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3.
Cardiovasc Diabetol ; 19(1): 55, 2020 05 06.
Article in English | MEDLINE | ID: mdl-32375806

ABSTRACT

Heart failure (HF) is a highly frequent disorder with considerable morbidity, hospitalization, and mortality; thus, it invariably places pressure on clinical and public health systems in the modern world. There have been notable advances in the definition, diagnosis, and treatment of HF, and newly developed agents and devices have been widely adopted in clinical practice. Here, this review first summarizes the current emerging therapeutic agents, including pharmacotherapy, device-based therapy, and the treatment of some common comorbidities, to improve the prognosis of HF patients. Then, we discuss and point out the commonalities and areas for improvement in current clinical studies of HF. Finally, we highlight the gaps in HF research. We are looking forward to a bright future with reduced morbidity and mortality from HF.


Subject(s)
Cardiac Resynchronization Therapy , Cardiovascular Agents/therapeutic use , Electric Countershock , Electric Stimulation Therapy , Heart Failure/therapy , Ventricular Function, Left/drug effects , Cardiac Resynchronization Therapy/adverse effects , Cardiac Resynchronization Therapy/mortality , Cardiac Resynchronization Therapy Devices , Cardiovascular Agents/adverse effects , Comorbidity , Defibrillators, Implantable , Electric Countershock/adverse effects , Electric Countershock/instrumentation , Electric Countershock/mortality , Electric Stimulation Therapy/adverse effects , Electric Stimulation Therapy/instrumentation , Heart Failure/diagnosis , Heart Failure/mortality , Heart Failure/physiopathology , Heart-Assist Devices , Humans , Recovery of Function , Risk Factors , Treatment Outcome , Vagus Nerve Stimulation
4.
Circ Arrhythm Electrophysiol ; 13(6): e008733, 2020 06.
Article in English | MEDLINE | ID: mdl-32423252

ABSTRACT

In the past year, there have been numerous advances in our understanding of arrhythmia mechanisms, diagnosis, and new therapies. We have seen advances in basic cardiac electrophysiology with data suggesting that secretoneurin may be a biomarker for patients at risk of ventricular arrhythmias, and we have learned of the potential role of an NPR-C (natriuretic peptide receptor-C) in atrial fibrosis and the role of an atrial specific 2-pore potassium channel TASK-1 as a therapeutic target for atrial fibrillation. We have seen studies demonstrating the role of sensory neurons in sleep apnea-related atrial fibrillation and the association between bariatric surgery and atrial fibrillation ablation outcomes. Artificial intelligence applied to electrocardiography has yielded estimates of age, sex, and overall health. We have seen new tools for collection of patient-centered outcomes following catheter ablation. There have been significant advances in the ability to identify ventricular tachycardia termination sites through high-density mapping of deceleration zones. We have learned that right ventricular dysfunction may be a predictor of survival benefit after implantable cardioverter-defibrillator implantation in patients with nonischemic cardiomyopathy. We have seen further insights into the role of His bundle pacing on improving outcomes. As our understanding of cardiac laminopathies advances, we may have new tools to predict arrhythmic event rates in gene carriers. Finally, we have seen numerous advances in the treatment of arrhythmias in patients with congenital heart disease.


Subject(s)
Arrhythmias, Cardiac , Heart Conduction System , Action Potentials , Animals , Arrhythmias, Cardiac/diagnosis , Arrhythmias, Cardiac/mortality , Arrhythmias, Cardiac/physiopathology , Arrhythmias, Cardiac/therapy , Cardiac Pacing, Artificial , Catheter Ablation , Cryosurgery , Defibrillators, Implantable , Electric Countershock/instrumentation , Electrocardiography , Electrophysiologic Techniques, Cardiac , Genetic Predisposition to Disease , Heart Conduction System/physiopathology , Heart Conduction System/surgery , Heart Rate , Humans , Pacemaker, Artificial , Risk Factors , Treatment Outcome
6.
J Cardiovasc Electrophysiol ; 31(6): 1493-1506, 2020 06.
Article in English | MEDLINE | ID: mdl-32333433

ABSTRACT

BACKGROUND: Morphology algorithms are currently recommended as a standalone discriminator in single-chamber implantable cardioverter defibrillators (ICDs). However, these proprietary algorithms differ in both design and nominal programming. OBJECTIVE: To compare three different algorithms with nominal versus advanced programming in their ability to discriminate between ventricular (VT) and supraventricular tachycardia (SVT). METHODS: In nine European centers, VT and SVTs were collected from Abbott, Boston Scientific, and Medtronic dual- and triple-chamber ICDs via their respective remote monitoring portals. Percentage morphology matches were recorded for selected episodes which were classified as VT or SVT by means of atrioventricular comparison. The sensitivity and related specificity of each manufacturer discriminator was determined at various values of template match percentage from receiving operating characteristics (ROC) curve analysis. RESULTS: A total of 534 episodes were retained for the analysis. In ROC analyses, Abbott Far Field MD (area under the curve [AUC]: 0.91; P < .001) and Boston Scientific RhythmID (AUC: 0.95; P < .001) show higher AUC than Medtronic Wavelet (AUC: 0.81; P < .001) when tested for their ability to discriminate VT from SVT. At nominal % match threshold all devices provided high sensitivity in VT identification, (91%, 100%, and 90%, respectively, for Abbott, Boston Scientific, and Medtronic) but contrasted specificities in SVT discrimination (85%, 41%, and 62%, respectively). Abbott and Medtronic's nominal thresholds were similar to the optimal thresholds. Optimization of the % match threshold improved the Boston Scientific specificity to 79% without compromising the sensitivity. CONCLUSION: Proprietary morphology discriminators show important differences in their ability to discriminate SVT. How much this impact the overall discrimination process remains to be investigated.


Subject(s)
Algorithms , Defibrillators, Implantable , Electric Countershock/instrumentation , Electrophysiologic Techniques, Cardiac/instrumentation , Signal Processing, Computer-Assisted , Tachycardia, Supraventricular/diagnosis , Tachycardia, Ventricular/diagnosis , Telemetry/instrumentation , Action Potentials , Diagnosis, Differential , Equipment Design , Europe , Heart Rate , Humans , Predictive Value of Tests , Reproducibility of Results , Retrospective Studies , Tachycardia, Supraventricular/physiopathology , Tachycardia, Supraventricular/therapy , Tachycardia, Ventricular/physiopathology , Tachycardia, Ventricular/therapy
7.
J Cardiovasc Electrophysiol ; 31(4): 846-853, 2020 04.
Article in English | MEDLINE | ID: mdl-32064713

ABSTRACT

INTRODUCTION: Atrial tachycardia/fibrillation (AT/AF) episodes are common in implantable cardioverter-defibrillator (ICD) recipients and can be undetected by standard single-chamber devices. This study aims to explore whether a single-lead ICD with an atrial dipole (ICD DX; BIOTRONIK SE & Co, Berlin, Germany) could improve the AT/AF diagnosis and management as compared to standard ICD (ICD VR). METHODS AND RESULTS: We selected patients without AT/AF history from the THINGS registry which included consecutive patients implanted with ICD for standard indications. The ICD VR and the ICD DX groups included 236 (62.8%) and 140 (37.2%) patients, respectively, and had no significant differences in baseline characteristics. During a median follow-up of 27 months, there were 7 AT/AF diagnoses in the ICD VR and 18 in the ICD DX group. The 2-year incidence of AT/AF diagnosis was 3.6% (95% confidence interval [CI]: 1.6%-9.6%) for the ICD VR and 11.4% (95% CI: 6.8%-18.9%) for the ICD DX group (adjusted hazard ratio [HR]: 3.85 [95% CI: 1.58-9.41]; P = .003). Initiation of oral anticoagulation (OAC) due to AT/AF diagnosis was reported in 15 patients. The 2-year incidence of OAC onset was 3.6% (95% CI: 1.6%-7.8%) for the ICD VR and 6.3% (95% CI: 3.0%-12.7%) for ICD DX group (adjusted HR: 1.99 [95% CI: 0.72-5.56]; P = .184). CONCLUSION: We observed that atrial sensing capability in single-chamber ICD patients without evidence of atrial arrhythmias at implant is associated with a greater likelihood of detecting AT/AF episodes. The management of these diagnosed arrhythmias often led to clinical interventions, mainly represented by initiation of OAC therapy.


Subject(s)
Atrial Fibrillation/diagnosis , Atrial Function , Cardiomyopathies/therapy , Defibrillators, Implantable , Electric Countershock/instrumentation , Electrophysiologic Techniques, Cardiac/instrumentation , Heart Failure/therapy , Tachycardia, Supraventricular/diagnosis , Administration, Oral , Aged , Anti-Arrhythmia Agents/administration & dosage , Anticoagulants/administration & dosage , Atrial Fibrillation/epidemiology , Atrial Fibrillation/physiopathology , Atrial Fibrillation/therapy , Cardiomyopathies/diagnosis , Cardiomyopathies/epidemiology , Cardiomyopathies/physiopathology , Catheter Ablation , Electric Countershock/adverse effects , Female , Heart Failure/diagnosis , Heart Failure/epidemiology , Heart Failure/physiopathology , Humans , Incidence , Male , Middle Aged , Predictive Value of Tests , Prospective Studies , Prosthesis Design , Registries , Tachycardia, Supraventricular/epidemiology , Tachycardia, Supraventricular/physiopathology , Tachycardia, Supraventricular/therapy , Time Factors , Treatment Outcome
8.
J Cardiovasc Med (Hagerstown) ; 21(3): 171-181, 2020 Mar.
Article in English | MEDLINE | ID: mdl-32004241

ABSTRACT

: In recent years, the increasing number of patients with a cardiac implantable electronic device (CIED) has required different approaches in terms of the device's control and surveillance. It is increasingly difficult to keep the traditional in-office protocol device's control: we must think of a different organization dedicated to the activity of remote control and monitoring (RC/RM) of devices and patients.A CIED team structured with nurses, technicians and physicians should be organized inside the hospital, with the aim of CIED patients' managing and of creating a network between the various departments.Small hospitals may not be able to manage independently the CIEDs RC/RM and it is possible to hypothesize the creation of a collaborative network between neighbouring structures.This activity must combine the use of technology with the ability to take care of patients and to maintain adequate and meaningful relationships.


Subject(s)
Cardiac Pacing, Artificial , Cardiology Service, Hospital/organization & administration , Defibrillators, Implantable , Delivery of Health Care, Integrated/organization & administration , Electric Countershock/instrumentation , Heart Diseases/therapy , Models, Organizational , Pacemaker, Artificial , Remote Sensing Technology , Consensus , Cooperative Behavior , Heart Diseases/diagnosis , Heart Diseases/physiopathology , Humans , Interdisciplinary Communication , Patient Care Team/organization & administration , Patient Participation , Predictive Value of Tests , Treatment Outcome
10.
Heart Vessels ; 35(1): 118-124, 2020 Jan.
Article in English | MEDLINE | ID: mdl-31250130

ABSTRACT

The association between periprocedural hypotension and conscious sedation (CS) during defibrillator implantation remains to be elucidated. The aim of the present study was to compare the occurrence of periprocedural hypotension after CS or local anesthesia (LA) during defibrillator implantation in a retrospective cohort study using a national inpatient database. Using the Japanese Diagnosis Procedure Combination database, we retrospectively collected data for adult inpatients who underwent implantation of a cardioverter defibrillator or cardiac resynchronization therapy device from July 2010 to March 2016. Multivariable logistic regression analyses were performed to compare the occurrence of periprocedural hypotension between the CS and LA groups with adjustment for patient background characteristics and hospital factors. Additional analysis was performed after dividing the CS group into each specific anesthetic use. We identified 4842 patients, comprising 1533 patients with CS and 3309 with LA. The CS group had a significantly higher proportion of periprocedural hypotension than the LA group (13.4% versus 9.7%; adjusted odds ratio, 1.56; 95% confidence interval, 1.19-2.04; p = 0.001). Body mass index < 18.5 kg/m2, New York Heart Association Class IV, and use of cardiac resynchronization therapy device were independently associated with occurrence of periprocedural hypotension. Additionally, ketamine and dexmedetomidine were significantly associated with higher incidence of hypotension than the LA group (adjusted odds ratio, 2.64; 95% confidence interval, 1.32-5.26; p = 0.006; adjusted odds ratio, 1.86; 95% confidence interval, 1.11-3.12; p = 0.019, respectively). Periprocedural hypotension was significantly more likely to occur in the CS group than the LA group, and was associated with CS.


Subject(s)
Anesthesia, Local/adverse effects , Blood Pressure , Conscious Sedation/adverse effects , Defibrillators, Implantable , Electric Countershock/instrumentation , Hypotension/etiology , Ventricular Dysfunction, Left/therapy , Ventricular Function, Left , Aged , Aged, 80 and over , Anesthesia, Local/mortality , Conscious Sedation/mortality , Databases, Factual , Electric Countershock/adverse effects , Electric Countershock/mortality , Female , Humans , Hypotension/diagnosis , Hypotension/mortality , Hypotension/physiopathology , Inpatients , Japan/epidemiology , Male , Middle Aged , Retrospective Studies , Risk Assessment , Risk Factors , Time Factors , Treatment Outcome , Ventricular Dysfunction, Left/diagnosis , Ventricular Dysfunction, Left/mortality , Ventricular Dysfunction, Left/physiopathology
11.
J Cardiovasc Electrophysiol ; 30(11): 2441-2447, 2019 11.
Article in English | MEDLINE | ID: mdl-31512339

ABSTRACT

BACKGROUND: Since the introduction of the subcutaneous implantable-defibrillator (S-ICD) knowledge of factors elevating the defibrillation threshold (DFT), have increased. Optimal device positioning most likely results in a decrease in DFT. The PRAETORIAN score is a tool to systematically evaluate S-ICD implant position and predict conversion success by estimating the DFT on a chest X-ray. The objective of this study is to determine DFT in de novo S-ICD patients. METHODS: De novo S-ICD patients were enrolled with DFT testing using a single 30 J shock or a prespecified step-down protocol. Chest X-rays were obtained and implant position was evaluated using the PRAETORIAN score. RESULTS: Fifteen patients, age 47 (±18) years and body mass index (BMI) 25(±3) kg/m2 , underwent conversion testing with a single 30 J shock. Fourteen patients (93%) converted successfully. Implant position was optimal in all patients, PRAETORIAN score of 30 (100%). Twelve patients were available for analysis in the step-down DFT cohort, age 46 years (±15), left ventricular ejection fraction 50% (±15%) and a median BMI of 25.7 kg/m2 (range 23.8-29.1 kg/m2 ). No complications occurred during DFT testing. Mean DFT was 29 J (±12 J). Time to shock was 11 seconds (±2 seconds) for a 20 J shock compared with 17 seconds in case of a 70 J shock output. Nearly all patients were implanted with optimal device positioning with the lowest possible PRAETORIAN score of 30 (92%). CONCLUSION: DFT in S-ICD patients with optimal device positioning is lower than previously reported. Conversion testing using a low shock output reduced time to therapy by 6 seconds on average.


Subject(s)
Arrhythmias, Cardiac/therapy , Defibrillators, Implantable , Electric Countershock/instrumentation , Action Potentials , Adult , Aged , Arrhythmias, Cardiac/diagnosis , Arrhythmias, Cardiac/physiopathology , Electric Countershock/adverse effects , Electrophysiologic Techniques, Cardiac , Female , Heart Rate , Humans , Male , Middle Aged , Prospective Studies , Prosthesis Design , Radiography, Thoracic , Time Factors , Treatment Outcome
12.
J Cardiovasc Electrophysiol ; 30(11): 2334-2343, 2019 11.
Article in English | MEDLINE | ID: mdl-31433089

ABSTRACT

INTRODUCTION: The majority of patients with nonischemic cardiomyopathy (NICM) present a perivalvular substrate that is either predominantly antero-septal (AS) or infero-lateral (IL), corresponding to specific ventricular tachycardia (VT) morphologies. The relative timing of far-field and near-field ventricular electrograms (EGMs) from stored implantable cardioverter-defibrillator (ICD) events of VT may be used to distinguish AS from IL VT in NICM. METHODS AND RESULTS: We analyzed 48 patients with NICM with either a primarily AS (54%) or IL (56%) VT source undergoing catheter ablation between 2003 and 2018. Only patients with retrievable ICD-EGMs of spontaneous VT events which could be matched with VTs induced during the ablation procedure were included. A total of 56 VT events (52% AS origin and 48% IL origin) were analyzed, yielding a mean far-field to near-field interval of 31 ± 13 milliseconds for AS VTs and 47 ± 19 milliseconds for IL VTs (P = .001). At receiver operating characteristic analysis (AUC = 0.734), a far-field to near-field interval of ≥ 60 milliseconds ruled out AS VTs in 29 (100%) cases and diagnosed IL VTs with a positive predictive value (PPV) of 100% and a negative predictive value (NPV) of 63%. An interval of ≤ 20 milliseconds ruled out IL VTs in 25 (93%) cases and diagnosed AS VTs with a PPV of 83% and NPV of 57%. Significant overlap between the two groups was observed among far-field to near-field intervals in between 20 milliseconds and 60 milliseconds. CONCLUSIONS: The relative timing of far-field and near-field EGMs from stored clinical ICD events of VT can be helpful to differentiate AS vs IL origin of VT in NICM.


Subject(s)
Action Potentials , Cardiomyopathies/therapy , Defibrillators, Implantable , Electric Countershock/instrumentation , Electrophysiologic Techniques, Cardiac/instrumentation , Heart Rate , Tachycardia, Ventricular/diagnosis , Aged , Cardiomyopathies/complications , Cardiomyopathies/diagnosis , Cardiomyopathies/physiopathology , Catheter Ablation , Electric Countershock/adverse effects , Female , Humans , Male , Middle Aged , Predictive Value of Tests , Prognosis , Retrospective Studies , Tachycardia, Ventricular/etiology , Tachycardia, Ventricular/physiopathology , Tachycardia, Ventricular/surgery , Time Factors
14.
J Cardiovasc Electrophysiol ; 30(10): 1994-2001, 2019 10.
Article in English | MEDLINE | ID: mdl-31328298

ABSTRACT

INTRODUCTION: Subclinical atrial fibrillation (AF), in the form of cardiac implantable device-detected atrial high rate episodes (AHREs), has been associated with increased thromboembolism. An implantable cardioverter-defibrillator (ICD) lead with a floating atrial dipole may permit a single lead (DX) ICD system to detect AHREs. We sought to assess the utility of the DX ICD system for subclinical AF detection in patients, with a prospective multicenter, cohort-controlled trial. METHODS AND RESULTS: One hundred fifty patients without prior history of AF (age 59 ± 13 years; 108 [72%] male) were enrolled into the DX cohort and implanted with a Biotronik DX ICD system at eight centers. Age-, sex-, and left ventricular ejection fraction-matched single- and dual-chamber ICD cohorts were derived from a Cornell database and from the IMPACT trial, respectively. The primary endpoint were AHRE detection at 12 months. During median 12 months follow-up, AHREs were detected in 19 (13%) patients in the DX, 8 (5.3%) in the single-chamber, and 19 (13%) in the dual-chamber cohorts. The rate of AHRE detection was significantly higher in the DX cohort compared to the single-chamber cohort (P = .026), but not significantly different compared to the dual-chamber cohort. There were no inappropriate ICD therapies in the DX cohort. At 12 months, only 3.0% of patients in the DX cohort had sensed atrial amplitudes less than 1.0 mV. CONCLUSION: Use of a DX ICD lead allows subclinical AF detection with a single lead DX system that is superior to that of a conventional single-chamber ICD system.


Subject(s)
Atrial Fibrillation/diagnosis , Death, Sudden, Cardiac/prevention & control , Defibrillators, Implantable , Electric Countershock/instrumentation , Electrophysiologic Techniques, Cardiac/instrumentation , Remote Sensing Technology/instrumentation , Action Potentials , Adult , Aged , Asymptomatic Diseases , Atrial Fibrillation/physiopathology , Female , Heart Rate , Humans , Male , Middle Aged , Predictive Value of Tests , Prospective Studies , Time Factors , United States
16.
J Cardiovasc Electrophysiol ; 30(10): 1939-1948, 2019 10.
Article in English | MEDLINE | ID: mdl-31257683

ABSTRACT

INTRODUCTION: While cardiac sarcoidosis (CS) carries a risk of ventricular arrhythmias (VAs) and sudden cardiac death (SCD), risk stratification of patients with CS and preserved left ventricular/right ventricular (LV/RV) systolic function remains challenging. We sought to evaluate the role of electrophysiologic testing and programmed electrical stimulation of the ventricle (EPS) in patients with suspected CS with preserved ventricular function. METHODS: One hundred twenty consecutive patients with biopsy-proven extracardiac sarcoidosis and preserved LV/RV systolic function underwent EPS. All patients had either probable CS defined by an abnormal cardiac positron emission tomography or cardiac magnetic resonance imaging, or possible CS with normal advanced imaging but abnormal echocardiogram (ECG), SAECG, Holter, or clinical factors. Patients were followed for 4.5 ± 2.6 years for SCD and VAs. RESULTS: Seven of 120 patients (6%) had inducible ventricular tachycardia (VT) with EPS and received an implantable cardioverter defibrillator (ICD). Three patients (43%) with positive EPS later had ICD therapies for VAs. Kaplan-Meier analysis stratified by EPS demonstrated a significant difference in freedom from VAs and SCD (P = 0.009), though this finding was driven entirely by patients within the cohort with probable CS (P = 0.018, n = 69). One patient with possible CS and negative EPS had unrecognized progression of the disease and unexplained death with evidence of CS at autopsy. CONCLUSIONS: EPS is useful in the risk stratification of patients with probable CS with preserved LV and RV function. A positive EPS was associated with VAs. While a negative EPS appeared to confer low risk, close follow-up is needed as EPS cannot predict fatal VAs related to new cardiac involvement or disease progression.


Subject(s)
Action Potentials , Arrhythmias, Cardiac/diagnosis , Cardiomyopathies/diagnosis , Electrocardiography , Electrophysiologic Techniques, Cardiac , Heart Rate , Sarcoidosis/diagnosis , Ventricular Function, Left , Ventricular Function, Right , Aged , Arrhythmias, Cardiac/mortality , Arrhythmias, Cardiac/physiopathology , Arrhythmias, Cardiac/therapy , Cardiomyopathies/mortality , Cardiomyopathies/physiopathology , Cardiomyopathies/therapy , Death, Sudden, Cardiac/prevention & control , Defibrillators, Implantable , Disease Progression , Electric Countershock/instrumentation , Female , Humans , Male , Middle Aged , Predictive Value of Tests , Prognosis , Retrospective Studies , Risk Assessment , Risk Factors , Sarcoidosis/mortality , Sarcoidosis/physiopathology , Sarcoidosis/therapy , Stroke Volume , Systole , Time Factors
17.
J Cardiovasc Electrophysiol ; 30(10): 2020-2026, 2019 10.
Article in English | MEDLINE | ID: mdl-31343808

ABSTRACT

INTRODUCTION: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited progressive cardiomyopathy characterized by frequent life-threatening arrhythmias. The diagnosis of ARVC is challenging and is on the basis of a set of major and minor criteria as described by the modified Task Force Criteria (TFC). We report our clinical experience in a series of patients who were misdiagnosed with ARVC and subsequently underwent removal of their implantable cardioverter defibrillator (ICD) after a re-evaluation at our center. METHODS AND RESULTS: We studied 12 patients who were misdiagnosed with ARVC and had ICD implantation before our assessment. All patients had a repeat evaluation and were scored according to TFC before ICD removal. Cardiac magnetic resonance imaging (CMR) studies performed at outside institutions during the initial evaluation were reported abnormal and classified as meeting major TFC in ninety percent of patients. The most common abnormality reported was fatty infiltration of the right ventricular (RV) free wall and/or presence of focal intra-myocardial fat in six patients (50%). On re-evaluation, none of these findings fulfilled the TFC for the diagnosis. CONCLUSION: This study demonstrated that high dependence on misinterpretation of CMR along with a misunderstanding of the TFC evaluation are the main reasons for the misdiagnosis of ARVC. Despite the updated criteria for almost a decade, this study reminds that the diagnosis of ARVC is complex and hence careful TFC evaluation and consideration of multiple cardiac test results should be the focused approach for clinicians when confronted with suspected ARVC patients.


Subject(s)
Arrhythmogenic Right Ventricular Dysplasia/diagnosis , Defibrillators, Implantable , Device Removal , Diagnostic Errors , Electric Countershock/instrumentation , Electrophysiologic Techniques, Cardiac , Magnetic Resonance Imaging, Cine , Unnecessary Procedures/instrumentation , Adult , Arrhythmogenic Right Ventricular Dysplasia/pathology , Arrhythmogenic Right Ventricular Dysplasia/physiopathology , Female , Heart Conduction System/physiopathology , Humans , Male , Middle Aged , Myocardium/pathology , Predictive Value of Tests , Registries , Unnecessary Procedures/methods , Young Adult
18.
J Cardiovasc Electrophysiol ; 30(10): 1932-1938, 2019 10.
Article in English | MEDLINE | ID: mdl-31240821

ABSTRACT

BACKGROUND: Danon disease is a rare X-linked storage disorder characterized by hypertrophic cardiomyopathy leading to arrhythmias and heart failure. A preexcitation pattern on electrocardiogram (ECG) has been described in these patients, however, invasive studies to distinguish between Wolff-Parkinson-White syndrome syndrome and fasciculoventricular pathways (FVP) are limited. OBJECTIVES: The purpose of this study was to delineate the electrophysiological cardiac abnormalities in patients with Danon disease and to describe the presence of FVP in this population. METHODS: We performed a retrospective study of all patients with a confirmed diagnosis of Danon disease presenting to a single center from May 2005 to May 2018. Baseline demographics, clinical characteristics, ECG findings, and electrophysiology study (EPS) results were collected. RESULTS: Ten patients with Danon disease (30% male, average age 17.4 years) were identified. Seven patients (70%) had tachyarrhythmias including five with atrial arrhythmias and six with nonsustained ventricular tachycardia. Preexcitation pattern on ECG was found in four (40%) patients. Of these, two underwent an EPS which confirmed the presence of an FVP. One patient underwent an adenosine challenge which supported a FVP. Implantable cardioverter defibrillator was placed in five patients for primary prevention with no patients receiving an appropriate discharge. Over a follow-up of 5.3 years, five underwent heart transplantation. CONCLUSIONS: This study reports a high incidence of FVP in patients with Danon disease and preexcitation. It underscores an alternate etiology of preexcitation in this population which can potentially be diagnosed without invasive EPS testing. Future multicenter studies are needed to expand this experience.


Subject(s)
Accessory Atrioventricular Bundle/etiology , Arrhythmias, Cardiac/etiology , Cardiomyopathy, Hypertrophic/etiology , Glycogen Storage Disease Type IIb/complications , Accessory Atrioventricular Bundle/diagnosis , Accessory Atrioventricular Bundle/physiopathology , Action Potentials , Adolescent , Adult , Arrhythmias, Cardiac/diagnosis , Arrhythmias, Cardiac/physiopathology , Arrhythmias, Cardiac/therapy , Cardiomyopathy, Hypertrophic/diagnosis , Cardiomyopathy, Hypertrophic/physiopathology , Cardiomyopathy, Hypertrophic/therapy , Child , Death, Sudden, Cardiac/etiology , Death, Sudden, Cardiac/prevention & control , Defibrillators, Implantable , Electric Countershock/instrumentation , Electrocardiography , Electrophysiologic Techniques, Cardiac , Female , Genetic Predisposition to Disease , Glycogen Storage Disease Type IIb/diagnosis , Glycogen Storage Disease Type IIb/genetics , Glycogen Storage Disease Type IIb/therapy , Heart Failure/etiology , Heart Failure/physiopathology , Heart Failure/therapy , Heart Rate , Humans , Lysosomal-Associated Membrane Protein 2/genetics , Male , Mutation , Ohio , Primary Prevention , Retrospective Studies , Risk Factors , Treatment Outcome , Young Adult
19.
J Cardiovasc Electrophysiol ; 30(9): 1602-1609, 2019 09.
Article in English | MEDLINE | ID: mdl-31190453

ABSTRACT

INTRODUCTION: Emerging medical technology has allowed for monitoring of heart rhythm abnormalities using smartphone compatible devices. The safety and utility of such devices have not been established in patients with cardiac implantable electronic devices (CIEDs). We sought to assess the safety and compatibility of the Food and Drug Administration-approved AliveCor Kardia device in patients with CIEDs. METHODS AND RESULTS: We prospectively recruited patients with CIED for a Kardia recording during their routine device interrogation. A recording was obtained in paced and nonpaced states. Adverse clinical events were noted at the time of recording. Electrograms (EGMs) from the cardiac device were obtained at the time of recording to assess for any electromagnetic interference (EMI) introduced by Kardia. Recordings were analyzed for quality and given a score of 3 (interpretable rhythm, no noise), 2 (interpretable rhythm, significant noise) or 1 (uninterpretable). A total of 251 patients were recruited (59% with a pacemaker and 41% with ICD). There were no adverse clinical events noted at the time of recording and no changes to CIED settings. Review of all EGMs revealed no EMI introduced by Kardia. Recordings were correctly interpreted in 90% of paced recordings (183 had a score of 3, 43 of 2, and 25 of 1) and 94.7% of nonpaced recordings (147 of 3, 15 of 2, and 9 of 1). CONCLUSION: The AliveCor Kardia device has an excellent safety profile when used in conjunction with most CIEDs. The quality of recordings was preserved in this population. The device, therefore, can be considered for heart rhythm monitoring in patients with CIEDs.


Subject(s)
Arrhythmias, Cardiac/therapy , Cardiac Pacing, Artificial , Defibrillators, Implantable , Electric Countershock/instrumentation , Electrophysiologic Techniques, Cardiac/instrumentation , Heart Rate , Mobile Applications , Pacemaker, Artificial , Remote Sensing Technology/instrumentation , Smartphone , Aged , Aged, 80 and over , Arrhythmias, Cardiac/diagnosis , Arrhythmias, Cardiac/physiopathology , Artifacts , Cardiac Pacing, Artificial/adverse effects , Defibrillators, Implantable/adverse effects , Electric Countershock/adverse effects , Electrophysiologic Techniques, Cardiac/adverse effects , Female , Humans , Male , Middle Aged , Pacemaker, Artificial/adverse effects , Predictive Value of Tests , Prospective Studies , Remote Sensing Technology/adverse effects , Reproducibility of Results , Risk Factors , Signal Processing, Computer-Assisted , Time Factors
20.
J Cardiovasc Electrophysiol ; 30(9): 1526-1534, 2019 09.
Article in English | MEDLINE | ID: mdl-31187564

ABSTRACT

INTRODUCTION: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is characterized by an epicardial (EPI) to endocardial (ENDO) fibrofatty infiltration of the RV predisposing to both EPI and ENDO ventricular tachycardia (VT). The relative timing between the VT QRS onset on the far-field ventricular electrogram (VEGM) to the local activation time recorded at the RV apex on the near-field VEGM from stored implantable cardioverter-defibrillator (ICD) events of VT can be helpful to discriminate ENDO from EPI VT in ARVC. METHODS AND RESULTS: We analyzed consecutive ARVC patients undergoing catheter ablation between 2006 and 2018. Only patients with retrievable ICD VEGMs of clinical VTs which could be matched with VTs induced at the time of ablation were included. A total of 26 VT events (16 ENDO, 10 EPI) from 19 ARVC patients were examined, yielding a mean far-field to near-field interval of 33 ± 15 ms for ENDO VTs and 52 ± 20 ms for EPI VTs (P = .020). At receiver-operating characteristic analysis, a far-field to a near-field interval of 60 ms or more ruled out ENDO VTs in 16 (100%) cases and identified EPI VTs with a positive predictive value (PPV) of 100% and a negative predictive value (NPV) of 73%. An interval of less than or equal to 30 ms ruled out EPI VTs in eight (80%) cases and diagnosed ENDO VTs with a PPV of 80% and an NPV of 50%. CONCLUSION: Far-field to near-field ICD VEGM timing may be used to predict ENDO vs EPI VT in ARVC before ablation, indicating an ENDO origin if the timing is less than or equal to 30 ms and an EPI origin if greater than or equal to 60 ms.


Subject(s)
Arrhythmogenic Right Ventricular Dysplasia/diagnosis , Defibrillators, Implantable , Electric Countershock/instrumentation , Electrophysiologic Techniques, Cardiac/instrumentation , Endocardium/physiopathology , Pericardium/physiopathology , Tachycardia, Ventricular/diagnosis , Action Potentials , Aged , Arrhythmogenic Right Ventricular Dysplasia/physiopathology , Arrhythmogenic Right Ventricular Dysplasia/therapy , Catheter Ablation , Diagnosis, Differential , Endocardium/surgery , Female , Heart Rate , Humans , Male , Middle Aged , Pericardium/surgery , Predictive Value of Tests , Retrospective Studies , Tachycardia, Ventricular/physiopathology , Tachycardia, Ventricular/therapy , Time Factors
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