Exercise Intolerance in McArdle Disease: A Role for Cardiac Impairment? A Preliminary Study in Humans and Mice.

INTRODUCTION: Whether cardiac impairment can be fully discarded in McArdle disease-the paradigm of 'exercise intolerance', caused by inherited deficiency of the skeletal muscle-specific glycogen phosphorylase isoform ('myophosphorylase')-remains to be determined. METHODS: Eight patients with McArdle disease and seven age/sex-matched controls performed a 15-minute moderate, constant-load cycle-ergometer exercise bout followed by a maximal ramp test. Electrocardiographic and two-dimensional transthoracic (for cardiac dimension's assessment) and speckle tracking [for left-ventricle global longitudinal (GLS) assessments] echocardiographic evaluations were performed at baseline. Electrocardiographic and GLS assessments were also performed during constant-load exercise and immediately upon maximal exertion. Four human heart biopsies were obtained in individuals without McArdle disease, and in-depth histological/molecular analyses were performed in McArdle and wild-type mouse hearts. RESULTS: Exercise intolerance was confirmed in patients ('second wind' during constant-load exercise, -55% peak power output vs controls). As opposed to controls, patients showed a decrease in GLS during constant-load exercise, especially upon second wind occurrence, but with no other between-group difference in cardiac structure/function. Human cardiac biopsies showed that all three glycogen phosphorylase-myophosphorylase, but also liver and especially brain-isoforms are expressed in the normal adult heart, thereby theoretically compensating for eventual myophosphorylase deficiency. No overall histological (including glycogen depots), cytoskeleton, metabolic or mitochondrial (morphology/network/distribution) differences were found between McArdle and wild-type mouse hearts, except for lower levels of pyruvate kinase M2 and translocase of outer membrane 20 kDa subunit in the former. CONCLUSIONS: This study provides preliminary evidence that cardiac structure and function seem to be preserved in patients with McArdle disease. However, the role for an impaired cardiac contractility associated with the second wind phenomenon should be further explored.

COVID-19 y afectación cardíaca / Cardiac implications and COVID-19

The coronavirus pandemic has generated a serious global health problem. COVID-19 mainly affects the lung, but it has been seen that myocardial involvement also occurs in some patients, producing myocarditis and arrhythmias.

La pandemia por coronavirus ha generado un grave problema sanitario mundial. El COVID-19 afecta fundamentalmente el pulmón, pero se ha visto que en algunos pacientes también se produce afectación del miocardio produciendo miocarditis y arritmias.

Prevalence of extra-appendage thrombosis in non-valvular atrial fibrillation and atrial flutter in patients undergoing cardioversion: a large transoesophageal echo study.

AIMS: The aim of our study was to evaluate the prevalence of left atrial cavity and appendage thrombosis in patients undergoing cardioversion for non-valvular atrial tachyarrhythmias. In persistent atrial tachyarrhythmias, 90% of thromboses are reported to be located inside the left atrial appendage. This prevalence refers to old studies and meta-analysis in a mixed population of valvular and non-valvular atrial fibrillation. Left atrial cavity thrombosis in non-valvular atrial fibrillation has not been investigated recently in large-scale studies. METHODS AND RESULTS: A total of 1,420 consecutive adult patients with paroxysmal or persistent atrial tachyarrhythmias, candidates to cardioversion, who opted for a transoesophageal echocardiography-guided strategy, were enrolled in the study. Mitral stenosis, rheumatic valve disease and mechanical prostheses were excluded. In total there were 91 thrombi in 87 patients with a prevalence of 6.13% (87/1,420). Patients with left atrial thrombosis had predisposing clinical and echo characteristics (heart failure, lower ventricular function and higher atrial volume). Except for one case in which the thrombus was located in the left atrial cavity (0.07%), and three in the right appendage, all thromboses were detected in the left atrial appendage. CONCLUSIONS: Extra-appendage thrombosis is a very rare finding in non-valvular persistent and paroxysmal atrial tachyarrhythmias and, when present, a left appendage thrombus is usually concomitant.

Discongruence Index　- Simple Indicator to Predict Prosthesis-Patient Mismatch After Transcatheter Aortic Valve Replacement.

BACKGROUND: Prosthesis-patient mismatch (PPM) after transcatheter aortic valve replacement (TAVR) remains an important issue. The aim of this study was to assess the value of a new discongruence index, to predict PPM after TAVR.Methods and Results: A total of 185 patients with severe aortic stenosis who underwent TAVR with the Edwards Sapien prosthesis or CoreValve Revalving system were included (Edwards valve, n=119; Core Valve Revalving system, n=66). Discongruence index was calculated pre-procedurally as the ratio of selected transcatheter valve size (mm) to body surface area (cm2). PPM was defined as effective orifice area (EOA) ≤0.85 cm2/m2 on transthoracic echocardiography before hospital discharge. Mean age was 82±5 years and 72 patients (38.9%) were men. The overall incidence of post-TAVR PPM was 35.1% (n=65). Discongruence index correlated with post-TAVR indexed EOA (y=0.18+0.057x; P<0.001). On multivariate logistic regression analysis, discongruence index was the only independent predictor of post-TAVR PPM (OR, 0.15; 95% CI: 0.03-0.66; P=0.012), and the area under the receiver operating characteristic curve was 0.62 (95% CI: 0.54-0.70, P=0.003), with an optimal cut-off point of 15.02 (sensitivity, 86.2%; specificity, 72.5%; positive predictive value, 74.3%; negative predictive value, 83.4%). CONCLUSIONS: The new discongruence index may be useful tool to predict PPM after TAVR.

Impacto de la puntuación de calcio en la concordancia entre la tomografía computarizada con multidetectores y la coronariografía invasiva / Impact of calcium score on agreement between multidetector computed tomography and invasive coronary angiography

Introducción y objetivos: Está demostrado que la tomografía computarizada con multidetectores (TCMD) es una alternativa factible a la coronariografía invasiva (CI). Sin embargo, se han indicado resultados contradictorios sobre el efecto de la puntuación de calcio (PC) coronario en la precisión diagnóstica de la TCMD. El objetivo de este estudio es evaluar la concordancia entre la TCMD y la CI y evaluar la influencia de la PC en ella. Métodos: Se incluyó a 266 pacientes consecutivos sometidos a evaluación por TCMD de 64 cortes y por CI. Se utilizó el software habitual para la PC mediante el método Agatston. Un observador clasificó cualitativamente y de manera enmascarada las estenosis como leve, moderada o grave, y se compararon con los resultados obtenidos por la CI, utilizada como método de referencia. Resultados: La media de edad de los pacientes era 65,4 ± 11,2 años, y 188 (70,3%) eran varones. Se evaluó cualitativamente y se cuantificó por TCMD un total de 484 segmentos con estenosis coronaria al menos leve. Las mediciones no invasivas concordaban con la CI en 402 estenosis (el 83,05%; kappa = 0,684), sin diferencias significativas entre vasos y sin una influencia estadística significativa de la PC en la concordancia (OR = 0,93; IC95%, 0,76-1,09; p = 0,21). La TCMD tuvo sensibilidad, especificidad, valor predictivo positivo y valor predictivo negativo altos en los análisis por segmento, por vaso y por paciente. Conclusiones: La coronariografía no invasiva mediante TCMD mostró buena concordancia con la CI en la cuantificación cualitativa de las estenosis coronarias, y la PC no tuvo un impacto significativo en esa concordancia (AU)

Introduction and objectives: Multidetector computed tomography (MDCT) has been demonstrated as a feasible alternative to invasive coronary angiography (ICA). However, contradictory results have been reported regarding the effect of coronary artery calcium score (CS) on the diagnostic accuracy of MDCT. Our aim was to assess the agreement of MDCT and ICA and to evaluate the influence of CS on this agreement. Methods: We enrolled 266 consecutive patients who underwent evaluation with 64-slice MDCT and ICA. Standard CS software tools were used to calculate the Agatston score. Stenosis was qualitatively classified as mild, moderate, or severe by 1 blinded observer and the results were compared with those of ICA, which was used as the gold standard. Results: The mean age of the patients was 65.4 11.2 years, and 188 patients (70.3%) were men. A total of 484 segments with coronary stenosis mild were qualitatively evaluated and quantified with MDCT. Noninvasive measurements were concordant with ICA in 402 stenoses (83.05%; Kappa, 0.684), with no significant differences between vessels and with no statistically significant influence of CS on this agreement (OR, 0.93; 95%CI, 0.76-1.09; P = .21). Multidetector computed tomography had high sensitivity, specificity, positive predictive value, and negative predictive value on a per-segment, per-vessel, and per-patient basis. Conclusions: Non-ICA using MDCT showed good agreement with ICA in the qualitative quantification coronary stenosis and CS had no significant impact on this agreement (AU)

Fusión de ecocardiografía 2D/3D-fluoroscopia: nueva herramienta en el intervencionismo cardiaco / Fusion of 2 D and 3 D echocardiography with fluoroscopy: a new tool in cardiac interventionism

Resumen La evolución en el intervencionismo estructural percutáneo ha generado un desarrollo paralelo en las técnicas de imagen avanzada. Teniendo en cuenta que este tipo de procedimientos requiere un entendimiento total de la anatomía cardiaca y con el fin de garantizar los mejores resultados y seguridad para el paciente durante procedimientos complejos, se desarrolló un software capaz de fusionar la fluoroscopia y la ecocardiografía 3 D en una sola imagen. La tecnología del Echonavigator® integra dos técnicas de imágenes dinámicas de un corazón en movimiento; el adecuado co-registro y visualización de fluoroscopia y ecocardiografía transesofágica 2D/3D es la clave en la obtención de resultados óptimos. La fusión de dos imágenes dinámicas en tiempo real es una herramienta factible y segura en los procedimientos de intervencionismo estructural del corazón; los estudios iniciales han logrado disminuir el tiempo de realización de los procedimientos y las dosis de radiación al paciente. El futuro es prometedor y su progresiva expansión de uso teniendo en cuenta su reciente aparición, aportará más datos en el crecimiento de esta tecnología.

Abstract The evolution of percutaneous structural interventionism has led to the simultaneous development of advanced imaging techniques. Taking into account that these types of procedures require a full understanding of cardiac anatomy, and with the aim of ensuring the best results and patient safety during complex procedures, a software program was developed that was capable of fusing and 3D-echocardiography into a single image. The Echonavigator® technology combines two dynamic imaging techniques of a heart in movement. The appropriate co-recording and visualising of fluoroscopy and 2D/3D transoesophageal echocardiography is essential for obtaining optimal results. The fusion of two images in real-time is a feasible and safe tool in structural heart interventionist procedures. Initial studies have managed to decrease the time of performing the procedures, as well as the dose of radiation to the patient. The future is promising, and its gradual expansion of use, taking into account its recent appearance, will provide more data on the growth of this technology.

Impact of Calcium Score on Agreement Between Multidetector Computed Tomography and Invasive Coronary Angiography.

INTRODUCTION AND OBJECTIVES: Multidetector computed tomography (MDCT) has been demonstrated as a feasible alternative to invasive coronary angiography (ICA). However, contradictory results have been reported regarding the effect of coronary artery calcium score (CS) on the diagnostic accuracy of MDCT. Our aim was to assess the agreement of MDCT and ICA and to evaluate the influence of CS on this agreement. METHODS: We enrolled 266 consecutive patients who underwent evaluation with 64-slice MDCT and ICA. Standard CS software tools were used to calculate the Agatston score. Stenosis was qualitatively classified as mild, moderate, or severe by 1 blinded observer and the results were compared with those of ICA, which was used as the gold standard. RESULTS: The mean age of the patients was 65.4 ± 11.2 years, and 188 patients (70.3%) were men. A total of 484 segments with coronary stenosis ≥ mild were qualitatively evaluated and quantified with MDCT. Noninvasive measurements were concordant with ICA in 402 stenoses (83.05%; Kappa, 0.684), with no significant differences between vessels and with no statistically significant influence of CS on this agreement (OR, 0.93; 95%CI, 0.76-1.09; P = .21). Multidetector computed tomography had high sensitivity, specificity, positive predictive value, and negative predictive value on a per-segment, per-vessel, and per-patient basis. CONCLUSIONS: Non-ICA using MDCT showed good agreement with ICA in the qualitative quantification coronary stenosis and CS had no significant impact on this agreement.

Fusión eco-escopia en el cierre percutáneo de la orejuela / Eco-X ray fusion in left atrial appendage closure

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Selección de lo mejor del año 2016 en tomografía computarizada cardiaca / Selection of the best of 2016 in cardiac computed tomography

No disponible

Tricuspid annular plane systolic excursion inaccuracy to assess right ventricular function in patients with previous tricuspid annulopasty.

OBJETIVES: The clinical and prognostic usefulness of tricuspid annular plane systolic excursion (TAPSE) is well established. However, the ability of TAPSE to assess right ventricular (RV) function in patients with previous tricuspid valve annulopasty is controversial. This study examined the TAPSE suitability in patients with previous tricuspid valve annuloplasty using right ventricular fractional area change (RVFAC) as reference method. METHODS: We retrospectively analyzed 53 patients who underwent tricuspid valve annuloplasty at our hospital between 2013 and 2016. TAPSE and RVFAC were obtained in preoperative and postoperative periods using standard methodology. RESULTS: Mean age was 68±12years and 34 patients (64.1%) were women. TAPSE decreased significantly after surgery in comparison with pre-surgical values (17±4.2 Vs 12.9±4.1mm, p<0.001). On the contrary, RVFAC did not change significantly after surgery (37±9.2 Vs 36.2.9, p=0.25). The correlation between RVFAC and TAPSE was better in the preoperative (r=0.63, p<0.0001) than in the postoperative period (r=0.38, P=0.005). Good intra- and interobserver agreement for TAPSE and RVFAC was obtained, with intraclass correlation coefficients of 0.97 and 0.92 for TAPSE; and 0.90 and 0.85 for RVFAC, respectively. CONCLUSIONS: These findings suggest that TAPSE is not suitable after tricuspid valve annuloplasty and it leads to an underestimation of RV systolic function. It seems to be appropriate to rely on echocardiographic parameters of global RV function such as RVFAC in this context.

Prevalence of auricular thrombosis before atrial flutter cardioversion: a 17-year transoesophageal echocardiographic study.

AIMS: Prevalence of left appendage thrombosis ranges from 6 to 18% in persistent atrial fibrillation (AF). Few and low sample size studies have assessed left and right atrial thrombosis in persistent atrial flutter (AFL) and a wide variety of frequencies, from 1 to 21%, has been reported. The aim of this study was to evaluate the prevalence of atrial appendage thrombosis in a large population of patients undergoing transoesophageal echocardiography (TEE)-guided cardioversion (CV) for recent AFL onset and compare it with AF. METHODS AND RESULTS: From 1999 to September 2014, we collected data of 1081 patients to CV: 877 affected by AF (81.1%) and 204 by AFL (18.9%). The presence of auricular thrombosis was evaluated by TEE in AF or AFL persisting for more than 48 h. The presence of appendage thrombosis, Doppler emptying velocities, and severe spontaneous echo contrast (SEC) was studied. The overall prevalence of atrial thrombosis was 9.62% (104/1081). Frequency of atrial thrombosis in AFL patients was 6.4% (13/204) vs. 10.5% among AF (92/877), P = 0.074. Comparing the two appendages, frequency of left atrial appendage thrombosis was in AFL 5.9% (12/204) vs. 9.9% (87/877) in the AF group, P = 0.07. Right atrial appendage thrombosis was present in 0.5% (1/204) in the AFL group vs. 0.8% (7/877) in the AF group, P = 0.64. Moderate to severe SEC (3+/4+) was present in 28% of AFL patients (57/204) vs. 35% of AF patients (307/877), P = 0.05. CONCLUSION: Auricular thrombosis is not an infrequent finding in AFL before CV. Our study suggests the use of TEE screening in AFL, as well as in AF, when patients arrive to clinical attention after more than 48 h from arrhythmia onset.

M-mode apical systolic excursion: A new and simple method to evaluate global left ventricular longitudinal strain.

INTRODUCTION: Since M-mode measurements can assess deformation of specific regions of the left ventricle, we hypothesized that M-mode measurements like M-mode apical systolic excursion (MMASE) and mitral annular plane systolic excursion (MAPSE) may be correlated with left ventricular longitudinal strain (LVLS). METHODS: All subjects of the study underwent a full echocardiographic evaluation and MMASE and MAPSE measurement. Three-dimensional wall motion tracking (3D-WMT) was performed. RESULTS: Thirty-one patients were evaluated. Significant correlations between MAPSE and LVLS (-0.372; p=0.04) and between MMASE and LVLS (-0.398; p=0.027) were found. LVLS was linearly related to MAPSE and MMASE (in mm) as follows: ST=-10.6 -0.4 * MAPSE (r2=0.14) and ST=-13.1 -0.5 * MMASE (r2=0.16). CONCLUSIONS: Our results demonstrate that simpler and faster methods than strain based on complex speckle analysis can also have a role in predicting subclinical left ventricular systolic dysfunction.

Efficacy and learning curve of a hand-held echocardiography device in an oncology outpatient clinic: Expanding the use of echoscopic heart examination beyond cardiology.

Certain chemotherapy drugs for breast cancer may induce cardiotoxicity and these patients should be echocardiographically monitored. The performance of a focused echocardiographic evaluation (echoscopy) at the patient's location by a non-cardiologist appears to be feasible. The aim of the present study was to assess the accuracy of echoscopy performed by medical oncologists in an outpatient clinic using hand-held echocardiography devices. The study cohort comprised consecutive unselected patients who attended an oncology outpatient clinic. Two medical oncologists attended a one-week training period, which included theoretical and practical teaching by an expert cardiologist. Every subject underwent two echo examinations. The first examination was performed by an oncologist using a hand-held echo device and the second was performed by a cardiologist using a 'premium' device. Out of the 101 enrolled patients, 32 were men (31.7%) and the mean age was 56.03±16.88 years. There was a good global agreement [intra-class correlation coefficient (ICC): 0.65 for left ventricular ejection fraction (LVEF)]. When the results were analyzed depending on the period of time when the echo studies were performed, a clear and short learning curve was observed: LVEF started at ICC=0.58 and increased to 0.66 and 0.77 in the second and third period, respectively. There were extremely few clinically significant differences and a learning curve was also evident. In conclusion, cardiac echoscopy performed by an oncologist with a hand-held device may lead to a similar clinical management as a study performed by an expert cardiologist with a 'premium' system in patients under chemotherapy following a short training period.

Giant and thrombosed left ventricular aneurysm.

Left ventricular aneurysms are a frequent complication of acute extensive myocardial infarction and are most commonly located at the ventricular apex. A timely diagnosis is vital due to the serious complications that can occur, including heart failure, thromboembolism, or tachyarrhythmias. We report the case of a 78-year-old male with history of previous anterior myocardial infarction and currently under evaluation by chronic heart failure. Transthoracic echocardiogram revealed a huge thrombosed and calcified anteroapical left ventricular aneurysm. Coronary angiography demonstrated that the left anterior descending artery was chronically occluded, and revealed a big and spherical mass with calcified borders in the left hemithorax. Left ventriculogram confirmed that this spherical mass was a giant calcified left ventricular aneurysm, causing very severe left ventricular systolic dysfunction. The patient underwent cardioverter-defibrillator implantation for primary prevention.