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BACKGROUND: Electrocardiographic (ECG) findings in arrhythmogenic left ventricular cardiomyopathy (ALVC) are limited to small case series. OBJECTIVES: This study aimed to analyze the ECG characteristics of ALVC patients and to correlate ECG with cardiac magnetic resonance and genotype data. METHODS: We reviewed data of 54 consecutive ALVC patients (32 men, age 39 ± 15 years) and compared them with 84 healthy controls with normal cardiac magnetic resonance. RESULTS: T-wave inversion was often noted (57.4%), particularly in the inferior and lateral leads. Low QRS voltages in limb leads were observed in 22.2% of patients. The following novel ECG findings were identified: left posterior fascicular block (LPFB) (20.4%), pathological Q waves (33.3%), and a prominent R-wave in V1 with a R/S ratio ≥0.5 (24.1%). The QRS voltages were lower in ALVC compared with controls, particularly in lead I and II. At receiver-operating characteristic analysis, the sum of the R-wave in I to II ≤8 mm (AUC: 0.909; P < 0.0001) and S-wave in V1 plus R-wave in V6 ≤12 mm (AUC: 0.784; P < 0.0001) effectively discriminated ALVC patients from controls. It is noteworthy that 4 of the 8 patients with an apparently normal ECG were recognized by these new signs. Transmural late gadolinium enhancement was associated to LPFB, a R/S ratio ≥0.5 in V1, and inferolateral T-wave inversion, and a ringlike pattern correlated to fragmented QRS, SV1+RV6 ≤12 mm, low QRS voltage, and desmoplakin alterations. CONCLUSIONS: Pathological Q waves, LPFB, and a prominent R-wave in V1 were common ECG signs in ALVC. An R-wave sum in I to II ≤8 mm and SV1+RV6 ≤12 mm were specific findings for ALVC phenotypes compared with controls.
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Cardiomiopatías , Medios de Contraste , Masculino , Humanos , Adulto Joven , Adulto , Persona de Mediana Edad , Gadolinio , Electrocardiografía , Arritmias Cardíacas , Bloqueo de RamaRESUMEN
AIMS: Left ventricular scar is an arrhythmic substrate that may be missed by echocardiography and diagnosed only by cardiac magnetic resonance (CMR), which is a time-consuming and expensive imaging modality. Premature ventricular complexes (PVCs) with a right-bundle-branch-block (RBBB) pattern are independent predictors of late gadolinium enhancement (LGE) but their positive predictive value is low. We studied which electrocardiographic features of PVCs with an RBBB pattern are associated with a higher probability of the absence of an underlying LGE. METHODS: The study included 121 athletes (36 ± 16 years; 48.8% men) with monomorphic PVCs with an RBBB configuration and normal standard clinical investigations who underwent CMR. LGE was identified in 35 patients (29%), predominantly in those with PVCs with a superior/intermediate axis (SA-IntA) compared to inferior axis (IA) (38% vs. 10%, P = 0.002). Among patients with SA-IntA morphology, the contemporary presence of qR pattern in lead aVR and V1 was exclusively found in patients without LGE at CMR (51.0% vs. 0%, P < 0.0001). Among patients with IA, the absence of LGE correlated to a narrow ectopic QRS (145 ± 16 vs. 184 ± 27â msec, P < 0.001). CONCLUSIONS: Among athletes with apparently idiopathic PVCs with a RBBB configuration, the presence of a concealed LGE at CMR was documented in 29% of cases, mostly in those with a SA-IntA. In our experience, the contemporary presence of qR pattern in lead aVR and V1 in PVCs with RBBB/SA-IntA morphology or, on the other hand, a relatively narrow QRS in PVCs with an IA, predicted absence of LGE.
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Cicatriz , Complejos Prematuros Ventriculares , Masculino , Humanos , Femenino , Medios de Contraste , Gadolinio , Ventrículos Cardíacos/diagnóstico por imagen , Electrocardiografía/métodos , Bloqueo de Rama , AtletasRESUMEN
Cardiomyopathies are a heterogeneous group of pathologies characterized by structural and functional alterations of the heart. Recent technological advances in cardiovascular imaging offer an opportunity for deep phenotypic and etiological definition. Electrocardiogram (ECG) is the first-line diagnostic tool in the evaluation of both asymptomatic and symptomatic individuals. Some electrocardiographic signs are pathognomonic or fall within validated diagnostic criteria of individual cardiomyopathy such as the inverted T waves in right precordial leads (V1-V3) or beyond in individuals with complete pubertal development in the absence of complete right bundle branch block for the diagnosis of arrhythmogenic cardiomyopathy of the right ventricle (ARVC) or the presence of low voltages typically seen in more than 60% of patients with amyloidosis. Most other electrocardiographic findings such as the presence of depolarization changes including QRS fragmentation, the presence of epsilon wave, the presence of reduced or increased voltages as well as alterations in the repolarization phase including the negative T waves in the lateral leads, or the profound inversion of the T waves or downsloping of the ST tract are more non-specific signs which can however raise the clinical suspicion of cardiomyopathy in order to initiate a diagnostic procedure especially using imaging techniques for diagnostic confirmation. Such electrocardiographic alterations not only have a counterpart in imaging investigations such as evidence of late gadolinium enhancement on magnetic resonance imaging, but may also have an important prognostic value once a definite diagnosis has been made. In addition, the presence of electrical stimulus conduction disturbances or advanced atrioventricular blocks that can be seen especially in conditions such as cardiac amyloidosis or sarcoidosis, or the presence of left bundle branch block or posterior fascicular block in dilated or arrhythmogenic left ventricular cardiomyopathies are recognized as a possible expression of advanced pathology. Similarly, the presence of ventricular arrhythmias with typical patterns such as non-sustained or sustained ventricular tachycardia of LBBB morphology in ARVC or non-sustained or sustained ventricular tachycardia with an RBBB morphology (excluding the "fascicular pattern") in arrhythmogenic left ventricle cardiomyopathy could have a significant impact on the course of each disease. It is therefore clear that a learned and careful interpretation of ECG features can raise suspicion of the presence of a cardiomyopathy, identify diagnostic "red flags" useful for orienting the diagnosis toward specific forms, and provide useful tools for risk stratification. The purpose of this review is to emphasize the important role of the ECG in the diagnostic workup, describing the main ECG findings of different cardiomyopathies.
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BACKGROUND: The 2 sarcomere genes most commonly associated with hypertrophic cardiomyopathy (HCM), MYBPC3 (myosin-binding protein C3) and MYH7 (ß-myosin heavy chain), are indistinguishable at presentation, and genotype-phenotype correlations have been elusive. Based on molecular and pathophysiological differences, however, it is plausible to hypothesize a different behavior in myocardial performance, impacting lifetime changes in left ventricular (LV) function. METHODS: We reviewed the initial and final echocardiograms of 402 consecutive HCM patients with pathogenic or likely pathogenic MYBPC3 (n=251) or MYH7 (n=151) mutations, followed over 9±8 years. RESULTS: At presentation, MYBPC3 patients were less frequently obstructive (15% versus 26%; P=0.005) and had lower LV ejection fraction compared with MYH7 (66±8% versus 68±8%, respectively; P=0.03). Both HCM patients harboring MYBPC3 and MYH7 mutations exhibited a small but significant decline in LV systolic function during follow-up; however, new onset of severe LV systolic dysfunction (LV ejection fraction, <50%) was greater among MYBPC3 patients (15% versus 5% among MYH7; P=0.013). Prevalence of grade II/III diastolic dysfunction at final evaluation was comparable between MYBPC3 and MYH7 patients (P=0.509). In a Cox multivariable analysis, MYBPC3-positive status (hazard ratio, 2.53 [95% CI, 1.09-5.82]; P=0.029), age (hazard ratio, 1.03 [95% CI, 1.00-1.06]; P=0.027), and atrial fibrillation (hazard ratio, 2.39 [95% CI, 1.14-5.05]; P=0.020) were independent predictors of severe systolic dysfunction. No statistically significant differences occurred with regard to incidence of atrial fibrillation, heart failure, appropriate implanted cardioverter defibrillator shock, or cardiovascular death. CONCLUSIONS: MYBPC3-related HCM showed increased long-term prevalence of systolic dysfunction compared with MYH7, in spite of similar outcome. Such observations suggest different pathophysiology of clinical progression in the 2 subsets and may prove relevant for understanding of genotype-phenotype correlations in HCM.
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Fibrilación Atrial , Cardiomiopatía Hipertrófica , Humanos , Prevalencia , Fenotipo , Cardiomiopatía Hipertrófica/epidemiología , Cardiomiopatía Hipertrófica/genética , Mutación , Proteínas del Citoesqueleto , Cadenas Pesadas de Miosina/genética , Miosinas Cardíacas/genéticaRESUMEN
Patients with clinically established atherosclerotic cardiovascular disease are at a very high risk of recurrent cardiovascular events. An adequate management of risk factors and the implementation of healthy behaviours significantly decrease the risk of unfavourable clinical outcomes and future cardiovascular events, including death. Patients discharged after an acute coronary syndrome should be managed according to their individual risk level in order to ensure the appropriate treatment. Nevertheless, care pathways should also take into consideration the available resources and the logistical/structural aspects. In this setting, cardiac rehabilitation is prosed as a multidisciplinary approach to improving daily function and reducing cardiovascular risk factors. The organization of a network with the involvement of different medical and non-medical figures is essential to ensure successful outcomes and expected cost-effectiveness.
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This article summarizes the main electrocardiogram (ECG) findings in dilated cardiomyopathy (DCM) patients. Recent reports are described in the great 'pot' of DCM peculiar ECG patterns that are typical of specific forms of DCM. Patients with late gadolinium enhancement on CMR, who are at greatest arrhythmic risk, have also distinctive ECG features. Future studies in large DCM populations should evaluate the diagnostic and prognostic value of the ECG.
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Fabry disease (FD) is a rare X-linked inherited lysosomal storage disorder caused by deficient a-galactosidase A activity that leads to an accumulation of glycolipids, mainly globotriaosylceramide (Gb3) and globotriaosylsphingosine, in affected tissues, including the heart. Cardiovascular involvement usually manifests as left ventricular hypertrophy (LVH), myocardial fibrosis, heart failure, and arrhythmias, which limit the quality of life and represent the most common causes of death. Following the introduction of enzyme replacement therapy, early diagnosis and treatment have become essential in slowing down the disease progression and preventing major cardiac complications. Recent advances in the understanding of FD pathophysiology suggest that in addition to Gb3 accumulation, other mechanisms contribute to the development of cardiac damage. FD cardiomyopathy is characterized by an earlier stage of glycosphingolipid accumulation and a later one of hypertrophy. Morphological and functional aspects are not specific in the echocardiographic evaluation of Anderson-Fabry disease. Cardiac magnetic resonance with tissue characterization capability is an accurate technique for the differential diagnosis of LVH. Progress in imaging techniques has improved the diagnosis and staging of FD-related cardiac disease: a decreased myocardial T1 value is specific of FD. Late gadolinium enhancement is typical of the later stage of cardiac involvement but as in other cardiomyopathy is also valuable to predict the outcome and cardiac response to therapy.
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Criteria for diagnosis of arrhythmogenic cardiomyopathy (ACM) were first proposed in 1994 and subsequently revised in 2010 and in 2020 by an international task force. According to the last consensus of 2020, ACM is defined as a heart muscle disease affecting right ventricle, left ventricle or both, whose principal pathologic feature is fibrofatty myocardial replacement that impairs systolic ventricular function and predisposes to lethal ventricular arrhythmias. ECG findings not only could help to early recognize affected patients but also could identify the ones with maximum risk of ventricular arrhythmias and sudden cardiac death.
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AIMS: Low QRS voltages (LQRSV) are an unexpected finding in left ventricular hypertrophy, i.e. hypertrophic cardiomyopathy (HCM) or athlete's heart. METHODS AND RESULTS: Prevalence and clinical correlates of LQRSV were investigated in 197 consecutive HCM patients, aged 58 ± 13 years and comparatively in 771 Olympic athletes, aged 23 ± 4. Clinical characterization included family/personal history, symptoms, New York Heart Association (NYHA) functional class, electrocardiographic pattern, ventricular arrhythmias, and cardiac magnetic resonance (CMR). Twenty-two (11%) of HCM and 18 (2.3%) of athletes presented LQRSV. At initial evaluation, in HCM, LQRSV showed no differences vs. non-LQRSV for functional class (90% vs. 91%, in Classes I and II; P = 0.983), symptoms (27% vs. 18%; P = 0.478), and ventricular arrhythmias (40% vs. 39%; P = 857) but showed larger extent of late gadolinium enhancement (LGE) at CMR (4.1 ± 1.5 vs. 1.5 ± 0.7 affected segments; P < 0.001). In athletes, LQRSV was associated with larger prevalence of inverted T-waves (22% vs. 9%; P < 0.001) and ventricular arrhythmias (28% vs. 8%; P = 0.005). In one LQRSV athlete, arrhythmogenic cardiomyopathy was identified. Over 4.5 ± 2.6-year follow-up, presence of LQRSV in HCM was associated with larger incidence of functional deterioration (31% vs. 14%; P = 0.038), stroke (22% vs. 6%; P = 0.008), and implantable cardioverter defibrillator (ICD) implant (27% vs. 10%; P = 0.015). No clinical events occurred in LQRSV athletes without initial evidence of cardiac disease. CONCLUSION: LQRSV are relatively common (11%) in HCM and have clinical relevance, being predictive over a medium term for a worsening functional class, incidence of stroke, and ICD implant. Instead, LQRSV are rare (2.3%) in athletes but may occasionally be a marker that raises suspicion for underlying cardiac disease at risk.
In the present investigation, we sought to assess prevalence and clinical correlates of LQRSV in 197 consecutive HCM patients and, comparatively, in 771 Olympic athletes. Twenty-two (11%) of HCM presented LQRSV. At initial evaluation, LQRSV patients showed no differences vs. non-LQRSV for functional class (90% vs. 91%, in Classes I and II; P = 0.983), symptoms (27% vs. 18%; P = 0.478), and ventricular arrhythmias (40% vs. 39%; P = 857) but showed larger extent of LGE at CMR (4.1 ± 1.5 vs. 1.5 ± 0.7 affected segments; P < 0.001). Over 4.5 ± 2.6-year follow-up, presence of LQRSV was associated with larger incidence of functional class deterioration (31% vs. 14%; P = 0.038), stroke (22% vs. 6%; P = 0.008), and ICD implant (27% vs. 10%; P = 0.015).Eighteen (2.3%) of athletes presented LQRSV. In athletes, LQRSV was associated with larger prevalence of inverted T-waves (22% vs. 9%; P < 0.001) and ventricular arrhythmias (28% vs. 8%; P = 0.005). In one LQRSV athlete, arrhythmogenic cardiomyopathy was identified.In conclusion, LQRSV are relatively common (11%) in HCM and have clinical relevance, being predictive over a medium term for a worsening functional class, incidence of stroke, and ICD implant. Instead, LQRSV are rare (2.3%) in athletes but may be a marker that raises suspicion for underlying cardiac disease at risk.
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Cardiomiopatía Hipertrófica , Accidente Cerebrovascular , Humanos , Hipertrofia Ventricular Izquierda/diagnóstico por imagen , Hipertrofia Ventricular Izquierda/epidemiología , Medios de Contraste , Gadolinio , Cardiomiopatía Hipertrófica/diagnóstico , Arritmias Cardíacas/diagnóstico , Arritmias Cardíacas/epidemiología , Arritmias Cardíacas/terapiaRESUMEN
BACKGROUND: Left bundle branch area pacing (LBBAP) is an emerging technique to achieve cardiac resynchronization therapy (CRT), but its feasibility and safety in elderly patients with heart failure with reduced ejection fraction and left bundle branch block is hardly investigated. METHODS: We enrolled consecutive patients with an indication for CRT comparing pacing parameters and complication rates of LBBAP-CRT in elderly patients (≥ 75 years) versus younger patients (< 75 years) over a 6-month follow-up. RESULTS: LBBAP was successful in 55/60 enrolled patients (92%), among which 25(45%) were elderly. In both groups, LBBAP significantly reduced the QRS duration (elderly group: 168 ± 15 ms to 136 ± 12 ms, p < 0.0001; younger group: 166 ± 14 ms to 134 ± 11 ms, p < 0.0001) and improved LVEF (elderly group: 28 ± 5% to 40 ± 7%, p < 0.0001; younger group: 29 ± 5% to 41 ± 8%, p < 0.0001). The pacing threshold was 0.9 ± 0.8 V in the elderly group vs. 0.7 ± 0.5 V in the younger group (p = 0.350). The R wave was 9.5 ± 3.9 mV in elderly patients vs. 10.7 ± 2.7 mV in younger patients (p = 0.341). The fluoroscopic (elderly: 13 ± 7 min vs. younger: 11 ± 7 min, p = 0.153) and procedural time (elderly: 80 ± 20 min vs. younger: 78 ± 16 min, p = 0.749) were comparable between groups. Lead dislodgement occurred in 2(4%) patients, 1 in each group (p = 1.000). Intraprocedural septal perforation occurred in three patients (5%), 2(8%) in the elderly group (p = 0.585). One patient (2%) in the elderly group had a pocket infection. CONCLUSIONS: LBBAP is a feasible and safe technique for delivering physiological pacing in elderly patients who are candidates for CRT with suitable pacing parameters and low complication rates.
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Terapia de Resincronización Cardíaca , Humanos , Anciano , Terapia de Resincronización Cardíaca/métodos , Fascículo Atrioventricular , Estimulación Cardíaca Artificial/efectos adversos , Estimulación Cardíaca Artificial/métodos , Estudios de Factibilidad , Electrocardiografía/métodos , Resultado del Tratamiento , Volumen SistólicoRESUMEN
BACKGROUND: His-Bundle pacing (HBP) is an emerging technique for physiological pacing. However, its effects on right ventricle (RV) performance are still unknown. METHODS: We enrolled consecutive patients with an indication for pacemaker (PM) implantation to compare HBP versus RV pacing (RVP) effects on RV performance. Patients were evaluated before implantation and after 6 months by a transthoracic echocardiogram. RESULTS: A total of 84 patients (age 75.1±7.9 years, 64% male) were enrolled, 42 patients (50%) underwent successful HBP, and 42 patients (50%) apical RVP. At follow up, we found a significant improvement in RV-FAC (Fractional Area Change)% [baseline: HBP 34 IQR (31-37) vs. RVP 33 IQR (29.7-37.2),p = .602; 6-months: HBP 37 IQR (33-39) vs. RVP 30 IQR (27.7-35), p < .0001] and RV-GLS (Global Longitudinal Strain)% [baseline: HBP -18 IQR (-20.2 to -15) vs. RVP -16 IQR (-18.7 to -14), p = .150; 6-months: HBP -20 IQR(-23 to -17) vs. RVP -13.5 IQR (-16 to -11), p < .0001] with HBP whereas RVP was associated with a significant decline in both parameters. RVP was also associated with a significant worsening of tricuspid annular plane systolic excursion (TAPSE) (p < .0001) and S wave velocity (p < .0001) at follow up. Conversely from RVP, HBP significantly improved pulmonary artery systolic pressure (PASP) [baseline: HBP 38 IQR (32-42) mmHg vs. RVP 34 IQR (31.5-37) mmHg,p = .060; 6-months: HBP 32 IQR (26-38) mmHg vs. RVP 39 IQR (36-41) mmHg, p < .0001] and tricuspid regurgitation (p = .005) irrespectively from lead position above or below the tricuspid valve. CONCLUSIONS: In patients undergoing PM implantation, HBP ensues a beneficial and protective impact on RV performance compared with RVP.
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Fascículo Atrioventricular/fisiopatología , Estimulación Cardíaca Artificial/métodos , Marcapaso Artificial , Disfunción Ventricular Derecha/fisiopatología , Disfunción Ventricular Derecha/terapia , Anciano , Femenino , Humanos , Masculino , Volumen SistólicoRESUMEN
OBJECTIVES: To retrospectively characterize clinical predictors and impact on left ventricular (LV) ejection fraction (EF) of microvascular dysfunction (MVD) beyond microvascular obstruction (MVO), in 49 consecutive patients (58 ± 11 years), with successfully treated ST-elevation myocardial infarction. METHODS: By myocardial contrast echocardiography, MVD was considered as myocardial segments with delayed/patchy opacification, while MVO as areas without any opacification. Both MVD and MVO were planimetered and expressed as percentage of total LV wall area. Patients were divided into tertiles of MVO: I (MVO 0%), II (MVO 4-17%), and III (MVO 18-38%) groups. Cardiac troponin T (cTnT) values obtained at admission and at peak were considered for analysis. RESULTS: MVD correlated inversely with EF in groups I and II (p = 0.025, p = 0.019, respectively), but not in group III. MVD was independently predicted by cTnT on admission (ß = 1.85; 95%CI = 0.46-3.24, p = 0.011) and female sex (ß for male sex = -14.46; 95% CI = -27.96-0.95), while MVO by anterior MI (ß = 0.57; 95% CI = 0.26-0.88, p = 0.008) and peak cTnT (ß = 0.97; 95%CI = 0.57-1.38, p < 0.001). Altogether, MVD plus MVO predicted EF (ß = -0.18; 95%CI = -0.28--0.07, p = 0.002). CONCLUSIONS: Even in patients with limited amount of MVO, EF may be impaired by MVD. MVO and MVD have different predictors, which probably reflect their different pathogenesis.
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Infarto del Miocardio con Elevación del ST , Circulación Coronaria , Ecocardiografía , Femenino , Humanos , Masculino , Microcirculación , Estudios Retrospectivos , Infarto del Miocardio con Elevación del ST/diagnóstico por imagen , Volumen SistólicoRESUMEN
BACKGROUND: Intracardiac thrombosis is a relatively common pathological condition. Often, it is diagnosed at echocardiography during the subacute or chronic phase. In the very acute phase, tissue composition can make thrombus appearance very different from that usually seen. Fresh thrombosis has been previously found also in peripartum cardiomyopathy (PPC), but with imaging features different from our case. CASE SUMMARY: A 27-year-old woman was referred to our hospital for PPC, with echocardiographic finding of intraventricular masses, resembling big bubbles. Cardiac magnetic resonance (CMR) allowed definitively diagnosing intracardiac 'very acute' thrombosis, which is rarely detected. DISCUSSION: Our case provides a practical lesson about management of an unusual presentation of a common problem. When early echocardiography does not allow making a certain diagnosis, CMR can be helpful and decisive, due to its unique ability to provide characterization of intracardiac masses.
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BACKGROUND: Several ECG voltage criteria have been proposed for the diagnosis of left ventricular hypertrophy (LVH). Notably, ECG criteria have been historically validated in concentric LVH but not in hypertrophic cardiomyopathy (HCM), wherein the hypertrophy pattern is typically asymmetric. OBJECTIVES: The aim of our study was to evaluate the performance of ECG voltage criteria for LVH diagnosis in the HCM population. MATERIAL AND METHODS: The electrocardiograms of 92 HCM patients and 41 sex- and age-matched controls were evaluated with the most frequently used ECG voltage criteria for LVH diagnosis. Cardiac magnetic resonance (MRI) was performed in HCM and controls in order to quantify LVH and its distribution. RESULTS: In the HCM population, the maximal diagnostic accuracy was achieved by Amplitude total and Amplitude total product criteria (58% for both), while the Cornell Voltage best performed in septal HCM (62%), the Sokolov in aVL and Gubner criteria in apical HCM (79% for both) and the Cornell Voltage and Product in anterior HCM (86% for both). All the ECG voltage criteria showed a poor correlation with left ventricular mass and maximal thickness measured by cardiac MRI. CONCLUSIONS: In our study, only a few ECG voltage criteria used for the detection of LVH in clinical practice showed an acceptable performance in the HCM population. Further studies are needed to clarify the role of ECG for LVH detection in HCM patients.
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Potenciales de Acción , Cardiomiopatía Hipertrófica/diagnóstico , Electrocardiografía , Sistema de Conducción Cardíaco/fisiopatología , Frecuencia Cardíaca , Hipertrofia Ventricular Izquierda/diagnóstico , Adulto , Anciano , Cardiomiopatía Hipertrófica/fisiopatología , Femenino , Humanos , Hipertrofia Ventricular Izquierda/fisiopatología , Imagen por Resonancia Cinemagnética , Masculino , Persona de Mediana Edad , Valor Predictivo de las Pruebas , Reproducibilidad de los Resultados , Estudios Retrospectivos , Función Ventricular Izquierda , Remodelación VentricularRESUMEN
PURPOSE OF REVIEW: The management of hypertrophic cardiomyopathy (HCM) has changed considerably over the years, although molecular therapies targeting core mechanisms of the disease are still lacking. This review provides an overview of the contemporary medical approach to patients with HCM, and of promising novel developments hopefully soon to enter the clinical arena. RECENT FINDINGS: Our perception of therapeutic targets for medical therapy in HCM is rapidly evolving. Novel approaches include myocardial metabolic modulation, late sodium current inhibition, and allosteric myosin inhibition, actively pursued to reduce and hopefully prevent the development of severe HCM phenotypes, improve symptom control, and preserve patients from disease-related complications. Clinical management of patients with HCM should be guided by in-depth knowledge of the complex mechanisms at the energetic, metabolic, and electrophysiologic level. Until new experimental therapies become available, tailored management of modifiable disease manifestations should be pursued, including lifestyle counseling and prevention of comorbidities.
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Cardiomiopatía Hipertrófica/terapia , Bloqueadores de los Canales de Sodio , Humanos , MiocardioRESUMEN
We report the case of a 74-year-old male, with a medical history of cor triatriatum, admitted with a 10-day history of intermittent fever. Three sets of blood cultures were positive for Providencia rettgeri. Transthoracic and transesophageal echocardiogram excluded infective endocarditis, but revealed a congenital accessory tissue adhering to the mitral valve, causing supravalvular mitral stenosis. Cor triatriatum sinistrum is a rare congenital cardiac anomaly, even more uncommon in adults, and quite exceptional when associated with mitral valve disease. Because the patient had no symptoms related to the heart valve disease, no surgical indication was given and he was managed conservatively.
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Anomalías Múltiples , Corazón Triatrial/diagnóstico , Ecocardiografía Tridimensional/métodos , Ecocardiografía Transesofágica/métodos , Ventrículos Cardíacos/diagnóstico por imagen , Estenosis de la Válvula Mitral/diagnóstico , Válvula Mitral/diagnóstico por imagen , Anciano , Diagnóstico Diferencial , Humanos , Masculino , Válvula Mitral/anomalías , Estenosis de la Válvula Mitral/congénito , Enfermedades RarasRESUMEN
Intra-cardiac thrombi can be incidentally found in recurrent melanoma and need careful assessment. An 81-year-old woman, with a history of malignant nasopharyngeal melanoma, was evaluated by echocardiography and cardiac magnetic resonance due to the detection of undefined masses localized both in right atrium and ventricle during contrast-enhanced thoraco-abdominal computed tomography.
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Trombosis Coronaria/diagnóstico por imagen , Trombosis Coronaria/etiología , Neoplasias Cardíacas/complicaciones , Neoplasias Cardíacas/secundario , Melanoma/complicaciones , Anciano de 80 o más Años , Ecocardiografía , Femenino , Atrios Cardíacos/diagnóstico por imagen , Atrios Cardíacos/patología , Ventrículos Cardíacos/diagnóstico por imagen , Ventrículos Cardíacos/patología , Humanos , Imagen por Resonancia Cinemagnética , Tomografía Computarizada por Rayos XRESUMEN
OBJECTIVES: To angiographically assess myocardial perfusion in patients with Tako-Tsubo syndrome (TTS) in comparison with control individuals and patients with ST-elevation myocardial infarction (STEMI). BACKGROUND: Coronary microvascular dysfunction has been proposed as the pathophysiological mechanism underlying TTS. METHODS: We retrospectively selected consecutive TTS patients showing typical left ventricular (LV) apical dysfunction admitted to our Department in the period 2007-2011 (n=25). We also enrolled an age and gender-matched control group showing normal coronary arteries (CTR, n=25), patients with STEMI undergoing primary percutaneous intervention with myocardial reperfusion (SR, n=25) or microvascular obstruction (SMVO, n=25). TIMI flow, TIMI frame count (TFC) and both qualitative and quantitative myocardial blush grade in LV apex were assessed. Specifically, myocardial perfusion was quantitatively evaluated using 'Quantitative Blush Evaluator' (QuBE), an open source software previously validated in the setting of STEMI. RESULTS: In TTS, TIMI flow on the LAD was significantly lower and TFC significantly higher compared to CTR and SR (p=0.008 for both), while it did not significantly differ compared to SMVO (p=0.06). In TTS, MBG was significantly lower than that in CTR and SR (p=0.001 for both), while it was significantly higher than that in SMVO (p<0.001). In TTS, QuBE score was significantly lower than that in CTR and SR (p=0.001 for both) and higher than in SMVO (p=0.02). CONCLUSIONS: Our data indicate that myocardial perfusion assessed during angiography is more impaired in patients with TTS than in patients with STEMI exhibiting myocardial reperfusion, while it is less impaired than in patients with STEMI exhibiting MVO.