Cost of Pacing in Pediatric Patients With Postoperative Heart Block After Congenital Heart Surgery.

Importance: Surgical correction of congenital heart defects (CHDs) has improved the lifespan and quality of life of pediatric patients. The number of congenital heart surgeries (CHSs) in children has grown continuously since the 1960s. This growth has been accompanied by a rise in the incidence of postoperative heart block requiring permanent pacemaker (PPM) implantation. Objective: To assess the trends in permanent pacing after CHS and estimate the economic burden to patients and their families after PPM implantation. Design, Setting, and Participants: In this economic evaluation study, procedure- and diagnosis-specific codes within a single-institution database were used to identify patients with postoperative heart block after CHS between January 1, 1960, and December 31, 2018. Patients younger than 4 years with postoperative PPM implantation were selected, and up to 20-year follow-up data were used for cost analysis based on mean hospital event charges and length of stay (LOS) data. Data were analyzed from January 1, 2020, to November 30, 2022. Exposure: Implantation of PPM after CHS in pediatric patients. Main Outcomes and Measures: Annual trends in CHS and postoperative PPM implantations were assessed. Direct and indirect costs associated with managing conduction health for the 20 years after PPM implantation were estimated using Markov model simulation and patient follow-up data. Results: Of the 28 225 patients who underwent CHS, 968 (437 female [45.1%] and 531 male [54.9%]; 468 patients aged <4 years) received a PPM due to postoperative heart block. The rate of CHS and postoperative PPM implantations increased by 2.2% and 7.2% per year between 1960 and 2018, respectively. In pediatric patients younger than 4 years with PPM implantation, the mean (SD) 20-year estimated direct and indirect costs from Markov model simulations were $180 664 ($32 662) and $15 939 ($1916), respectively. Using follow-up data of selected patients with clinical courses involving 1 or more complication events, the mean (SD) direct and indirect costs were $472 774 ($212 095) and $36 429 ($16 706), respectively. Conclusions and Relevance: In this economic evaluation study, the cost of PPM implantation in pediatric patients was found to accumulate over the lifespan. This cost may represent not only a substantial financial burden but also a health care burden to patient families. Reducing the incidence of PPM implantation should be a focused goal of CHS.

Aseptic presentation of interventricular septal abscess with progressive heart block: a case report.

BACKGROUND: Infective endocarditis can progress to an intracardiac abscess in 20% to 30% of cases, with interventricular septal abscess (IVSA) being one of the rare complications usually presenting with sepsis. We present a case of IVSA presenting with a new-onset second-degree heart block, which rapidly progressed to a complete heart block. CASE PRESENTATION: A 80-year-old Caucasian female with a past medical history of hypertension and hyperlipidemia presented with exertional chest pain, lightheadedness, and shortness of breath with telemetry and electrocardiogram revealing persistent Mobitz type II second degree atrioventricular block. The rest of the vitals were normal. As she was being planned for a pacemaker placement, she spiked a temperature of 103F. Blood cultures grew methicillin-sensitive Staphylococcus aureus, and appropriate antibiotics were initiated. Transthoracic echocardiogram was grossly normal. However, transesophageal echocardiogram revealed a heterogeneous extension of an echodensity from the aortic root, along the aorto-mitral cushion and into the interventricular septum, indicating an interventricular septal abscess. Her course was complicated by altered mental status, with computed tomography of the brain revealing hypodense regions in the left lentiform nucleus and anterior caudate nucleus representing acute/subacute stroke. Surgery was deferred as she was deemed a poor candidate. She succumbed to her illness on day 6 of hospitalization. CONCLUSION: Intracardiac abscesses should be considered a possible initial differential in patients with progressive heart block despite aseptic presentation and no risk factors.

Cohort study of congenital complete heart block among preterm neonates: a single-center experience over a 15-year period.

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 (< 37 weeks) were compared with a control cohort of term neonates (≥ 37 weeks). Antenatal data, complications of prematurity, medical, and surgical management of CCHB were recorded. Twenty-four neonates with isolated CCHB (16 preterm and 8 term) were born during the study period, including 5 very preterm (< 32 weeks) and 11 preterm (32 to 37 weeks). All very preterm were born via emergency caesarian section without antenatal steroid administration. They had multiple severe morbidities including chronic lung disease, necrotizing enterocolitis, grades 3-4 intraventricular hemorrhage, cystic periventricular leukomalacia, and longer periods of mechanical and non-invasive ventilatory support than preterm. Thirteen out of sixteen preterm infants had permanent pacemakers inserted, compared to 1/8 for term newborns. All babies born before 35-week gestation were either paced or died.Conclusion: Premature neonates with CCHB have high risk of mortality and morbidity especially if undiagnosed and born by unnecessary emergency caesarian section without antenatal steroids. Prematurity below 35 weeks may be associated with death or pacemaker insertion. This supports better antenatal screening to avoid induced prematurity. What is Known: • Congenital complete heart block is a very rare condition associated with high morbidity and mortality. • Antenatal risk factors for poor outcome include fetal hydrops, low ventricular rate (HR <55 beats per minute), and congenital heart defect. What is New: • Infants born <32 weeks with CCHB had no antenatal steroid administration, and sustained high burden of morbidity (chronic lung disease, intraventricular hemorrhage, and cystic periventricular leukomalacia). • Birth <35 weeks is strongly associated with requiring pacing prior to discharge or death.

A rare cause of complete heart block in an adult: Primary cardiac lymphoma.

Primary cardiac lymphoma (PCL) is a rare primary cardiac neoplasm with a relatively poor prognosis despite confinement to the heart and/or pericardium. We report a 54-year-old man who had presented with complete heart block for which he had undergone permanent pacemaker implantation, and six months later he was referred to us for evaluation of progressive exertional dyspnea. Multislice spiral computed tomography angiography of the heart and major vasculature showed infiltrative tumoral lesion that has involved interatrial septum, right atrium, left atrium, and left ventricle with invasion into the pulmonary artery. Tissue samples were taken using uniportal video-assisted thoracoscopic surgery, and the histologic examination revealed diffuse large B-cell lymphoma. Chemoimmunotherapy was effective in inducing tumor regression and the patient was still in remission during the next six months after treatment.

Conduction abnormalities and role of cardiac pacing in cardiac amyloidosis: A systematic review.

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.

Outcomes and periprocedural management of cardiac implantable electronic devices in patients with carcinoid heart disease.

BACKGROUND: Carcinoid heart disease (CHD) is a rare complication of hormonally active neuroendocrine tumors that often requires surgical intervention. Data on cardiac implantable electronic device (CIED) implantation in patients with CHD are limited. OBJECTIVE: The purpose of this study was to evaluate the experience of CIED implantation in patients with CHD. METHODS: Patients with a diagnosis of CHD and a CIED procedure from January 1, 1995, through June 1, 2020, were identified using a Mayo Clinic proprietary data retrieval tool. Retrospective review was performed to extract relevant data, which included indications for implant, procedural details, complications, and mortality. RESULTS: A total of 27 patients (55.6% male; mean age at device implant 65.6 ± 8.8 years) with cumulative follow-up of 75 patient-years (median 1.1 years; interquartile range 0.4-4.6 years) were included for analysis. The majority of implanted devices were dual-chamber permanent pacemakers (63%). Among all CHD patients who underwent any cardiac surgery, the incidence of CIED implantation was 12%. The most common indication for implantation was high-grade heart block (66.7%). Device implant complication rates were modest (14.8%). No patient suffered carcinoid crisis during implantation, and there was no periimplant mortality. Median time from implant to death was 2.5 years, with 1-year mortality of 15%. CONCLUSION: CHD is a morbid condition, and surgical valve intervention carries associated risks, particularly a high requirement for postoperative pacing needs. Our data suggest that CIED implantation can be performed relatively safely. Clinicians must be aware of the relevant carcinoid physiology and take appropriate precautions to mitigate risks.

Adverse device-device interaction between pacemaker and subcutaneous implantable cardiac defibrillator.

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.

Infective Endocarditis with Pseudoaneurysm of the Mitral-Aortic Intervalvular Fibrosa Presenting as Complete Heart Block.

A 79-year-old male with a history of ESRD and treated MRSA endocarditis was found to have a recurrence of MRSA bacteremia. He was treated with antibiotics. During his hospitalization, he suddenly developed complete heart block requiring transcutaneous pacing, and subsequently transvenous pacing wires were placed. Transesophageal echocardiography demonstrated pseudo- aneurysm of the mitral-aortic intervalvular fibrosa as well as aortic valve thickening, and a mitral vegetation. Cardiothoracic surgery was consulted to obtain source control, but the patient was deemed to be a poor surgical candidate. While continuing medical therapy and transvenous pacing, the patient developed refractory hypotension, acidosis, and ultimately expired.

Reversible complete heart block in a patient with coronavirus disease 2019.

Patients infected with novel coronavirus (SARS-CoV-2) can present with a variety of arrhythmias. We report an unusual case of reversible complete heart block (CHB) in the setting of acute coronavirus disease 2019 (COVID-19). A 23-year-old male with a history of Hodgkin's Lymphoma presented with dizziness and syncope. He was found to be in CHB associated with hypotension requiring a transvenous pacemaker. Methylprednisolone and remdesivir were started with rapid resolution of the CHB. Further study is needed to determine the mechanism of CHB in COVID-19. This case underscores the importance of including COVID-19 in one's differential diagnosis for acute CHB.

A Case of Lymphocytic Myocarditis With Complete Heart Block Requiring Permanent Pacemaker Implantation.

Lymphocytic myocarditis (LM) is a rare condition with a broad spectrum of clinical presentations. Complete heart block (CHB) is an uncommon sequela. We present a case of a young man with biopsy-proven LM who presented with cardiogenic shock and went on to develop nonresolving CHB, requiring permanent pacemaker implantation.

Complete heart block as a herald sign for cardiac lymphoma.

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 <20%. Heart failure medical therapy was optimised and the pacemaker was upgraded to a resynchronisation device. A repeat scan 6 months post device upgrade showed an improvement in ejection fraction to 45%-50%.

Early Versus Delayed Pacemaker for Heart Block After Valve Surgery: A Cost-Effectiveness Analysis.

BACKGROUND: A significant percentage of patients who acutely develop high-grade atrioventricular block after valve surgery will ultimately recover, yet the ability to predict recovery is limited. The purpose of this analysis was to evaluate the cost-effectiveness of two different management strategies for the timing of permanent pacemaker implantation for new heart block after valve surgery. METHODS: A cost-effectiveness model was developed using costs and probabilities of short- and long-term complications of pacemaker placement, short-term atrioventricular node recovery, intensive care unit stays, and long-term follow-up. We aggregated the total expected cost and utility of each option over a 20-y period. Quality-adjusted survival with a pacemaker was estimated from the literature and institutional patient-reported outcomes. Primary decision analysis was based on an expected recovery rate of 36.7% at 12 d with timing of pacemaker implantation: early placement (5 d) versus watchful waiting for 12 d. RESULTS: A strategy of watchful waiting was more costly ($171,798 ± $45,695 versus $165,436 ± $52,923; P < 0.0001) but had a higher utility (9.05 ± 1.36 versus 8.55 ± 1.33 quality-adjusted life years; P < 0.0001) than an early pacemaker implantation strategy. The incremental cost-effectiveness ratio of watchful waiting was $12,724 per quality-adjusted life year. The results are sensitive to differences in quality-adjusted survival and rates of recovery of atrioventricular node function. CONCLUSIONS: Watchful waiting for pacemaker insertion is a cost-effective management strategy compared with early placement for acute atrioventricular block after valve surgery. Although this is cost-effective from a population perspective, clinical risk scores predicting recovery will aid in personalized decision-making.

Case Report: Complete heart block as a manifestation of cardiac metastasis of oral cancer.

Metastatic tumors of the heart presenting with complete heart block (CHB) is an extremely uncommon case. There are no available guidelines in managing CHB in terminal cancer. Permanent pacemaker implantation in such cases is a challenge in terms of clinical utility and palliative care. We report a case of a 24-year-old man suffering from tongue cancer presenting with CHB. A intracardiac mass and moderate pericardial effusion were present, presumed as the metastatic tumor of tongue cancer. We implanted a temporary pacemaker for his symptomatic heart block and cardiogenic shock, and pericardiocentesis for his massive pericardial effusion. We decided that a permanent pacemaker would not be implanted based on the low survival rate and significant comorbidities. Multiple studies report a variable number of cardiac metastasis incidence ranging from 2.3% to 18.3%. It is rare for such malignancies to present with CHB. The decision to implant a permanent pacemaker is highly specific based on the risks and benefits of each patient. It needs to be tailored to the patient's functional status, comorbid diseases, prognosis, and response to conservative management.

Short term clinical outcomes and analysis of risk factors for pacemaker implantation: a single center experience of self-expandable TAVI valves.

OBJECTIVES: Transcatheter aortic valve implantation is a recognized treatment for patients with severe aortic stenosis at all risk groups. However, permanent pacemaker rates remain high for self expandable transcatheter valves and permanent pacemaker implantation has been associated with increased morbidity. In this analysis we aim to evaluate short term clinical outcomes post self expandable transcatheter aortic valve implantation and determine risk factors for permanent pacemaker implantation. METHODS: 88 patients with severe aortic stenosis with transcatheter aortic valve implantation performed between the year 2016-2018 were retrospectively analyzed. Outcomes of interest included 1- year all cause mortality, 30-day major adverse cardiovascular events, permanent pacemaker and paravalvular leak rates. Survival analysis was performed with Kaplan Meier analysis and risk factors for survival and permanent pacemaker rates were identified with log rank test and regression analysis. RESULTS: The mean age of the cohort was 80.3 +/- 6.9 years. The mean STS score was 9.25. The 30 day all-cause mortality was 5.7% and 1-year all cause mortality was 16.7%. 80 patients had transfemoral transcatheter aortic valve implantation, and a majority of the patients (85.2%) were implanted with Corevalve Evolut R device. The device success rate was 88.6%. Multivariate analysis identified concomitant severe coronary artery disease (OR = 18.2 +/- 0.9; P = 0.002), pre transcatheter aortic valve implantation atrial fibrillation (OR = 8.6 +/- 0.91; P = 0.02) and post procedural disabling stroke (OR = 32.6 +/- 1.35; P = 0.01) as risk factors for 1-year mortality. The 30-day pacemaker rate was 17.6%. The presence of right bundle branch block (OR 11.1 +/- 0.86; P = 0.005), non-coronary cusp implantation depth (OR = 1.34 +/- 0.15; P = 0.05) and a non coronary cusp implantation depth / membranous septal length ratio of more than 50% were associated with post procedural pacemaker implantation (OR = 29.9 +/- 1.72; P = 0.05). Among the 15 patients with post procedural pacemaker implantation, 40% were found to be non-pacemaker dependent at 1 year. CONCLUSION: Short term outcomes of transcatheter aortic valve implantation in severe aortic stenosis patients are promising. Pacemaker rates remain high. More studies are needed to evaluate the factors that influence pacemaker rates and dependence to further improve transcatheter aortic valve implantation outcomes.

A case of cardiac strangulation following epicardial pacemaker implantation.

An 8-year-old boy had undergone permanent epicardial pacemaker implantation with a Y-shaped bipolar ventricular lead on day 6 after birth for treatment of congenital complete atrioventricular block. He was found to have pulmonary stenosis and mitral stenosis by follow-up echocardiography. Further studies including computed tomography and cardiac catheterization revealed that the pacemaker lead had completely encircled the cardiac silhouette and was in a state of "cardiac strangulation". We removed the previous pacing leads and generator and implanted a new epicardial dual-chamber pacing system in the right atrium and right ventricle. Additionally, an expanded polytetrafluoroethylene sheet was placed between the new leads and the heart to prevent recurrence of cardiac strangulation.

Pacemaker Implantation in Neonates and Infants: Favorable Outcomes with Epicardial Pacing Systems.

The implantation of pacemakers (PM) in neonates and infants requires particular consideration of small body size, marked body growth potential, and the decades of future pacing therapy to be expected. The aim of this study is to quantify the complications of implantation and outcome occurring at our center and to compare these with other centers. Retrospective analysis of 52 consecutive patients undergoing PM implantation at a single tertiary care center within the first year of life. PMs were implanted at a median age of 3 months (range 0-10 months). Structural heart defects were present in 44 of 52 patients. During a median follow-up time of 40.4 months (range 0.1-114 months), measurements for sensing, pacing thresholds, and lead impedance remained stable. No adverse pacing effect was observed in left ventricular function or dimensions over time. There were 20 reoperations in 13 patients at a median time of 4.7 years (range 0.05-8.2 years) after implantation, for end of battery life (n = 10), lead dysfunction (n = 3), device dislocation (n = 3), infection (n = 3), and diaphragmatic paresis (n = 1). No PM-related mortality occurred. Epicardial pacemaker implantation in neonates and infants is an invasive but safe and effective procedure with a relatively low risk of complications. Our current implantation technique and the use of bipolar steroid-eluting electrodes, which we prefer to implant on the left ventricular apex, lead to favorable long-term results.