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BACKGROUND: Imaging evaluation of arrhythmogenic right ventricular cardiomyopathy (ARVC) remains challenging. Myocardial strain assessment by echocardiography is an increasingly utilized technique for detecting subclinical left ventricular (LV) and right ventricular (RV) dysfunction. We aimed to evaluate the diagnostic and prognostic utility of LV and RV strain in ARVC. METHODS: Patients with suspected ARVC (n = 109) from a multicenter registry were clinically phenotyped using the 2010 ARVC Revised Task Force Criteria and underwent baseline strain echocardiography. Diagnostic performance of LV and RV strain was evaluated using the area under the receiver operating characteristic curve analysis against the 2010 ARVC Revised Task Force Criteria, and the prognostic value was assessed using the Kaplan-Meier analysis. RESULTS: Mean age was 45.3±14.7 years, and 48% of patients were female. Estimation of RV strain was feasible in 99/109 (91%), and LV strain was feasible in 85/109 (78%) patients. ARVC prevalence by 2010 ARVC Revised Task Force Criteria is 91/109 (83%) and 83/99 (84%) in those with RV strain measurements. RV global longitudinal strain and RV free wall strain had diagnostic area under the receiver operating characteristic curve of 0.76 and 0.77, respectively (both P<0.001; difference NS). Abnormal RV global longitudinal strain phenotype (RV global longitudinal strain > -17.9%) and RV free wall strain phenotype (RV free wall strain > -21.2%) were identified in 41/69 (59%) and 56/69 (81%) of subjects, respectively, who were not identified by conventional echocardiographic criteria but still met the overall 2010 ARVC Revised Task Force Criteria for ARVC. LV global longitudinal strain did not add diagnostic value but was prognostic for composite end points of death, heart transplantation, or ventricular arrhythmia (log-rank P=0.04). CONCLUSIONS: In a prospective, multicenter registry of ARVC, RV strain assessment added diagnostic value to current echocardiographic criteria by identifying patients who are missed by current echocardiographic criteria yet still fulfill the diagnosis of ARVC. LV strain, by contrast, did not add incremental diagnostic value but was prognostic for identification of high-risk patients.
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Displasia Ventricular Derecha Arritmogénica , Disfunción Ventricular Derecha , Humanos , Femenino , Adulto , Persona de Mediana Edad , Masculino , Displasia Ventricular Derecha Arritmogénica/diagnóstico por imagen , Displasia Ventricular Derecha Arritmogénica/genética , Estudios Prospectivos , Función Ventricular Derecha , Miocardio , Disfunción Ventricular Derecha/diagnóstico por imagen , Disfunción Ventricular Derecha/etiología , Sistema de RegistrosAsunto(s)
Displasia Ventricular Derecha Arritmogénica , Bisoprolol/administración & dosificación , Desmogleína 2/genética , Electrocardiografía/métodos , Flecainida/administración & dosificación , Ventrículos Cardíacos , Taquicardia Ventricular , Adulto , Antiarrítmicos/administración & dosificación , Displasia Ventricular Derecha Arritmogénica/genética , Displasia Ventricular Derecha Arritmogénica/fisiopatología , Displasia Ventricular Derecha Arritmogénica/terapia , Desfibriladores Implantables , Cardioversión Eléctrica/métodos , Ventrículos Cardíacos/diagnóstico por imagen , Ventrículos Cardíacos/patología , Ventrículos Cardíacos/fisiopatología , Humanos , Imagen por Resonancia Cinemagnética/métodos , Masculino , Mutación Missense , Taquicardia Ventricular/diagnóstico , Taquicardia Ventricular/etiología , Taquicardia Ventricular/terapia , Resultado del TratamientoRESUMEN
Advances in technology have reshaped the practice of medicine. These changes have greatly benefited our patients. However, in the setting of these advances, the importance of basic clinical tools is more pertinent than ever. Despite the growing reliance on technology, the physical exam remains valuable and cost effective, often enabling the well-trained clinician to arrive at the diagnosis, rapidly and accurately. The physical exam must not become a relic of a distant past. We aim to investigate current competency and proficiency, proposals for change in teaching curriculums, and the relationship with technology such as hand-held echocardiography. A skillful exam provides both emotional and intellectual satisfaction. It may be a lost art but it is well worth the effort to restore.
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Competencia Clínica , Técnicas de Diagnóstico Cardiovascular , Educación Médica , Examen Físico , Curriculum , Ecocardiografía , Humanos , Sistemas de Atención de PuntoRESUMEN
Excellence in recording and interpretation of electrocardiogram (ECG) is a necessity for optimal electrocardiography. This includes data to properly interpret the ECG, including data on age, gender, cardiovascular diagnosis, medications, abnormal laboratory findings (eg, data on electrolytes), and the indications for the electrocardiogram. The ECG needs to be performed by a qualified technician and interpreted by an experienced physician.
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Electrocardiografía/métodos , Electrocardiografía/normas , Personal de Salud/educación , Personal de Salud/normas , Competencia Clínica , Humanos , Estándares de ReferenciaRESUMEN
Arrhythmogenic cardiomyopathy (ACM) is an arrhythmogenic disorder of the myocardium not secondary to ischemic, hypertensive, or valvular heart disease. ACM incorporates a broad spectrum of genetic, systemic, infectious, and inflammatory disorders. This designation includes, but is not limited to, arrhythmogenic right/left ventricular cardiomyopathy, cardiac amyloidosis, sarcoidosis, Chagas disease, and left ventricular noncompaction. The ACM phenotype overlaps with other cardiomyopathies, particularly dilated cardiomyopathy with arrhythmia presentation that may be associated with ventricular dilatation and/or impaired systolic function. This expert consensus statement provides the clinician with guidance on evaluation and management of ACM and includes clinically relevant information on genetics and disease mechanisms. PICO questions were utilized to evaluate contemporary evidence and provide clinical guidance related to exercise in arrhythmogenic right ventricular cardiomyopathy. Recommendations were developed and approved by an expert writing group, after a systematic literature search with evidence tables, and discussion of their own clinical experience, to present the current knowledge in the field. Each recommendation is presented using the Class of Recommendation and Level of Evidence system formulated by the American College of Cardiology and the American Heart Association and is accompanied by references and explanatory text to provide essential context. The ongoing recognition of the genetic basis of ACM provides the opportunity to examine the diverse triggers and potential common pathway for the development of disease and arrhythmia.
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Displasia Ventricular Derecha Arritmogénica/diagnóstico , Displasia Ventricular Derecha Arritmogénica/terapia , Consenso , Humanos , Medición de RiesgoAsunto(s)
Aniversarios y Eventos Especiales , Arritmias Cardíacas/cirugía , Ablación por Radiofrecuencia/historia , Arritmias Cardíacas/diagnóstico por imagen , Ablación por Catéter/historia , Ablación por Catéter/métodos , Femenino , Historia del Siglo XX , Humanos , Masculino , Sensibilidad y Especificidad , Estados UnidosRESUMEN
Arrhythmogenic cardiomyopathy (ACM) is an inherited arrhythmia syndrome characterized by severe structural and electrical cardiac phenotypes, including myocardial fibrofatty replacement and sudden cardiac death. Clinical management of ACM is largely palliative, owing to an absence of therapies that target its underlying pathophysiology, which stems partially from our limited insight into the condition. Following identification of deceased ACM probands possessing ANK2 rare variants and evidence of ankyrin-B loss of function on cardiac tissue analysis, an ANK2 mouse model was found to develop dramatic structural abnormalities reflective of human ACM, including biventricular dilation, reduced ejection fraction, cardiac fibrosis, and premature death. Desmosomal structure and function appeared preserved in diseased human and murine specimens in the presence of markedly abnormal ß-catenin expression and patterning, leading to identification of a previously unknown interaction between ankyrin-B and ß-catenin. A pharmacological activator of the WNT/ß-catenin pathway, SB-216763, successfully prevented and partially reversed the murine ACM phenotypes. Our findings introduce what we believe to be a new pathway for ACM, a role of ankyrin-B in cardiac structure and signaling, a molecular link between ankyrin-B and ß-catenin, and evidence for targeted activation of the WNT/ß-catenin pathway as a potential treatment for this disease.
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Ancirinas , Displasia Ventricular Derecha Arritmogénica , Miocardio , Vía de Señalización Wnt , Animales , Ancirinas/genética , Ancirinas/metabolismo , Displasia Ventricular Derecha Arritmogénica/genética , Displasia Ventricular Derecha Arritmogénica/metabolismo , Displasia Ventricular Derecha Arritmogénica/patología , Modelos Animales de Enfermedad , Femenino , Humanos , Indoles/farmacología , Masculino , Maleimidas/farmacología , Ratones , Ratones Noqueados , Miocardio/metabolismo , Miocardio/patología , beta Catenina/genética , beta Catenina/metabolismoRESUMEN
Arrhythmogenic cardiomyopathy (ACM) is an arrhythmogenic disorder of the myocardium not secondary to ischemic, hypertensive, or valvular heart disease. ACM incorporates a broad spectrum of genetic, systemic, infectious, and inflammatory disorders. This designation includes, but is not limited to, arrhythmogenic right/left ventricular cardiomyopathy, cardiac amyloidosis, sarcoidosis, Chagas disease, and left ventricular noncompaction. The ACM phenotype overlaps with other cardiomyopathies, particularly dilated cardiomyopathy with arrhythmia presentation that may be associated with ventricular dilatation and/or impaired systolic function. This expert consensus statement provides the clinician with guidance on evaluation and management of ACM and includes clinically relevant information on genetics and disease mechanisms. PICO questions were utilized to evaluate contemporary evidence and provide clinical guidance related to exercise in arrhythmogenic right ventricular cardiomyopathy. Recommendations were developed and approved by an expert writing group, after a systematic literature search with evidence tables, and discussion of their own clinical experience, to present the current knowledge in the field. Each recommendation is presented using the Class of Recommendation and Level of Evidence system formulated by the American College of Cardiology and the American Heart Association and is accompanied by references and explanatory text to provide essential context. The ongoing recognition of the genetic basis of ACM provides the opportunity to examine the diverse triggers and potential common pathway for the development of disease and arrhythmia.
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Displasia Ventricular Derecha Arritmogénica/diagnóstico , Displasia Ventricular Derecha Arritmogénica/terapia , Consenso , Humanos , Medición de RiesgoRESUMEN
The epsilon wave of the electrocardiogram (ECG) together with fragmented QRS (fQRS), the terminal conduction delay, incomplete right bundle branch block (IRBBB) and complete/advanced RBBB (CRBBB) of peripheral origin are part of a spectrum of ventricular depolarization abnormalities of arrhythmogenic cardiomyopathy(AC). Although the epsilon wave is considered a major diagnostic criterion for AC since 2010 (AC Task Force Criteria), its diagnostic value is limited because it is a sign of the later stage of the disease. It would be more appropriate to say that the epsilon wave is a "hallmark" of AC, but is of low diagnostic sensitivity. Although the epsilon wave has high specificity for AC, it can be present in other pathological conditions. In this update we will cover the nomenclature, association with disease states and electrocardiographic aspects of the epsilon wave.
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OBJECTIVE: Changes in heart rate variability (HRV) associated with breathing (respiratory sinus arrhythmia) are known to be parasympathetically (vagally) mediated when the breathing rate is within the typical frequency range (9-24 breaths per minute [bpm]; high-frequency HRV). Slow yogic breathing occurs at rates below this range and increases low-frequency HRV power, which may additionally reflect a significant sympathetic component. Yogic breathing techniques are hypothesized to confer health benefits by increasing cardiac vagal control, but increases in low-frequency HRV power cannot unambiguously distinguish sympathetic from parasympathetic contributions. The aim of this study was to investigate the autonomic origins of changes in low-frequency HRV power due to slow-paced breathing. METHODS: Six healthy young adults completed slow-paced breathing with a cadence derived from yogic breathing patterns. The paced breathing took place under conditions of sympathetic blockade, parasympathetic (vagal) blockade, and placebo. HRV spectral power was compared under 11 breathing rates during each session, in counterbalanced order with frequencies spanning the low-frequency range (4-9 bpm). RESULTS: HRV power across the low-frequency range (4-9 bpm) was nearly eliminated (p = .016) by parasympathetic blockade (mean (SD) spectral power at breathing frequency = 4.1 (2.1)) compared with placebo (69.5 (8.1)). In contrast, spectral power during sympathetic blockade 70.2 (9.1) and placebo (69.5 (8.1)) was statistically indistinguishable (p = .671). CONCLUSIONS: These findings clarify the interpretation of changes in HRV that occur during slow-paced breathing by showing that changes in low-frequency power under these conditions are almost entirely vagally mediated. Slow-paced breathing is an effective tool for cardiac vagal activation.
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Sistema Nervioso Autónomo/fisiología , Frecuencia Cardíaca/fisiología , Frecuencia Respiratoria/fisiología , Nervio Vago/fisiología , Yoga , Adolescente , Antagonistas de Receptores Adrenérgicos beta 1/farmacología , Adulto , Sistema Nervioso Autónomo/efectos de los fármacos , Electrocardiografía , Femenino , Humanos , Masculino , Antagonistas Muscarínicos/farmacología , Sistema Nervioso Parasimpático/efectos de los fármacos , Sistema Nervioso Parasimpático/fisiología , Frecuencia Respiratoria/efectos de los fármacos , Sistema Nervioso Simpático/efectos de los fármacos , Sistema Nervioso Simpático/fisiología , Nervio Vago/efectos de los fármacos , Adulto JovenRESUMEN
OBJECTIVES: To verify accurate placement of the precordial ECG leads by identifying the 4th and 5th intercostal spaces as a function of the length of the sternum. This should decrease the percentage of lead misplacement leading to misdiagnoses. METHODS: The population consisted of patients and healthy volunteers. The proposed method compared palpation of the 4th and 5th intercostal spaces to a percentile of the sternal length. Location of the 4th and 5th intercostal space using a simple device was evaluated to assist in proper placement of the precordial leads to obtain accurate diagnosis. RESULTS: The location of the 4th and 5th intercostal space is related to the length of the sternum. It is 77% of the sternal length that measures 15cm for the 4th intercostal space. The position of the V1 and V2 electrodes decreases to 57% when the sternal length is 26cm. Similar data was obtained to locate the 5th intercostal space with proper position of V4-V6 electrodes. Tables are provided to facilitate this process. An instrument was designed to measure the 4th and 5th intercostal space as a function of the sternal length. CONCLUSIONS: The location of the 4th and 5th intercostal space is identified based on the length of the sternum.
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Electrocardiografía/métodos , Costillas/anatomía & histología , Esternón/anatomía & histología , Adulto , Voluntarios Sanos , Humanos , Apófisis Xifoides/anatomía & histologíaRESUMEN
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy characterized by ventricular arrhythmias and an increased risk of sudden cardiac death. Although structural abnormalities of the right ventricle predominate, it is well recognized that left ventricular involvement is common, particularly in advanced disease, and that left-dominant forms occur. The pathological characteristic of ARVC is myocyte loss with fibrofatty replacement. Since the first detailed clinical description of the disorder in 1982, significant advances have been made in understanding this disease. Once the diagnosis of ARVC is established, the single most important clinical decision is whether a particular patient's sudden cardiac death risk is sufficient to justify placement of an implantable cardioverter-defibrillator. The importance of this decision reflects the fact that ARVC is a common cause of sudden death in young people and that sudden death may be the first manifestation of the disease. This decision is particularly important because these are often young patients who are expected to live for many years. Although an implantable cardioverter-defibrillator can save lives in individuals with this disease, it is also well recognized that implantable cardioverter-defibrillator therapy is associated with both short- and long-term complications. Decisions about the placement of an implantable cardioverter-defibrillator are based on an estimate of a patient's risk of sudden cardiac death, as well as their preferences and values. The primary purpose of this article is to provide a review of the literature that concerns risk stratification in patients with ARVC and to place this literature in the framework of the 3 authors' considerable lifetime experiences in caring for patients with ARVC. The most important parameters to consider when determining arrhythmic risk include electric instability, including the frequency of premature ventricular contractions and sustained ventricular arrhythmia; proband status; extent of structural disease; cardiac syncope; male sex; the presence of multiple mutations or a mutation in TMEM43; and the patient's willingness to restrict exercise and to eliminate participation in competitive or endurance exercise.
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Arritmias Cardíacas/etiología , Displasia Ventricular Derecha Arritmogénica/complicaciones , Muerte Súbita/etiología , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Arritmias Cardíacas/mortalidad , Arritmias Cardíacas/fisiopatología , Arritmias Cardíacas/terapia , Displasia Ventricular Derecha Arritmogénica/mortalidad , Displasia Ventricular Derecha Arritmogénica/fisiopatología , Displasia Ventricular Derecha Arritmogénica/terapia , Niño , Preescolar , Muerte Súbita/prevención & control , Desfibriladores Implantables , Cardioversión Eléctrica/instrumentación , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Persona de Mediana Edad , Selección de Paciente , Medición de Riesgo , Factores de Riesgo , Resultado del Tratamiento , Adulto JovenRESUMEN
INTRODUCTION: We have observed electrocardiographic (ECG) changes primarily in women during tilt table testing. METHODS: We reviewed 12 lead ECGs during tilt studies between 2012 and 2016 for changes in ST segments and T waves during tilt table testing. Patients with distinctly abnormal baseline ECGs were excluded. RESULTS: Of the 180 tilt studies, 117 (65%) were in women. There were 32 patients with ECG changes during tilting. Of these, 28 (87.5%) were in women with an average age of 45years. None had a history of CAD or exertional chest pain. Echocardiograms were available in 21 of the 28 women with tilt induced ECG changes and all were normal. ECG changes during tilt table testing were found in 4/64 (6.25%) of men. The occurrence of ST-T wave changes during tilt testing was significantly higher among women compared to men, with a p value of 0.008. Of the 28 women with ECG changes during tilt, 11 had T wave inversions alone. ST segment depression alone was noted in 7 women. There were 10 women who had both ST segment depression and T wave inversions. Changes occurred immediately upon tilting in 6. In the remaining, they occurred at an average of 4.8±4min after tilting. The slight increase in heart rate in patients with ECG changes was similar to that in the patients without new ECG changes. The ECG changes were not related to the presence of syncope. CONCLUSIONS: ECG changes during the testing was observed at a relatively high incidence primarily in women. The clinical significance of these repolarization changes during tilt testing is unknown. These ECG changes during tilt testing may correlate with the high incidence of false positive ECGs in women during exercise testing but do not necessarily indicate the presence of ischemic coronary disease. Additional research is needed to explain this phenomenon.
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Sistema de Conducción Cardíaco/fisiopatología , Síncope/fisiopatología , Pruebas de Mesa Inclinada , Ecocardiografía , Electrocardiografía , Femenino , Hemodinámica/fisiología , Humanos , Incidencia , Masculino , Persona de Mediana Edad , Valor Predictivo de las Pruebas , Estudios RetrospectivosRESUMEN
OBJECTIVES: The primary objective of this study is risk stratification of patients with arrhythmic right ventricular cardiomyopathy (ARVC). BACKGROUND: There is a need to identify those who need an automatic implantable defibrillator (ICD) to prevent sudden death. METHODS: This is an analysis of 88 patients with ARVC from three centers who were not treated with an ICD. RESULTS: Risk factors for subsequent arrhythmic deaths were pre-enrollment sustained or nonsustained ventricular tachycardia (VT) and decreased left ventricular function. CONCLUSION: These factors serve as proposed guidelines for implantation of an ICD in patients with ARVC to prevent sudden death.