Asunto(s)
Ablación por Catéter , Taquicardia Ventricular , Complejos Prematuros Ventriculares , Humanos , Complejos Prematuros Ventriculares/cirugía , Complejos Prematuros Ventriculares/fisiopatología , Complejos Prematuros Ventriculares/diagnóstico , Ablación por Catéter/métodos , Taquicardia Ventricular/cirugía , Taquicardia Ventricular/fisiopatología , Taquicardia Ventricular/diagnóstico , Taquicardia Ventricular/etiología , Electrocardiografía , Resultado del Tratamiento , Sistema de Conducción Cardíaco/fisiopatología , Sistema de Conducción Cardíaco/cirugíaRESUMEN
The first symptoms of catecholaminergic polymorphic ventricular tachycardia (CPVT) usually occur in childhood and adolescence. 60% of patients experience syncope before the age of 40. Sudden cardiac death (SCD) is the first symptom of the disease in 30-50% of patients with CPVT. Early diagnosis is therefore crucial for the patient's prognosis. The diagnosis of CPVT is confirmed by a normal resting ECG, exclusion of structural heart disease, detection of bidirectional or polymorphic ventricular tachycardia (VT) in the stress ECG and/or detection of a pathogenic mutant in a gene associated with CPVT. Up to 60% of CPVT patients carry changes in the RYR2 gene. This gene encodes the cardiac ryanodine receptor, the most important Ca2+-releasing channel of the sarcoplasmic reticulum, which plays a central role in the contraction and relaxation of the heart muscle. If the function of the ryanodine receptor is impaired, too much calcium enters the cells, which triggers life-threatening arrhythmias. The overactive ryanodine receptor is therefore the main target for gene therapy methods. Even though the development of gene therapy is progressing, there is still no causal therapy available and it is all the more important to make a diagnosis as early as possible, which enables appropriate behavior and adequate symptomatic therapy. The decisive factor here is the evaluation of the genetic analysis in the context of the clinical findings. Based on this, recommendations can be made for preventive measures and the avoidance of specific triggers that could lead to life-threatening arrhythmias.
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Muerte Súbita Cardíaca , Canal Liberador de Calcio Receptor de Rianodina , Taquicardia Ventricular , Humanos , Taquicardia Ventricular/genética , Taquicardia Ventricular/diagnóstico , Taquicardia Ventricular/terapia , Muerte Súbita Cardíaca/etiología , Muerte Súbita Cardíaca/prevención & control , Canal Liberador de Calcio Receptor de Rianodina/genética , Adolescente , Niño , Electrocardiografía , Adulto , Pronóstico , Adulto JovenRESUMEN
This article describes the case of a 40-year-old individual who presented with fulminant myocarditis. Initial ECG displayed sinus tachycardia with a heart rate of 117 bpm, QS complexes in leads V1-V3, ST-segment depression in leads II, III, aVF, V5-V6, and ST-segment elevation >0.2 mV in leads V1 through V3. The initial clinical assessment suggested an acute anteroseptal myocardial infarction. However, subsequent diagnostic evaluation through coronary angiography disclosed that the coronary arteries were normal. Therefore, clinicians should carefully consider the differential diagnosis between these conditions, as their management strategies differ markedly. Two hours after admission, the patient unexpectedly developed syncope. The ECG findings were consistent with the typical characteristics of bidirectional ventricular tachycardia. Our report described the appearance and morphology as well as mechanism of bidirectional ventricular tachycardia in detail. Additionally, we delineate differential diagnoses for disease that can cause bidirectional ventricular tachycardia, such as aconite poisoning, digoxin overdose, immune checkpoint inhibitor (ICI), myocardial ischemia, and hereditary channelopathies, such as catecholaminergic polymorphic ventricular tachycardia (CPVT) and Andersen-Tawil syndrome. Therefore, clinicians should recognize this ECG finding immediately and initiate appropriate treatment promptly as these measures may be vital in saving the patient's life.
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Electrocardiografía , Humanos , Electrocardiografía/métodos , Adulto , Diagnóstico Diferencial , Masculino , Taquicardia/diagnóstico , Taquicardia/fisiopatología , Miocarditis/diagnóstico , Miocarditis/fisiopatología , Miocarditis/complicaciones , Taquicardia Ventricular/diagnóstico , Taquicardia Ventricular/fisiopatologíaRESUMEN
BACKGROUND: Idiopathic ventricular arrhythmias (IVAs) arising from different portions of the communicating vein of the left ventricular summit (summit-CV) are not a rare phenomenon. Whereas its electrocardiographic (ECG) and electrophysiological characteristics are not fully investigated. OBJECTIVE: This study aimed to identify distinct ECG and electrophysiological features of IVAs originating from different portions of summit-CV. METHODS: Nineteen patients confirmed arising from summit-CV were included in this study. RESULTS: The 19 patients were divided into proximal and distal portion groups based on their target sites in summit-CV. In the proximal portion group, 100% (11/11) VAs showed dominant negative (rs or QS) waves in lead I, while in the distal portion group, 87.5% (7/8) showed dominant positive waves (R, Rs or r) (p < 0.000). In lead V1, 100% (11/11) of the proximal portion group showed dominant positive waves (R or Rs), while 62.50% (5/8) of the distal portion group showed positive and negative bidirectional or negative waves (RS or rS) (p < 0.005). RI>4mV, SI<3.5mV, RV1<13mV, SV1>3.5mV, RI/SI>0.83, and RV1/SV1< 2.6 indicated a distal portion of summit-CV with the predictive value of 0.909, 1.000, 0.653, 0.972, 0.903, 0.966, respectively. A more positive wave in lead I and a more negative wave in lead V1 indicated more distal origin in summit-CV. Target sites in proximal and distal summit-CV groups showed similar electrophysiological characteristics during mapping. CONCLUSIONS: There were significant differences in ECG characteristics of VAs at different portions of summit-CV, which could aid pre-procedure planning and facilitate radiofrequency catheter ablation (RFCA) procedures.
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Potenciales de Acción , Ablación por Catéter , Electrocardiografía , Frecuencia Cardíaca , Ventrículos Cardíacos , Valor Predictivo de las Pruebas , Humanos , Ablación por Catéter/efectos adversos , Femenino , Masculino , Persona de Mediana Edad , Adulto , Resultado del Tratamiento , Ventrículos Cardíacos/fisiopatología , Ventrículos Cardíacos/cirugía , Taquicardia Ventricular/fisiopatología , Taquicardia Ventricular/cirugía , Taquicardia Ventricular/diagnóstico , Técnicas Electrofisiológicas Cardíacas , Estudios Retrospectivos , AncianoRESUMEN
BACKGROUND: Electrocardiographic abnormalities are common in arrhythmogenic right ventricular cardiomyopathy and are included in the 2010 Task Force Criteria. Their time course, however, remains uncertain. In this retrospective observational study, we aimed to assess the long-term evolution of electrocardiographic characteristics and their relation to ventricular arrhythmias. METHODS AND RESULTS: Three hundred fifty-three patients with arrhythmogenic right ventricular cardiomyopathy as per the 2010 Task Force Criteria with 6871 automatically processed 12-lead digital ECGs were included. The relationship between the electrocardiographic parameters and the risk of ventricular arrhythmias was assessed at 10 years from the first ECG. Electrocardiographic parameters were compared between the first contact ECG, the ECG at diagnosis, and the most recent ECG. Median time between the first and the latest ECG was 6 [interquartile range, 1-14] years. Reductions of QRS voltage, R- and T-wave amplitudes between the first, diagnostic, and the latest ECGs were observed across precordial and extremity leads. Mean QRS duration increased from 96 to 102 ms (P<0.001), terminal activation duration (V1) from 47 to 52 ms (P<0.001), and QTc from 419 to 432 ms (P<0.001). T-wave inversions in leads V3 to V6 and aVF at first ECG were associated with ventricular arrhythmias (adjusted hazard ratio [HRadj][V3], 2.03 [95% CI, 1.23-3.34] and HRadj[aVF], 1.87 [95% CI, 1.13-3.08]). CONCLUSIONS: Depolarization and repolarization parameters evolved over time in patients with arrhythmogenic right ventricular cardiomyopathy, supporting the progressive nature of arrhythmogenic right ventricular cardiomyopathy. Electrocardiographic abnormalities may be detected before diagnosis and might, although not fulfilling the 2010 Task Force Criteria, be markers of early disease. T-wave inversion in leads V3 or aVF before diagnosis was associated with ventricular arrhythmias during follow-up.
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Displasia Ventricular Derecha Arritmogénica , Electrocardiografía , Humanos , Displasia Ventricular Derecha Arritmogénica/diagnóstico , Displasia Ventricular Derecha Arritmogénica/fisiopatología , Displasia Ventricular Derecha Arritmogénica/complicaciones , Masculino , Estudios Retrospectivos , Femenino , Adulto , Persona de Mediana Edad , Factores de Tiempo , Factores de Riesgo , Progresión de la Enfermedad , Taquicardia Ventricular/diagnóstico , Taquicardia Ventricular/fisiopatología , Taquicardia Ventricular/etiología , Potenciales de Acción , Valor Predictivo de las PruebasRESUMEN
PURPOSE OF REVIEW: Cardiac sarcoidosis (CS) refers to cardiac involvement in sarcoidosis and is usually associated with worse outcomes. This comprehensive review aims to elucidate the electrocardiographic (ECG) signs and features associated with CS, as well as examine modern techniques and their importance in CS evaluation. RECENT FINDINGS: The exact pathogenesis of CS is still unclear, but it stems from an abnormal immunological response triggered by environmental factors in individuals with genetic predisposition. CS presents with non-cardiac symptoms; however, conduction system abnormalities are common in patients with CS. The most common electrocardiographic (ECG) signs include atrioventricular blocks and ventricular tachyarrhythmia. Distinct patterns, such as fragmented QRS complexes, T-wave alternans, and bundle branch blocks, are critical indicators of myocardial involvement. The application of advanced ECG techniques such as signal-averaged ECG, Holter monitoring, wavelet-transformed ECG, microvolt T-wave alternans, and artificial intelligence-supported analysis holds promising outcomes for opportune detection and monitoring of CS. Timely utilisation of inexpensive and readily available ECG possesses the potential to allow early detection and intervention for CS. The integration of artificial intelligence models into ECG analysis is a promising approach for improving the ECG diagnostic accuracy and further risk stratification of patients with CS.
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Cardiomiopatías , Electrocardiografía , Sarcoidosis , Humanos , Sarcoidosis/fisiopatología , Sarcoidosis/diagnóstico , Electrocardiografía/métodos , Cardiomiopatías/fisiopatología , Cardiomiopatías/diagnóstico , Pronóstico , Taquicardia Ventricular/diagnóstico , Taquicardia Ventricular/fisiopatología , Electrocardiografía AmbulatoriaRESUMEN
BACKGROUND: Differentiating wide complex tachycardias (WCTs) into ventricular tachycardia (VT) and supraventricular wide tachycardia via 12-lead ECG interpretation is a crucial but difficult task. Automated algorithms show promise as alternatives to manual ECG interpretation, but direct comparison of their diagnostic performance has not been undertaken. METHODS: Two electrophysiologists applied 3 manual WCT differentiation approaches (ie, Brugada, Vereckei aVR, and VT score). Simultaneously, computerized data from paired WCT and baseline ECGs were processed by 5 automated WCT differentiation algorithms (WCT Formula, WCT Formula II, VT Prediction Model, Solo Model, and Paired Model). The diagnostic performance of automated algorithms was compared with manual ECG interpretation approaches. RESULTS: A total of 212 WCTs (111 VT and 101 supraventricular wide tachycardia) from 104 patients were analyzed. WCT Formula demonstrated superior accuracy (85.8%) and specificity (87.1%) compared with Brugada (75.2% and 57.4%, respectively) and Vereckei aVR (65.3% and 36.4%, respectively). WCT Formula II achieved higher accuracy (89.6%) and specificity (85.1%) against Brugada and Vereckei aVR. Performance metrics of the WCT Formula (accuracy 85.8%, sensitivity 84.7%, and specificity 87.1%) and WCT Formula II (accuracy 89.8%, sensitivity 89.6%, and specificity 85.1%) were similar to the VT score (accuracy 84.4%, sensitivity 93.8%, and specificity 74.2%). Paired Model was superior to Brugada in accuracy (89.6% versus 75.2%), specificity (97.0% versus 57.4%), and F1 score (0.89 versus 0.80). Paired Model surpassed Vereckei aVR in accuracy (89.6% versus 65.3%), specificity (97.0% versus 75.2%), and F1 score (0.89 versus 0.74). Paired Model demonstrated similar accuracy (89.6% versus 84.4%), inferior sensitivity (79.3% versus 93.8%), but superior specificity (97.0% versus 74.2%) to the VT score. Solo Model and VT Prediction Model accuracy (82.5% and 77.4%, respectively) was superior to the Vereckei aVR (65.3%) but similar to Brugada (75.2%) and the VT score (84.4%). CONCLUSIONS: Automated WCT differentiation algorithms demonstrated favorable diagnostic performance compared with traditional manual ECG interpretation approaches.
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Algoritmos , Electrocardiografía , Taquicardia Supraventricular , Taquicardia Ventricular , Humanos , Electrocardiografía/métodos , Taquicardia Ventricular/diagnóstico , Taquicardia Ventricular/fisiopatología , Femenino , Persona de Mediana Edad , Masculino , Taquicardia Supraventricular/diagnóstico , Taquicardia Supraventricular/fisiopatología , Diagnóstico Diferencial , Valor Predictivo de las Pruebas , Adulto , Reproducibilidad de los Resultados , Anciano , Procesamiento de Señales Asistido por Computador , AutomatizaciónRESUMEN
The predictive value of various ECG repolarization markers for the emergence of VA in patients with TTS was reviewed. The literature reports on QT, more recently on Tpe, and rarely on some more QT-derived metrics, revealing a contribution of these variables for the prediction of VA, complicating the acute, subacute, and follow-up clinical trajectory of patients with TTS. More recent literature reveals that Tpe and some other QT-based metrics, have outperformed the traditionally employed QT marker, although certainty about this awaits confirmation by future carefully designed and implemented studies.
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Electrocardiografía , Cardiomiopatía de Takotsubo , Humanos , Electrocardiografía/métodos , Cardiomiopatía de Takotsubo/diagnóstico , Cardiomiopatía de Takotsubo/fisiopatología , Cardiomiopatía de Takotsubo/complicaciones , Valor Predictivo de las Pruebas , Taquicardia Ventricular/fisiopatología , Taquicardia Ventricular/diagnóstico , Taquicardia Ventricular/etiología , Arritmias Cardíacas/fisiopatología , Arritmias Cardíacas/diagnóstico , Arritmias Cardíacas/etiologíaRESUMEN
BACKGROUND: Cardiac magnetic resonance (CMR) allows comprehensive myocardial tissue characterisation, revealing areas of myocardial inflammation or fibrosis that may predispose to ventricular arrhythmias (VAs). With this study, we aimed to estimate the prevalence of structural heart disease (SHD) and decipher the prognostic implications of CMR in selected patients presenting with significant VAs. METHODS: Electronic databases were searched for studies enrolling adult patients that underwent CMR for diagnostic or prognostic purposes in the setting of significant VAs. A random effects model meta-analysis of proportions was performed to estimate the prevalence of SHD. HRs were pooled together in order to evaluate the prognostic value of CMR. RESULTS: The prevalence of SHD was reported in 18 studies. In all-comers with significant VAs, the pooled rate of SHD post-CMR evaluation was 39% (24% in the subgroup of premature ventricular contractions and/or non-sustained ventricular tachycardia vs 63% in the subgroup of more complex VAs). A change in diagnosis after use of CMR ranged from 21% to 66% with a pooled average of 35% (29%-41%). A non-ischaemic cardiomyopathy was the most frequently identified SHD (56%), followed by ischaemic heart disease (21%) and hypertrophic cardiomyopathy (5%). After pooling together data from six studies, we found that the presence of late gadolinium enhancement was associated with increased risk of major adverse outcomes in patients with significant VAs (pooled HR: 1.79; 95% CI 1.33 to 2.42). CONCLUSION: CMR is a valuable tool in the diagnostic and prognostic evaluation of patients with VAs. CMR should be considered early after initial evaluation in the diagnostic algorithm for VAs of unclear aetiology as this strategy may also define prognosis and improve risk stratification.
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Taquicardia Ventricular , Humanos , Arritmias Cardíacas/diagnóstico , Arritmias Cardíacas/epidemiología , Imagen por Resonancia Magnética/métodos , Prevalencia , Pronóstico , Taquicardia Ventricular/diagnóstico , Taquicardia Ventricular/epidemiología , Taquicardia Ventricular/etiología , Complejos Prematuros Ventriculares/diagnóstico , Complejos Prematuros Ventriculares/epidemiología , Complejos Prematuros Ventriculares/fisiopatologíaRESUMEN
Takotsubo syndrome (TTS), also known as stress-induced cardiomyopathy, is characterized by acute heart failure, reversible left ventricular dysfunction, and other complications such as life-threatening arrhythmias. The management of TTS is challenging due to its unpredictable clinical course and the lack of evidence-based treatment recommendations. In this case report, we present a 71-year-old female who developed TTS with ventricular tachycardia (VT) cardiac arrest following septic shock and an exploratory laparotomy for appendicitis. Despite the presence of VT cardiac arrest and a left ventricular ejection fraction of 30-35%, an implanted cardioverter-defibrillator (ICD) was not indicated due to the rapid and satisfactory recovery of the patient's ventricular function. This case highlights the importance of considering the clinical context and the transient nature of TTS in the decision-making process for ICD candidacy.
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Desfibriladores Implantables , Paro Cardíaco , Cardiomiopatía de Takotsubo , Humanos , Cardiomiopatía de Takotsubo/terapia , Cardiomiopatía de Takotsubo/diagnóstico , Cardiomiopatía de Takotsubo/complicaciones , Femenino , Anciano , Paro Cardíaco/terapia , Paro Cardíaco/etiología , Taquicardia Ventricular/terapia , Taquicardia Ventricular/etiología , Taquicardia Ventricular/diagnósticoRESUMEN
Genetic arrhythmia disorders are rare diseases; however, they are a common cause of sudden cardiac death in children, adolescents, and young adults. In principle, a distinction can be made between channelopathies and cardiomyopathies in the context of genetic diseases. This paper focuses on the channelopathies long and short QT syndrome, Brugada syndrome, and catecholaminergic polymorphic ventricular tachycardia (CPVT). Early diagnosis of these diseases is essential, as drug therapy, behavioral measures, and if necessary, implantation of a cardioverter defibrillator can significantly improve the prognosis and quality of life of patients. This paper highlights the pathophysiological and genetic basis of these channelopathies, describes their clinical manifestations, and comments on the principles of diagnosis, risk stratification and therapy.
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Arritmias Cardíacas , Síndrome de Brugada , Canalopatías , Humanos , Arritmias Cardíacas/genética , Arritmias Cardíacas/diagnóstico , Arritmias Cardíacas/terapia , Arritmias Cardíacas/fisiopatología , Canalopatías/genética , Canalopatías/diagnóstico , Canalopatías/terapia , Síndrome de Brugada/genética , Síndrome de Brugada/diagnóstico , Síndrome de Brugada/fisiopatología , Síndrome de Brugada/terapia , Taquicardia Ventricular/genética , Taquicardia Ventricular/terapia , Taquicardia Ventricular/diagnóstico , Taquicardia Ventricular/fisiopatología , Adolescente , Niño , Síndrome de QT Prolongado/genética , Síndrome de QT Prolongado/diagnóstico , Síndrome de QT Prolongado/terapia , Síndrome de QT Prolongado/fisiopatología , Muerte Súbita Cardíaca/prevención & control , Muerte Súbita Cardíaca/etiología , Adulto , Desfibriladores Implantables , ElectrocardiografíaRESUMEN
AIMS: Wearable cardioverter-defibrillators (WCDs) are indicated in patients at risk of sudden cardiac arrest who are not immediate candidates for implantable defibrillator therapy. Limitations of existing WCDs include poor compliance and high false alarm rates. The Jewel is a novel patch-WCD (P-WCD) that addresses these limitations with an adhesive-based design for near-continuous wear and a machine learning algorithm designed to minimize inappropriate detections. This was a first-in-human study of the Jewel P-WCD conducted in an electrophysiology (EP) lab to determine the safety and effectiveness of the device in terminating ventricular tachycardia/ventricular fibrillation (VT/VF) with a single shock. The aim was to evaluate the safety and effectiveness of terminating VT/VF with a single shock using the Jewel P-WCD. METHODS AND RESULTS: This was a first-in-human, prospective, single-arm, single-centre study in patients scheduled for an EP procedure in which VT/VF was expected to either spontaneously occur or be induced. The Jewel P-WCD was placed on consented patients; upon confirmation of VT/VF, a single shock (150 J) was delivered via the device. A group sequential design and Pocock alpha spending function was used to measure the observed proportion of successful VT/VF single-shock terminations. The endpoint was achieved if the lower confidence limit exceeded the performance goal of 62%, using a one-sided lower 97.4% exact confidence bound. Of 18 eligible subjects, 16 (88.9%, 97.4% confidence bound: 65.4%) were successfully defibrillated with a single shock, exceeding the primary endpoint performance goal with no adverse events. CONCLUSION: This first-in-human evaluation of the Jewel P-WCD demonstrated the safety and effectiveness of terminating VT/VF. CLINICAL TRIAL REGISTRATION: URL: https://clinicaltrials.gov/; Unique identifier: NCT05490459.
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Desfibriladores , Cardioversión Eléctrica , Taquicardia Ventricular , Fibrilación Ventricular , Dispositivos Electrónicos Vestibles , Humanos , Masculino , Femenino , Fibrilación Ventricular/terapia , Fibrilación Ventricular/diagnóstico , Cardioversión Eléctrica/instrumentación , Cardioversión Eléctrica/efectos adversos , Persona de Mediana Edad , Taquicardia Ventricular/terapia , Taquicardia Ventricular/diagnóstico , Taquicardia Ventricular/fisiopatología , Estudios Prospectivos , Resultado del Tratamiento , Anciano , Diseño de Equipo , Adulto , Muerte Súbita Cardíaca/prevención & controlRESUMEN
AIMS: Ventricular tachycardia (VT) non-inducibility in response to programmed ventricular stimulation (PVS) is a widely used procedural endpoint for VT ablation despite inconclusive evidence with respect to clinical outcomes in high-risk patients. The aim is to determine the utility of acute post-ablation VT inducibility as a predictor of VT recurrence, mortality, or mortality equivalent in high-risk patients. METHODS AND RESULTS: We conducted a retrospective analysis of high-risk patients (defined as PAINESD > 17) who underwent scar-related VT ablation at our institution between July 2010 and July 2022. Patients' response to PVS (post-procedure) was categorized into three groups: Group A, no clinical VT or VT with cycle length > 240 ms inducible; Group B, only non-clinical VT with cycle length > 240 ms induced; and Group C, all other outcomes (including cases where no PVS was performed). The combined primary endpoint included death, durable left ventricular assist device placement, and cardiac transplant (Cox analysis). Ventricular tachycardia recurrence was considered a secondary endpoint (competing risk analysis). Of the 1677 VT ablation cases, 123 cases met the inclusion criteria for analysis. During a 19-month median follow-up time (interquartile range 4-43 months), 82 (66.7%) patients experienced the composite primary endpoint. There was no difference between Groups A and C with respect to the primary [hazard ratio (HR) = 1.21 (0.94-1.57), P = 0.145] or secondary [HR = 1.18 (0.91-1.54), P = 0.210] outcomes. These findings persisted after multivariate adjustments. The size of Group B (n = 13) did not permit meaningful statistical analysis. CONCLUSION: The results of post-ablation PVS do not significantly correlate with long-term outcomes in high-risk (PAINESD > 17) VT ablation patients.
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Ablación por Catéter , Cicatriz , Recurrencia , Taquicardia Ventricular , Humanos , Taquicardia Ventricular/fisiopatología , Taquicardia Ventricular/cirugía , Taquicardia Ventricular/etiología , Taquicardia Ventricular/diagnóstico , Masculino , Femenino , Estudios Retrospectivos , Persona de Mediana Edad , Cicatriz/fisiopatología , Cicatriz/etiología , Anciano , Medición de Riesgo , Resultado del Tratamiento , Factores de RiesgoRESUMEN
We present the case of a 60-year-old male patient who was admitted to our hospital after experiencing a syncopal episode. First ECGs showed sinus rhythm with polymorphic premature ventricular complexes and later ventricular tachycardia with a left bundle branch block morphology were recorded. Imaging with TEE and MRI revealed a space-occupying lesion in the left ventricle, which was ultimately identified as a rare cardiac metastasis of renal cell carcinoma. Treatment was initiated with monoclonal antibodies resulting in lesion regression. This case highlights the importance of comprehensive diagnostic in patients with history of malignancy.
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Carcinoma de Células Renales , Electrocardiografía , Neoplasias Cardíacas , Ventrículos Cardíacos , Neoplasias Renales , Taquicardia Ventricular , Humanos , Masculino , Persona de Mediana Edad , Neoplasias Cardíacas/secundario , Neoplasias Cardíacas/complicaciones , Neoplasias Cardíacas/diagnóstico por imagen , Taquicardia Ventricular/etiología , Taquicardia Ventricular/fisiopatología , Taquicardia Ventricular/diagnóstico , Neoplasias Renales/patología , Neoplasias Renales/diagnóstico por imagen , Ventrículos Cardíacos/diagnóstico por imagen , Carcinoma de Células Renales/secundario , Carcinoma de Células Renales/complicaciones , Carcinoma de Células Renales/diagnóstico por imagen , Imagen por Resonancia Magnética , Diagnóstico Diferencial , Ecocardiografía TransesofágicaRESUMEN
Importance: Sudden death and cardiac arrest frequently occur without explanation, even after a thorough clinical evaluation. Calcium release deficiency syndrome (CRDS), a life-threatening genetic arrhythmia syndrome, is undetectable with standard testing and leads to unexplained cardiac arrest. Objective: To explore the cardiac repolarization response on an electrocardiogram after brief tachycardia and a pause as a clinical diagnostic test for CRDS. Design, Setting, and Participants: An international, multicenter, case-control study including individual cases of CRDS, 3 patient control groups (individuals with suspected supraventricular tachycardia; survivors of unexplained cardiac arrest [UCA]; and individuals with genotype-positive catecholaminergic polymorphic ventricular tachycardia [CPVT]), and genetic mouse models (CRDS, wild type, and CPVT were used to define the cellular mechanism) conducted at 10 centers in 7 countries. Patient tracings were recorded between June 2005 and December 2023, and the analyses were performed from April 2023 to December 2023. Intervention: Brief tachycardia and a subsequent pause (either spontaneous or mediated through cardiac pacing). Main Outcomes and Measures: Change in QT interval and change in T-wave amplitude (defined as the difference between their absolute values on the postpause sinus beat and the last beat prior to tachycardia). Results: Among 10 case patients with CRDS, 45 control patients with suspected supraventricular tachycardia, 10 control patients who experienced UCA, and 3 control patients with genotype-positive CPVT, the median change in T-wave amplitude on the postpause sinus beat (after brief ventricular tachycardia at ≥150 beats/min) was higher in patients with CRDS (P < .001). The smallest change in T-wave amplitude was 0.250 mV for a CRDS case patient compared with the largest change in T-wave amplitude of 0.160 mV for a control patient, indicating 100% discrimination. Although the median change in QT interval was longer in CRDS cases (P = .002), an overlap between the cases and controls was present. The genetic mouse models recapitulated the findings observed in humans and suggested the repolarization response was secondary to a pathologically large systolic release of calcium from the sarcoplasmic reticulum. Conclusions and Relevance: There is a unique repolarization response on an electrocardiogram after provocation with brief tachycardia and a subsequent pause in CRDS cases and mouse models, which is absent from the controls. If these findings are confirmed in larger studies, this easy to perform maneuver may serve as an effective clinical diagnostic test for CRDS and become an important part of the evaluation of cardiac arrest.
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Electrocardiografía , Humanos , Ratones , Estudios de Casos y Controles , Masculino , Animales , Femenino , Adulto , Taquicardia Ventricular/diagnóstico , Taquicardia Ventricular/fisiopatología , Taquicardia Ventricular/etiología , Paro Cardíaco/etiología , Paro Cardíaco/diagnóstico , Calcio/metabolismo , Calcio/sangre , Taquicardia Supraventricular/diagnóstico , Taquicardia Supraventricular/fisiopatología , Taquicardia Supraventricular/etiología , Persona de Mediana Edad , Modelos Animales de Enfermedad , Arritmias Cardíacas/diagnóstico , Arritmias Cardíacas/etiología , Adolescente , Adulto Joven , Canal Liberador de Calcio Receptor de Rianodina/genéticaAsunto(s)
Taquicardia Ventricular , Humanos , Taquicardia Ventricular/fisiopatología , Taquicardia Ventricular/terapia , Taquicardia Ventricular/diagnóstico , Taquicardia Ventricular/etiología , Electrocardiografía , Desfibriladores Implantables/efectos adversos , Masculino , Dispositivos de Terapia de Resincronización Cardíaca/efectos adversos , Resultado del Tratamiento , Diagnóstico DiferencialAsunto(s)
Ablación por Catéter , Etanol , Taquicardia Ventricular , Humanos , Etanol/administración & dosificación , Taquicardia Ventricular/cirugía , Taquicardia Ventricular/fisiopatología , Taquicardia Ventricular/diagnóstico , Ablación por Catéter/métodos , Resultado del Tratamiento , Ventrículos Cardíacos/cirugía , Ventrículos Cardíacos/fisiopatología , Masculino , Técnicas Electrofisiológicas Cardíacas , Catéteres CardíacosAsunto(s)
Potenciales de Acción , Ablación por Catéter , Cicatriz , Sistema de Conducción Cardíaco , Taquicardia Ventricular , Ablación por Catéter/métodos , Taquicardia Ventricular/fisiopatología , Taquicardia Ventricular/cirugía , Taquicardia Ventricular/diagnóstico , Humanos , Cicatriz/fisiopatología , Cicatriz/etiología , Sistema de Conducción Cardíaco/fisiopatología , Sistema de Conducción Cardíaco/cirugía , Técnicas Electrofisiológicas Cardíacas , Masculino , Frecuencia Cardíaca , Resultado del Tratamiento , Femenino , Valor Predictivo de las Pruebas , Factores de Tiempo , Persona de Mediana EdadRESUMEN
Special Operations Servicemembers presenting with palpitations, pre-syncope, or exertional syncope during rigorous physical training are often experiencing a benign condition; however, life-threatening etiologies should be considered. We describe a 43-year-old Special Operator who presented to his medics during selection physical assessment testing with palpitations and lightheadedness, with a subsequent workup revealing arrhythmogenic right ventricular cardiomyopathy (ARVC). His initial electrocardiogram was unremarkable without characteristic ARVC changes. Outpatient evaluation with ambulatory cardiac monitoring recorded numerous episodes of non-sustained ventricular tachycardia. Transthoracic echocardiography demonstrated findings concerning for ARVC, with subsequent cardiac MRI confirming the diagnosis via the 2020 Padua criteria. Management includes activity modification, class III anti-arrhythmic medications, and possible placement of an implantable cardioverter defibrillator to prevent sudden cardiac death. This case demonstrates the importance of maintaining high clinical suspicion for rare diagnoses that present with exertional palpitations, such as arrhythmogenic right ventricular cardiomyopathy, in even our fittest Special Operators.