Controversias sobre el tratamiento con IECA/ARA-II y Covid-19 / Controversy regarding ACE inhibitors / ARBs in Covid-19

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Factores que influyen en la expresión fenotípica de la miocardiopatía hipertrófica en portadores genéticos / Factors influencing the phenotypic expression of hypertrophic cardiomyopathy in genetic carriers

Introducción y objetivos: La miocardiopatía hipertrófica (MCH) es una enfermedad con expresión variable, causada principalmente por mutaciones en genes sarcoméricos, aunque otros factores podrían estar modulando el fenotipo. El objetivo es determinar si el sexo, la hipertensión arterial o la actividad física son moduladores de la gravedad de la enfermedad y establecer su papel en la penetrancia en relación con la edad al diagnóstico de la MCH. Métodos: Se evaluó a 272 individuos (media de edad, 49 ± 17 años; el 57% varones) procedentes de 72 familias con mutación causal y se estudió la relación del sexo, la hipertensión y la actividad física con la hipertrofia ventricular izquierda. Resultados: La proporción de afectados aumenta con la edad. Se diagnosticó a los varones una mediana ajustada de 12,5 años (IC95%, -17,52 a -6,48; p < 0,001) antes que a las mujeres. A los pacientes hipertensos, se les diagnosticó MCH una mediana ajustada de 10,8 años (IC95%, 6,28-17,09; p < 0,001) más tarde que a los normotensos. A los individuos que hacían ejercicio, se los diagnosticó significativamente antes (mediana ajustada, 7,3 años; IC95%, -14,49 a -1,51; p = 0,016). El sexo, la hipertensión y el ejercicio no resultaron significativamente asociados con la gravedad de la hipertrofia del ventrículo izquierdo. Los factores explorados no influyen en la supervivencia ajustada libre de muerte súbita y el evento combinado. Conclusiones: Se diagnostica a los varones y los deportistas portadores de mutaciones sarcoméricas antes que a las mujeres y los individuos sedentarios. Los portadores de mutaciones sarcoméricas hipertensos tienen un retraso en el diagnóstico. El sexo, la hipertensión y el ejercicio no se asocian con la gravedad de la enfermedad en portadores de mutaciones causales de MCH (AU)

Introduction and objectives: Hypertrophic cardiomyopathy (HCM) is a disorder with variable expression. It is mainly caused by mutations in sarcomeric genes but the phenotype could be modulated by other factors. The aim of this study was to determine whether factors such as sex, systemic hypertension, or physical activity are modifiers of disease severity and to establish their role in age-related penetrance of HCM. Methods: We evaluated 272 individuals (mean age 49 ± 17 years, 57% males) from 72 families with causative mutations. The relationship between sex, hypertension, physical activity, and left ventricular hypertrophy was studied. Results: The proportion of affected individuals increased with age. Men developed the disease 12.5 years earlier than women (adjusted median, 95%CI, -17.52 to -6.48; P < .001). Hypertensive patients were diagnosed with HCM later (10.8 years of delay) than normotensive patients (adjusted median, 95%CI, 6.28-17.09; P < .001). Individuals who performed physical activity were diagnosed with HCM significantly earlier (7.3 years, adjusted median, 95%CI, -14.49 to -1.51; P = .016). Sex, hypertension, and the degree of physical activity were not significantly associated with the severity of left ventricular hypertrophy. Adjusted survival both free from sudden death and from the combined event were not influenced by any of the exploratory variables. Conclusions: Men and athletes who are carriers of sarcomeric mutations are diagnosed earlier than women and sedentary individuals. Hypertensive carriers of sarcomeric mutations have a delayed diagnosis. Sex, hypertension, and physical activity are not associated with disease severity in carriers of HCM causative mutations (AU)

Factors Influencing the Phenotypic Expression of Hypertrophic Cardiomyopathy in Genetic Carriers.

INTRODUCTION AND OBJECTIVES: Hypertrophic cardiomyopathy (HCM) is a disorder with variable expression. It is mainly caused by mutations in sarcomeric genes but the phenotype could be modulated by other factors. The aim of this study was to determine whether factors such as sex, systemic hypertension, or physical activity are modifiers of disease severity and to establish their role in age-related penetrance of HCM. METHODS: We evaluated 272 individuals (mean age 49 ± 17 years, 57% males) from 72 families with causative mutations. The relationship between sex, hypertension, physical activity, and left ventricular hypertrophy was studied. RESULTS: The proportion of affected individuals increased with age. Men developed the disease 12.5 years earlier than women (adjusted median, 95%CI, -17.52 to -6.48; P < .001). Hypertensive patients were diagnosed with HCM later (10.8 years of delay) than normotensive patients (adjusted median, 95%CI, 6.28-17.09; P < .001). Individuals who performed physical activity were diagnosed with HCM significantly earlier (7.3 years, adjusted median, 95%CI, -14.49 to -1.51; P = .016). Sex, hypertension, and the degree of physical activity were not significantly associated with the severity of left ventricular hypertrophy. Adjusted survival both free from sudden death and from the combined event were not influenced by any of the exploratory variables. CONCLUSIONS: Men and athletes who are carriers of sarcomeric mutations are diagnosed earlier than women and sedentary individuals. Hypertensive carriers of sarcomeric mutations have a delayed diagnosis. Sex, hypertension, and physical activity are not associated with disease severity in carriers of HCM causative mutations.

Patrón electrocardiográfico de Brugada inducido por cannabis / Brugada electrocardiogram pattern induced by cannabis

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Trombosis sobre catéter en vena cava superior izquierda / Catheter-related thrombosis in left superior vena cava

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Gadolinium-enhanced cardiovascular magnetic resonance and exercise capacity in hypertrophic cardiomyopathy.

INTRODUCTION AND OBJECTIVES: Using gadolinium-enhanced cardiovascular magnetic resonance, it is possible to evaluate the presence of myocardial fibrosis in hypertrophic cardiomyopathy. Classical disease markers are weak predictors of functional disability in affected patients. Our objective was to study the relationship between the degree of myocardial fibrosis observed by cardiac magnetic resonance and exercise capacity. METHODS: We performed cardiac magnetic resonance, echocardiography, exercise testing and Holter monitoring, along with the usual clinical assessments, in 98 patients (age, 46.3+/-15.4 years, 71.4% male) referred from two specialist hypertrophic cardiomyopathy clinics. Cardiac magnetic resonance assessment included quantifying the degree of fibrosis (i.e., the percentage of the myocardium showing enhancement) 10 min after gadolinium infusion. Symptom-limited exercise testing was used to determine exercise capacity (in metabolic equivalent [MET] units). In 71 patients, the basal N-terminal probrain natriuretic peptide (NT-proBNP) level was also measured. RESULTS: Late enhancement was observed on cardiac magnetic resonance in 67 (68.4%) patients. These patients had a lower exercise capacity (8.04+/-3.56 MET vs. 10.41+/-3.57 MET; P=.003). There was an inverse correlation between the percentage of fibrosis and exercise capacity (r=-0.21; P=.044). The best predictor of exercise capacity was the logarithm of the NT-proBNP level (r=-0.5; P< .0001). Multivariate analysis confirmed that age, a history of atrial fibrillation, the basal NT-proBNP level and the presence of fibrosis were independent predictors of exercise capacity (r2 for the model=0.47). CONCLUSIONS: The observation of areas of late gadolinium enhancement on cardiac magnetic resonance was independently associated with poor exercise capacity in patients with hypertrophic cardiomyopathy.

Cardiorresonancia magnética con gadolinio y capacidad de esfuerzo en la miocardiopatía hipertrófica / Gadolinium-Enhanced Cardiovascular Magnetic Resonance and Exercise Capacity in Hypertrophic Cardiomyopathy

Introducción y objetivos. La cardiorresonancia magnéticacon gadolinio permite estudiar la fibrosis miocárdicaen la miocardiopatía hipertrófica. Los marcadores clásicos de la enfermedad predicen débilmente la limitación funcional de estos pacientes. Nuestro objetivo es estudiar la relación entre la fibrosis en la cardiorresonancia magnética y la capacidad de ejercicio.Métodos. Se realizó cardiorresonancia magnética, ecocardiografía, ergometría y Holter, junto con la valoración clínica habitual, a 98 pacientes procedentes de dos consultas especializadas de miocardiopatía hipertrófica (edad, 46,3 ± 15,4 años; el 71,4% varones). El estudio de cardiorresonancia magnética incluyó cuantificación de fibrosis (porcentaje de miocardio realzado) 10 min tras la inyección de gadolinio. Mediante ergometría limitada por síntomas, se determinó la capacidad de esfuerzo (MET). En 71 pacientes se obtuvieron además concentraciones plasmáticas basales de NT-proBNP.Resultados. Presentaron áreas de realce tardío en lacardiorresonancia magnética 67 (68,4%) pacientes. Estospacientes tenían peor capacidad de esfuerzo (8,04 ± 3,56frente a 10,41 ± 3,57 MET; p = 0,003). Se observó correlación negativa entre el porcentaje de fibrosis y los MET alcanzados (r = –0,21; p = 0,044). El mejor predictor de capacidad de esfuerzo fue el logNT-proBNP (r = –0,5; p <0,0001). El análisis multivariado confirmó que la edad, los antecedentes de fibrilación auricular, las concentraciones basales de NT-proBNP y la fibrosis son predictores independientes de la capacidad de esfuerzo (r2 del modelo = 0,47).Conclusiones. La presencia de áreas de realce tardíocon gadolinio en la cardiorresonancia magnética se asociade forma independiente con una peor capacidad deesfuerzo en pacientes con miocardiopatía hipertrófica

Introduction and objectives. Using gadoliniumenhancedcardiovascular magnetic resonance, it ispossible to evaluate the presence of myocardial fibrosis in hypertrophic cardiomyopathy. Classical disease markersare weak predictors of functional disability in affectedpatients. Our objective was to study the relationshipbetween the degree of myocardial fibrosis observed bycardiac magnetic resonance and exercise capacity.Methods. We performed cardiac magnetic resonance,echocardiography, exercise testing and Holter monitoring,along with the usual clinical assessments, in 98 patients(age, 46.3±15.4 years, 71.4% male) referred from twospecialist hypertrophic cardiomyopathy clinics. Cardiacmagnetic resonance assessment included quantifying thedegree of fibrosis (i.e., the percentage of the myocardium showing enhancement) 10 min after gadolinium infusion. Symptom-limited exercise testing was used to determine exercise capacity (in metabolic equivalent [MET] units). In 71 patients, the basal N-terminal probrain natriuretic peptide (NT-proBNP) level was also measured.Results. Late enhancement was observed on cardiacmagnetic resonance in 67 (68.4%) patients. Thesepatients had a lower exercise capacity (8.04±3.56 METvs. 10.41±3.57 MET; P=.003). There was an inversecorrelation between the percentage of fibrosis andexercise capacity (r=–0.21; P=.044). The best predictor of exercise capacity was the logarithm of the NT-proBNPlevel (r=–0.5; P<.0001). Multivariate analysis confirmedthat age, a history of atrial fibrillation, the basalNT-proBNP level and the presence of fibrosis wereindependent predictors of exercise capacity (r2 for themodel=0.47).Conclusions. The observation of areas of lategadolinium enhancement on cardiac magnetic resonancewas independently associated with poor exercise capacityin patients with hypertrophic cardiomyopathy