9; P.001), greater risk of VSD (OR, 9.3; CI ,4.2-20.4; P.001), increased incidence of complete heart block (OR, 2.0; CI, 1.5-2.7; P.001), and a higher likelihood of MV replacement (OR, 9.4; CI, 7.5-11.8; P.001).Volume of SM cases varies widely among institutions reporting to the Society of Thoracic Surgeons Adult Cardiac Surgery Database. There appears to be an important association between surgical experience, as reflected by institutional case volume, and early outcomes, including mortality, as well as the occurrence of VSD, heart block, and MV replacement.

Background: PoAF is the most common complication after cardiac surgery and may occur in patients with pre-existing arrhythmogenic substrate. Characterization of this substrate could aid in identifying patients at risk for PoAF. We therefore compared intra-atrial conduction parameters and electrogram morphology between patients without and with early- (≤5 days after surgery) and late- (up to 5 years) postoperative atrial fibrillation (PoAF). Methods and results: Epicardial mapping of the right and left atrium and Bachmann's Bundle (BB) was performed during sinus rhythm (SR) in 263 patients (207male, 67 ± 11 years). Unipolar potentials were classified as single, short or long double and fractionated potentials. Unipolar voltage, fractionation delay (time difference between the first and last deflection), conduction velocity (CV) and conduction block (CB) prevalence were measured. Comparing patients without (N = 166) and with PoAF (N = 97), PoAF was associated with lower CV and more CB at BB. Unipolar voltages were lower and more low-voltage areas were found at the left and right atrium and BB in PoAF patients. These differences were more pronounced in patients with late-PoAF (6%), which could even occur up to 5 years after surgery. Although several electrophysiological parameters were related to PoAF, age was the only independent predictor. Conclusions: Patients with de novo PoAF have more extensive arrhythmogenic substrate prior to cardiac surgery compared to those who remained in SR, which is even more pronounced in late-PoAF patients. Future studies should evaluate whether intra-operative electrophysiological examination enables identification of patients at risk for developing PoAF and hence (preventive) therapy.

Lyme carditis is an uncommon manifestation of Lyme disease. Most cases present with heart block of varying degrees, but the spectrum of disease includes other transient arrhythmias and structural manifestations, such as myopericarditis or cardiomyopathy. Antibiotics hasten the resolution of Lyme carditis, and cardiac pacing can be an adjunctive therapy. Outcomes are generally good, but there are rare fatalities associated with Lyme carditis. The latter underscores the continued need for improved modes of prevention of Lyme disease and the importance of its early recognition and treatment.

A 36-year-old woman with levo-transposition of the great arteries presented for a programmed visit. She was asymptomatic and the clinical examination showed no signs of decompensated heart failure. Standard 12-lead ECG showed isorhythmic atrioventricular dissociation. A 24-hour ambulatory recording demonstrated sinus rhythm with intermittent periods of isorhythmic dissociation. This case highlights the association between two rare conditions.

A 55-year-old man presented with occasional shortness of breath and palpitations for the last 6 months. Holter monitoring revealed intermittent complete heart block and junctional escape rhythm. Transthoracic echocardiography was unremarkable. Cardiac MR was performed to look for any scar or any

Acute type A aortic dissection (ATAAD) is a life-threatening condition and surgical repair often includes aortic valve replacement (AVR). Aortic valve repair (AVr) is increasingly being reported with favorable outcomes from single-center experiences. This study examined national trends and outcomes of AVr in patients with ATAAD.Adults with a primary diagnosis of acute thoracic aortic dissection who underwent proximal aortic surgery from January 2016 to December 2017 were obtained from the National Inpatient Sample. Patients were stratified into an isolated aortic surgery group (no aortic valve procedure), concomitant AVR, or concomitant AVr groups. The primary outcome was in-hospital mortality and secondary outcomes included stroke, acute kidney injury, heart block, and bleeding. Propensity score matching was used to address patient and hospital-level confounders between AVR and AVr groups.In total, 5115 patients underwent surgery for ATAAD and were included. Overall, 3220 (63%) underwent isolated ATAAD repair, while 1120 (22%) had concomitant AVR, and 775 (15%) had concomitant AVr. In 455 propensity-matched pairs, there was no difference in mortality or stroke between AVr and AVR groups, however, heart block (1.1% vs. 7.5%, p .001) and bleeding (65.9% vs. 81.3%, p .001) were significantly less common among those who underwent AVr. Patients who underwent AVr had shortest LOS (11.9 vs. 13.5 days, p .001). There were no differences in outcomes of AVr in ATAAD based on hospital size or teaching status.In selected patients, AVr is being performed safely in the setting of ATAAD with mortality and composite outcomes comparable to AVR.

Congenital complete heart block (CCHB) is seen in 1:15,000-20,000 births and commonly requires pacemaker placement by young adulthood. There is limited understanding of cardiac morbidity and mortality.The purpose of this study was to determine the long-term incidence of cardiac morbidity and mortality in subjects with CCHB and identify associated risk factors.Retrospective cohort analysis of subjects with CCHB at Children's Hospital of Philadelphia between 1976 and 2018 was performed. The primary outcome was a composite of death, left ventricular systolic dysfunction, heart failure, cardiomyopathy, or cardiac resynchronization therapy (CRT). Cox proportional hazard models assessed independent risk factors for the primary outcome and its components (death, heart failure and/or cardiomyopathy, CRT).One-hundred fourteen subjects (58% female; median age at last visit 15.2 years) were included. Eighty-eight (77%) underwent pacemaker implantation (median age at placement 1.9 years; interquartile range [IQR] 0.1-8.0 years). Twenty-six subjects (23%) reached the primary outcome; 7 (6%) died and 14 (12%) were diagnosed with heart failure and/or cardiomyopathy. Median time from diagnosis to primary outcome was 3.1 years (IQR 0.0-10.8 years). There were no significant associations between age at diagnosis1 year (hazard ratio [HR] 1.5; 95% confidence interval [CI] 0.6-3.9), fetal diagnosis (HR 2.3; 95% CI 0.96-5.6), or maternal antibody positivity (HR 2.4; 95% CI 0.9-6.6) and the primary outcome. Fetal diagnosis had a higher associated hazard of heart failure and/or cardiomyopathy (HR 4.5; 95% CI 1.3-15.0).In 114 subjects with CCHB, 23% reached the composite outcome of cardiac morbidity and mortality, with no significant association between age at diagnosis, fetal diagnosis, and maternal antibody status with composite cardiac morbidity and mortality.

The short and long-term outcomes of children with anti-Ro/La-related congenital heart block treated with a combined maternal-neonatal therapy protocol were compared with those of controls treated with other therapies.Sixteen mothers were treated during pregnancy with a therapy consisting of daily oral fluorinated steroids, weekly plasma exchange and fortnightly intravenous immunoglobulins and their neonates with intravenous immunoglobulins (study group); 19 mothers were treated with fluorinated steroids alone or associated to intravenous immunoglobulins or plasma exchange (control group).The combined-therapy children showed a significantly lower progression rate from 2nd to 3rd degree block at birth, a significant increase in heart rate at birth and a significantly lower number of pacemaker implants during post-natal follow-up with respect to those treated with the other therapies.The combined therapy produced better short and long term outcomes with respect to the other therapies studied.

Immune-checkpoint inhibitor-associated myocarditis (ICI-myocarditis) often presents with arrhythmias, but the prognostic value of early electrocardiogram findings is unclear. Although ICI-myocarditis and acute cellular rejection (ACR) following cardiac transplantation use similar treatment strategies, differences in arrhythmia burden are unknown.To evaluate the association of electrocardiogram findings in ICI-myocarditis with myocarditis-related mortality and life-threatening arrhythmia.A total of 125 cases of ICI-myocarditis were identified retrospectively across 49 hospitals worldwide; 50 cases of grade 2R or 3R ACR were included as comparators. Two cardiologists blinded to clinical data interpreted electrocardiograms. Associations between electrocardiogram features, myocarditis-related mortality and the composite of myocarditis-related mortality and life-threatening arrhythmias were examined. Adjusted hazard ratios (aHRs) were calculated.The cohort had 78 (62.4%) men; median (interquartile range) age was 67 (58-76) years. At 30 days, myocarditis-related mortality was 20/124 (16.1%), and 28/124 (22.6%) met the composite endpoint. Patients who developed complete heart block (aHR by subdistribution hazards model [aHR(sh)] 3.29, 95% confidence interval [CI] 1.24-8.68; P=0.02) or life-threatening cardiac arrhythmias (aHR(sh) 6.82, 95% CI: 2.87-16.21; P0.001) had a higher risk of myocarditis-related mortality. Pathological Q waves (aHR(sh) 3.40, 95% CI: 1.38-8.33; P=0.008), low QRS voltage (aHR(sh) 6.05, 95% CI: 2.10-17.39; P0.001) and Sokolow-Lyon index (aHR(sh)/mV 0.54, 95% CI: 0.30-0.97; P=0.04) on admission electrocardiogram were also associated with increased risk of myocarditis-related mortality. These associations were mirrored in the composite outcome analysis. Compared with ACR, ICI-myocarditis had a higher incidence of life-threatening cardiac arrhythmias (15/125 [12.0%] vs 1/50 [2%]; P=0.04) and third-degree heart block (19/125 [15.2%] vs 0/50 [0%]; P=0.004).Electrocardiograms in ICI-myocarditis with ventricular tachycardias, heart block, low-voltage and pathological Q waves were associated with myocarditis-related mortality and life-threating arrhythmia. Arrhythmia burden in ICI-myocarditis exceeds that of ACR after heart transplant.

Objective The leadless cardiac pacemaker is typically implanted percutaneously and has been widely used for patients who have already undergone valve surgery. We sought to determine the feasibility and safety of implanting the leadless pacemaker under direct visualization during valve surgery. Methods We performed a retrospective analysis of consecutive adult patients (n = 15) who underwent implantation of a leadless pacemaker under direct visualization at the time of valve surgery. Indications for single-chamber pacing were sick sinus syndrome with pauses (53.3%), atrial fibrillation with slow ventricular rates (13.3%) or complete heart block (6.6%), and elevated risk for postoperative heart block (26.6%). Leadless pacemaker performance and pacing percentage were assessed. Results Patients' age was 67.5 ± 17 years, 6 patients (40%) were male, and 14 patients (93%) had atrial fibrillation. Isolated tricuspid valve replacement was performed in 5 patients (33.3%), and the remainder underwent multivalve surgery that included concomitant tricuspid valve repair/replacement. In 93% of the patients (n = 14), the immediate post–cardiopulmonary bypass pacing thresholds were normal (≤2.0 V at 0.24 ms) and normalized in the remaining patient by the next morning. The impedance/sensing values were normal and stable through follow-up (151 ± 119 days) in all patients. Reliable leadless pacemaker performance allowed for deferral of temporary epicardial wires in 11 patients (73%). There were no procedural complications or device malfunction. Conclusions Leadless cardiac pacemaker implantation during valve surgery is feasible and safe. This hybrid approach to pacing may simplify the perioperative management of patients undergoing valve surgery who have an indication for single-chamber pacing.

Background: Obesity predisposes to the development of atrial fibrillation (AF); however, the pathophysiology underlying this relation is only partly understood. Objective: As low-voltage areas are considered indicators of the arrhythmogenic substrates promoting AF, our study aimed to compare the extensiveness of atrial low-voltage areas between obese and nonobese patients by using high-resolution epicardial mapping in order to identify predilection sites of low-voltage areas. Methods: A total of 430 patients (131 (30%) obese and 299 (70%) nonobese) were matched resulting in 212 patients (body mass index [BMI] ≥30 kg/m2: n = 106; BMI 2: n = 106) undergoing cardiac surgery (mean age 63 ± 11 years; 161 male). All patients underwent epicardial mapping of the right atrium, Bachmann bundle (BB), and left atrium during sinus rhythm. Low-voltage potentials were defined as potentials with peak-to-peak amplitudes below the fifth percentile of all potential amplitudes obtained from nonobese patients. Results: Compared with nonobese patients, obese patients have potentials with lower voltages (median of medians) (4.5 mV [0.4–16.2 mV] vs 5.5 mV [0.8–18.0 mV]; P < .001), especially at BB (4.1 mV [0.4–12.3 mV] vs 6.2 mV [1.0–14.3 mV]; P < .001) and left atrium (5.1 mV [0.5–10.1 mV] vs 6.2 mV [0.8–15.9 mV]; P = .003). The percentage of low-voltage potentials was higher in obese (median 3.6% [0.0%–77.1%]) than in nonobese (median 2.3% [0.0%–57.9%]) patients (P < .001), again at BB (obese: 2.9% [0.0%–77.1%] vs nonobese: 0.9% [0.0%–42.0%]; P < .001). Percentages of low-voltage potentials correlated with incidences of conduction block (P < .001), while BMI (P = .044) and low-voltage potentials (P = .001) were independent predictors for the incidence of early postoperative AF. Conclusion: Obesity may predispose to an overall decrease in atrial voltage and a higher percentage in low-voltage potentials. BB was a predilection area for low voltage within the atria of obese patients.

The Brugada pattern manifests as a spontaneous variability of the electrocardiographic marker, suggesting a variability of the underlying electrical substrate.The purpose of this study was to investigate the response of the epicardial substrate of Brugada syndrome (BrS) to programmed ventricular stimulation and to Na blocker infusion.We investigated 6 patients (all male; mean age 54 ± 14 years) with BrS and recurrent ventricular fibrillation. Five had no type 1 BrS electrocardiogram pattern at admission. They underwent combined epicardial-endocardial mapping using multielectrode catheters. Changes in epicardial electrograms were evaluated during single endocardial extrastimulation and after low-dose ajmaline infusion (0.5 mg/kg in 5 minutes).All patients had a region in the anterior epicardial right ventricle with prolonged multicomponent electrograms. Single extrastimulation prolonged late epicardial components by 59 ± 31 ms and in 4 patients abolished epicardial components at some sites, without reactivation by surrounding activated sites. These localized blocks occurred at an initial coupling interval of 335 ± 58 ms and then expanded to other sites, being observed in up to 40% of epicardial sites. Ajmaline infusion prolonged electrogram duration in all and produced localized blocks in 62% of sites in the same patients as during extrastimulation. Epicardial conduction recovery after ajmaline occurred intermittently and at discontinuous sites and produced beat-to-beat changes in local repolarization, resulting in an area of marked electrical disparity. These changes were consistent with models based on microstructural alterations under critical propagation conditions.In BrS, localized functional conduction blocks occur at multiple epicardial sites and with variable patterns, without being reactivated from the surrounding sites.

The electrophysiological properties of the gap associated with the cavotricuspid isthmus (CTI) block line near the inferior vena cava (IVC) are not fully elucidated. Of 143 patients who underwent CTI block line ablation between September 2020 and April 2021, high-resolution CTI gap mapping was performed for 15 patients. Four patients were identified as having a gap near the IVC (IVC-side gap) despite wide double potentials (DPs) with 90 ms intervals at the block line. Detailed gap mapping during coronary sinus ostial pacing was performed before and after touch-up ablation. CTI conduction delays caused by an IVC-side gap were classified into 3 patterns: (1) conduction delay at the IVC-side gap without detouring gap conduction, (2) detouring gap conduction due to intrinsic lower lateral right atrium (LLRA)-IVC functional block, and (3) detouring gap conduction due to LLRA-IVC conduction block created by lateral deviation of the CTI ablation line. In Pattern 2, IVC-side gap conduction traveled backward toward the crista terminalis below the LLRA-IVC junction and came back forward again above the border. One patient presented with a head-to-bottom activation pattern of the lateral right atrium (pseudo-CTI block). Pattern 3 was caused by lateral deviation of initial RF deliveries and presented with the same course as intrinsic LLRA-IVC functional block. All patients had wide DP intervals near the tricuspid annulus (mean, 112 ms) and just above the gap site (mean, 109 ms). An IVC-side gap associated with the CTI block line can present with various conduction delay patterns.

A previously well 48-year-old man presented with presyncope and was found to be in complete heart block. Blood tests, echocardiography and coronary angiography were reported as normal, and a dual chamber permanent pacemaker was inserted. Six months later he re-presented with breathlessness. His chest X-ray showed cardiomegaly and echocardiography revealed a 4.4 cm pericardial effusion. A CT thorax revealed a mass originating from the intra-atrial septum, extending into the right atrium and ventricle. There were multiple pulmonary lesions suspected to be metastases. Histology demonstrated high-grade B-cell lymphoma. He was treated with eight cycles of R-CHOP chemotherapy and showed good radiological and clinical improvement. Post-treatment echocardiography found severe left ventricular dysfunction with an ejection fraction of

Systemic diseases can cause heart block owing to the involvement of the myocardium and thereby the conduction system. Younger patients (

Little is known about the utility of transcatheter aortic valve implantation (TAVI) in patients with cirrhosis of the liver, and their outcomes have not been studied extensively in literature. We performed a retrospective analysis of patients with severe symptomatic aortic stenosis (AS) who underwent transfemoral TAVI with a SAPIEN 3 valve at our institution between April 2015 and December 2018. We identified 32 consecutive patients with evidence of cirrhosis of the liver on imaging (including ultrasound and/or computed tomography) and patients with severe symptomatic AS who underwent transfemoral TAVI with a SAPIEN 3 valve. Among 1,028 patients, 32 had cirrhosis of the liver and 996 constituted the control group without cirrhosis. Mean age in the cirrhosis group was 74.5 years compared with 81.2 years in the control group. Baseline variables were comparable between the groups. Compared with the noncirrhotic group, patients with cirrhosis had a similar 1-year mortality (12% vs 12%, p = 1), a lower 30-day new pacemaker after TAVI rate (6% vs 9%, p = 0.85), a higher 30-day and 1-year readmission rate for heart failure (11% vs 1% and 12% vs 5%, p = 0.12, respectively), and a similar 1-year major adverse cardiac and cerebrovascular event rate (15% vs 14%, p = 0.98). In conclusion, patients with severe AS with concomitant liver cirrhosis who underwent TAVI demonstrated comparable outcomes to their noncirrhotic counterparts.

Delayed atrioventricular (AV) conduction most commonly occurs in the AV node, resulting from AH prolongation on an intracardiac electrocardiogram and PR prolongation on a surface electrocardiogram. AV conduction may be blocked in a 2:1 manner, with a normal PR interval and wide QRS suggesting infranodal disease, whereas a prolonged PR interval and narrow QRS are more suggestive of AV nodal disease. Block within the His is suspected when there is 2:1 AV block with normal PR and QRS intervals. Complete heart block occurs when the atrial rhythm is totally independent of a junctional or lower escape rhythm.

Hypertrophic obstructive cardiomyopathy requires surgical myectomy when heart failure symptoms persist despite best medical therapy. Minimally invasively myectomy with robotic surgery can be performed in experienced centers allowing for surgical correction of accompanying mitral valve pathologies. The extent of myectomy is important to relieve left ventricular outflow tract obstruction while care should be taken to prevent iatrogenic ventricular septal defects or heart blocks caused by excessive removal of septal tissue. We report the use of intracardiac ultrasound during robotic surgery to intraoperatively assess the myocardium before and after myectomy to increase the safety of this procedure. Hypertrophic obstructive cardiomyopathy (HOCM) is a genetic myocardial disease characterized by thickened myocardium and obstruction of the left ventricular outflow tract (LVOT). 1 Patients with symptoms of heart failure resistant to drug treatment are candidates for surgical septal myectomy. Conventionally performed through a midline sternotomy, septal myectomy can be carried out via transapical, transaortic, or transmitral approaches. The transmitral approach allows surgical treatment of the concomitant mitral valve pathology through a left atriotomy. Robotic septal myectomy can be performed in experienced centers with results comparable to the classical sternotomy approach with the added benefits of minimally invasive surgery. 2 Excessive removal of septal tissue can result in a heart block or a ventricular septal defect while an inadequate myectomy can leave a high gradient in the LVOT. Intracardiac ultrasound can assist the surgeon in performing a safe and adequate myectomy. We describe our technique for intracardiac ultrasound guided robotic septal myectomy in a patient with HOCM and infective endocarditis.

Background Mortality in cohorts with a single ventricle remains high with multiple associated factors. The effect of heart block during stage I palliation remains unclear. Objective The purpose of this study was to study patient and surgical risks of heart block and its effect on 12-month transplant-free survival in patients with a single ventricle. Methods Patient, surgical, outcome data and heart block status (transient and permanent) were obtained from the National Pediatric Cardiology Quality Improvement Collaborative single ventricle database. Bivariate analysis was performed comparing patients with and without heart block, and multivariate modeling was used to identify variables associated with block. One-year outcomes were analyzed to identify variables associated with lower 12-month transplant-free survival. Results In total, 1423 patients were identified, of whom 28 (2%) developed heart block (second degree or complete) during their surgical admission. Associated risk factors for block included heterotaxy syndrome (odds ratio [OR] 6.4) and atrial flutter/fibrillation (OR 3.8). Patients with heart block had lower 12-month survival, though only in patients with complete heart block as opposed to second degree block. At 12 months of age, 43% (12/28) of patients with heart block died and were more likely to experience mortality at 12 months than patients without block (OR 4.9; 95% confidence interval 1.4–17.5; P = .01). Conclusion Although rare, complete heart block after stage I palliation represents an additional risk of poor outcomes in this high-risk patient population. Heterotaxy syndrome was the most significant risk factor for the development of heart block after stage I palliation. The role of transient block in outcomes and potential rescue with long-term pacing remains unknown and requires additional study.

Background Residual or new left ventricular outflow tract (LVOT) obstruction after an aortic valve replacement poses special challenges with respect to operative techniques. Our study assesses this gap. Methods From January 1993 to May 2019, 18 patients underwent a septal myectomy at Mayo Clinic for subaortic obstruction after aortic valve replacement. We evaluated their demographics, clinical presentation, and echocardiograms, and the type of prior valve replacement, need for repeat replacement, and their short- and long-term outcomes. The data were analyzed using descriptive statistics. Results All patients underwent septal myectomy for LVOT obstruction at a median interval of 7 years (interquartile range, 3-15 years) from their prior aortic valve procedure. Preoperatively, the median left ventricular outflow tract gradient was 57 mm Hg (interquartile range, 44-77 mm Hg); 10 patients (55.5%) had systolic anterior motion (SAM) of the mitral leaflets. Repeat replacement of the aortic valve at the time of myectomy was needed in 14 patients, and septal myectomy alone was performed in 4 patients. One hospital death occurred 34 days after myectomy and aortic valve replacement, and 2 patients needed permanent pacemaker placement for complete heart block. Conclusions Septal myectomy after aortic valve replacement may be performed with repeat replacement of the valve, if there is coexisting prosthetic dysfunction, through a normally functioning bioprosthesis or through an apical approach when visualization through the aortic prosthesis is poor. The complexity of reoperation supports a liberal approach to myectomy at the time of aortic valve replacement when there is significant subaortic septal hypertrophy.

Congenital complete heart block (CCHB) is a very rare condition, with high risk of mortality. Prematurity is associated with immaturity of the cardiovascular system. Morbidity related to CCHB and prematurity has never been described. We describe a tertiary perinatal center experience over a 15-year period on CCHB management and complications in preterm infants. This is a single-center observational cohort study. All neonates admitted to neonatal intensive care unit with a diagnosis of isolated CCHB between January 2006 and January 2021 were identified. All preterm neonates (

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.

The presence of conduction defects complicating acute myocardial infarction (MI) is relatively frequent and is associated with increased short and long term mortality. Thrombolytic therapy has been established to reduce the mortality in acute MI, however its role in reducing the incidence of conduction defects is less clearly defined. Morbidity and mortality associated with conduction defects also remain unchanged. This study was aimed to assess the correlation between conduction defects and adverse in-hospital outcome of patients with acute ST segment elevation MI. This cross sectional descriptive type of observational study was conducted among 100 purposively selected patients with acute ST segment elevation MI in the coronary care unit (CCU) of Mymensingh Medical College Hospital, Bangladesh from June 2012 to March 2013. The patients were divided into two groups depending on the presence or absence of conduction defects namely Group A- 40 patients with conduction defects and Group B- 60 patients without conduction defects. Highest number of the patients (36.0%) was in the age group of 45-55 years. In case of inferior MI, age groups 45-55 years and 55-65 years had equal number of patients (30.4%). Only 2.0% patients were in age group of 75-85 years and this group had only inferior MI. Total number of female patients were 16(16.0%). Female patients had more inferior MI (17.4%) than anterior MI (15.9%). Number of MI patients was slightly more in inferior MI (46.0%) than anterior MI (44.0%). Group B had equal number of patients in both anterior and inferior MI. Conduction defects were more common in inferior MI (43.5%) than anterior MI (40.9%). Atrio-ventricular conduction defects were more common in inferior MI whereas intra-ventricular conduction defects were more common in anterior MI. Complete heart block (CHB) was more prevalent in inferior MI. Complications were more common in Group A (65.0%) than Group B (18.3%). In terms of complications the difference between two groups were significant (p0.001). Group A showed higher rate of mortality (20.0%) than Group B (3.3%). In cases of anterior MI difference in mortality between Group A and Group B was highly significant (p0.001). Conduction defects in the setting of acute MI are a common finding. Atrio-ventricular conduction defects occur more frequently in inferior MI whereas intra-ventricular conduction defects were more frequently encountered in anterior MI. Patients with conduction defects had more complications than those without conduction defects. Presence of conduction defects significantly increases the mortality of patients with anterior MI.

Ablation of typical atrial flutter has a high safety and efficacy profile, but hidden pitfalls may be encountered. In some cases, a longer cycle length with isoelectric lines is associated with a different or more complex arrhythmogenic substrate, which may be missed if conduction block of the cavotricuspid isthmus is performed in the absence of the clinical arrhythmia. Prior surgery may have consistently modified the atrial substrate and complex or multiple arrhythmias associated with an isthmus-dependent circuit can be encountered. In these cases, electroanatomic mapping is useful to guide the procedure and plan an appropriate ablation strategy.

This article reviews the structure of the atrial chambers to consider the anatomic bases for obstacles and barriers in atrial flutter. In particular, the complex myocardial arrangement and composition of the cavotricuspid isthmus could account for a slow zone of conduction. Prominent muscle bundles within the atria and interatrial, and myoarchitecture of the walls, could contribute to preferential conduction pathways. Alterations from tissue damage as part of aging, or from surgical interventions could lead to re-entry.

In this paper, we present a computational fluid dynamic (CFD) analysis to capture the effect of physical stress and stenosis severity in coronary arteries leading to changes in coronary supply demand oxygen equilibrium. We propose a coupled Od-3d coronary vessel model to predict the variation in flow dynamics of coronary as well as arterial system, modeled using an in-silico model replicating cardiovascular hemodynamics. CFD simulation were solved using subject specific CT scan for coronary and arterial flow and pressure along with metrics related to arterial wall shear stress. Simulations were performed for three heart rates (75, 90 and 120 bpm) and four stenosis states representing different stages of Coronary artery disease (CAD) namely healthy, 50%, 75%, 90% blockage in left anterior descending artery (LAD). Myocardial oxygen supply demand equilibrium were calculated for each cases using hemodynamic surrogate markers naming Diastolic pressure time index for supply and Tension time index for demand. The proposed 0d-3d coupled hemodynamic model of the coronary vessel bed along with supply-demand equilibrium estimated for different stress level and stenosis severity may provide useful insights on the dynamics of CAD manifestation and predict vulnerable regions in coronary bed for early screening and interventions.

A 19-year-old woman with newly diagnosed Lyme disease presented with complete heart block and an accompanying escape rhythm with a right bundle branch block morphology. With antibiotics, her dysrhythmia resolved completely within 24 hours of presentation. This case highlights an unusual electrocardiographic manifestation of Lyme carditis.

Congenital complete heart block and acquired complete heart block are serious conduction abnormalities that may necessitate permanent pacemaker placement. Small patient size and anatomy preclude transvenous pacemaker insertion and risk subcutaneous generator pocket complications. This report describes a case of placement of a pediatric bipolar epicardial pacemaker through a right axillary incision with an intrapleural polytetrafluoroethylene membrane pocket to facilitate future generator exchange.

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

Background The occurrence of cardiovascular events is a major cause of death in patients with cancer. Small studies have documented a connection between specific brain alterations and autonomic cardiac dysfunctions, possibly resulting in a worse prognosis. We aimed to refine the knowledge of fatal cardiac events in patients with brain metastasis (BM). Methods We performed a Surveillance, Epidemiology, and End Results SEER registry‐based investigation (timeline: 2010–2016) and extracted all the advanced patients who had experienced fatal cardiac outcomes. Populations were compared according to the presence or not BM. Kaplan–Meier (KM) methodology was used for survival analysis and a multivariate model was developed by adjusting for multiple possible confounders. Results Most related BM and cardiac death were observed at the site of lung cancer (81.4%). We extracted 3187 patients with lung cancer site, including 417 patients who had experienced fatal heart‐specific with a history of BM, which is considered a BM group. The second group of heart‐specific death included 2770 patients was stated as a non‐BM group. Patients who had experienced heart‐specific death in the BM group were predominately male, right side, upper site, and non‐small type (62.11%, 54.92%, 51.56%, 69.78%), respectively. The survival outcomes between BM and the non‐ BM was significantly prominent (p = 0.003; median: 2 months vs. 3 months).The negative prognostic independent significance of heart‐fatal events was confirmed after adjusting for multiple variables (HR = 0.76, CI = 0.68–84, p, Various studies reported the relation of brain incidents and associated heart dysfunction but no in cancer patients. This first study reported the association between brain metastasis and related lower survival in heat‐related mortality in cancer patients. We uncovered that the associated heart‐specific death and brain metastasis has been occurred mostly occurs in the lung cancer type among all cancer types. We might open a new era for a new field of neuro‐cardio‐oncology. However, the specific mechanisms of pathogenesis warrant further research in the cancer field.

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.

The genetic causes of atrial fibrillation (AF) with slow conduction are unknown. Eight kindreds with familial AF and slow conduction, including a family affected by early onset AF, heart block and incompletely penetrant non-ischemic dilated cardiomyopathy (DCM) underwent whole exome sequencing. A known pathogenic mutation in the desmin (DES) gene resulting in p.S13F substitution (NM_001927.3:c.38C>T) at a PKC phosphorylation site was identified in all four members of the kindred with early-onset AF and heart block, while only two developed DCM. Higher penetrance for AF and heart block prompted a genetic screening for DES modifier(s). A deleterious mutation in the phosphodiesterase-4D-interacting-protein (PDE4DIP) gene resulting in p.A123T substitution (NM_001002811:c.367G>A) was identified that segregated with early onset AF, heart block and the DES mutation. Three additional novel deleterious PDE4DIP mutations were identified in four other unrelated kindreds. Characterization of PDE4DIP(A123T) in vitro suggested impaired compartmentalization of PKA and PDE4D characterized by reduced colocalization with PDE4D, increased cAMP activation leading to higher PKA phosphorylation of the β2-adrenergic-receptor, and decreased PKA phosphorylation of desmin after isoproterenol stimulation. Our findings identify PDE4DIP as a novel gene for slow AF and unravel its epistatic interaction with DES mutations in development of conduction disease and arrhythmia.

Data on mitral valve replacement (MVR) in young children is still limited. Our objective was to evaluate MVR in children below 5 years and identify factors affecting the outcomes. This retrospective study included 29 patients who had MVR from 2002 to 2020. We grouped the patients into two groups according to their age: age ≤ 24 months (n = 18) and > 24 months (n = 11). Primary cardiac diagnoses were Shone complex (n = 7; 24%), isolated congenital mitral valve abnormality (n = 11; 38%), and complete atrioventricular septal defect (n = 3; 10%). The median age was 19 month (25th–75th percentile: 11–32) and 59% were females (n = 17). The hemodynamic lesions were mitral regurgitation in 66%, mitral stenosis in 10%, and combined mitral stenosis and regurgitation in 24% of the patients. St. Jude mitral valve was the most common valve implanted (n = 19, 66%), followed by CarboMedics in 21% of the patients (n = 6). The mitral valve was implanted in the supra-annular position in 6 cases (21%). Preoperative and operative data were comparable between both groups. There was no association between valve size and position with postoperative heart block (P > 0.99, for both). The median follow-up duration was 19.4 months (8.6–102.5). Nine patients had mitral valve reoperation, six had MVR, and three had clot removal from the mitral valve. There was no effect for age group on reoperation (SHR 0.89 (95% CI 0.27–2.87), P = 0.84). Valve size significantly affected reoperation (SHR 0.39 (95% CI 0.18–0.87), P = 0.02). The supra-annular position was associated with an increased risk of reoperation (SHR 3.1 (95% CI 1.003–9.4), P = 0.049). There was no difference in survival according to the age (Log-rank P = 0.57) or valve size (Log-rank P = 0.66). Mitral valve replacement in children is associated with low morbidity and mortality. The risk of reoperation could be affected by the valve size and position rather than the age.

Background Rotational atherectomy (RA) during PCI is linked to a higher likelihood coronary perforations (CP). However, the evidence base on incidence, predictors and outcomes of this complication in RA-PCI remains limited. Methods Using the British Cardiac Intervention Society database, data were analysed on all RA-PCI procedures in UK 2007–2014. Descriptive statistics and multivariate logistic regressions were used to examine baseline, procedural and outcome associations. Results During 10,980 RA-PCI procedures, 167 CPs were recorded (1.52%) with a stable annual incidence. Baseline and procedural covariates associated with higher rates of RA perforation were number of stents used, female gender, smoking, and left-main stenosis. CP was significantly associated with shock, DC cardioversion, heart block, transfusion, emergency surgery, periprocedural MI, in-hospital major bleed, acute kidney injury, dissection, side branch loss and in-hospital death. CP was also associated with higher rates of in-hospital MACCE (OR 12.22, 95% CI 7.67–19.47), 30-day mortality (OR 10.02, 95% CI 5.87–17.09) and 12-month mortality (OR 3.90, 95% CI 2.53–6.02). Conclusions CP is more frequent in RA-PCI than all-comer PCI and is associated with a significant burden of morbidity and mortality. There are a limited number of baseline and procedural co-variates associated with CP in RA-PCI, making it difficult to predict.

Introduction Pediatric patients who receive pacemakers may have a lifetime of repeat exposure to fluoroscopy. His bundle pacing may provide better long-term pacing for these patients. To minimize fluoroscopy and for improved localization of the His bundle, we utilized three-dimensional mapping to guide His bundle pacing and we follow-up short-term results. Methods An Octapolar Livewire catheter (Abbott, Minneapolis, USA) was used for mapping and location of the His bundle while 3-dimensional mapping via the EnSite Precision system (Abbott Medical, Abbott Park, IL) was utilized to create a 3-dimensional geometry. The EnSite Precision system also mapped and recorded His bundle signal strengths and earliest ventricular activation or retrograde His bundle activation. Results Three patients are presented and followed for 4–5 months. The ages ranged from 10 to 15 years with 1 male patient. Diagnoses including progressive atrioventricular block, alternating bundle branch block and atrioventricular canal defect with complete heart block. All patients received selective His-bundle pacing systems. The range in fluoroscopy time was 6.4 to 9.9 min with 5.64 mGy to 10.18 mGy. Stable lead thresholds, impedances and sensing were present at last follow-up. Conclusions His bundle pacing in our 3 pediatric patients including one with altered His-bundle physiology (case 3 with atrioventricular canal defect) was successful with good short-term follow-up results.

Aim: In this study, our aim was to determine clinical factors related to the recovery of the conduction system in patients presenting with atrioventricular (AV) block. Materials & methods: A total of 178 patients who were hospitalized at a tertiary center due to second- or third-degree AV block were retrospectively analyzed. Results: During hospital follow-up, 19.1% of patients had fully recovered from AV block. According to a logistic regression analysis; younger age (odds ratio [OR]: 0.950; 95% CI: 0.932–0.967; p

Sarcoidosis is a granulomatous disease with the potential of multiple organ system involvement and its etiology remains unknown. Cardiac involvement is associated with worse clinical outcome, and has been reported to be 20%-30% in Caucasian and as high as 58% in Japanese populations with sarcoidosis. Clinical manifestations of cardiac sarcoidosis highly depend on the extent and location of granulomatous inflammation. The most frequent presentations include heart block, tachyarrhythmia, or heart failure. Endomyocardial biopsy is the most specific diagnostic test, but has poor sensitivity due to often patchy involvement. The diagnosis of cardiac sarcoidosis remains challenging due to non-specific imaging findings. Both 18F-fluorodeoxyglucose-positron emission tomography (FDG-PET) and cardiac magnetic resonance imaging can be used to evaluate cardiac sarcoidosis, but evaluate different stages of the disease process. FDG-PET detects metabolically-active inflammatory cells while cardiac magnetic resonance imaging with late gadolinium enhancement reveals areas of myocardial necrosis and fibrosis. Aggressive therapy of symptomatic cardiac sarcoidosis is often sought due to the high risk of sudden death and/or progression to heart failure. Prednisone 20-40 mg a day is the recommended initial treatment. In refractory or severe cases, higher doses of prednisone, 1 to 1.5 mg/kg/day (or its equivalent) and addition of a steroid sparing agent have been utilized. Methotrexate is added most commonly. Long-term improvement has been reported with the use of a combination of weekly methotrexate and prednisone versus prednisone alone. After initiation of treatment, a cardiac FDG-PET scan may be performed 2-3 months later to assess treatment response.

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.