Agalsidase alfa long-term effect on left ventricular hypertrophy in Fabry disease / Efecto a largo plazo de la agalsidasa alfa sobre la hi pertrofia ventricular izquierda en la enfermedad de Fabry

Abstract Introduction : Fabry disease (FD) is an X-linked ly sosomal storage disorder affecting glycosphingolipid metabolism. Most FD patients have cardiac involvement, mainly manifested as left ventricular hypertrophy (LVH), leading to early death due to complications (arrhyth mias, valvular disease, vascular involvement). Early initiation of enzyme replacement therapy (ERT) before fibrosis development has been associated with better cardiac outcomes in terms of left ventricular mass index (LVMI) and functional parameters. Methods : A retrospective observational study was conducted in patients with FD treated with agalsidase alfa for at least 2 years. The primary objectives were: [a] to assess the annual rate of change in LVMI; [b] to define the overall incidence of stability, regression or progression of LVMI. Results : Forty-nine patients were included in the final analysis, with a median follow-up of 7 years. The overall change in LVMI was 0.38 g/m2.73/year, without significant influence of baseline LVH, gender, age at ERT initiation, LV ejection fraction, body mass index, renal disease, and classical cardiovascular risk factors. Long-term ERT with agalsidase alfa was associated with stabilization of LVMI in 98% of patients with FD and was independent of the same covariables. Conclusion : Our results are in line with previous literature of comparable FD populations and probably represent the first study of its kind in Argentina. We here highlight the importance of cardiac morphomet ric stability as a positive outcome of ERT.

Resumen Introducción : La enfermedad de Fabry (EF) es una enfermedad de almacenamiento lisosomal ligada al cromosoma X que afecta el metabolismo de glicoes fingolípidos. La mayoría de pacientes EF tienen afec tación cardíaca, manifestada principalmente como hipertrofia ventricular izquierda (HVI), que conduce a muerte prematura secundaria a complicaciones (arritmias, valvulopatías, afectación vascular). El trata miento de reemplazo enzimático (TRE) precoz, iniciado antes del desarrollo de la fibrosis, se relaciona con mejores resultados cardíacos en términos del índice de masa ventricular izquierda (IMVI) y parámetros funcionales. Métodos : Se realizó un estudio retrospectivo obser vacional en que se incluyeron pacientes con EF tratados con agalsidasa alfa por al menos 2 años. Los objetivos primarios fueron: [a] evaluar el cambio anual del IMVI; [b] definir la incidencia global de estabilidad, regresión o progresión del IMVI. Resultados : Se incluyeron 49 pacientes, con segui miento (mediana) de 7 años. El cambio global en el IMVI fue 0.38 g/m2.73/año, sin influencia significativa de HVI basal, sexo, edad de inicio de TRE, fracción de eyección del VI, índice de masa corporal, insuficiencia renal y factores de riesgo cardiovascular clásicos. La TRE a largo plazo con agalsidasa alfa se relacionó con la estabilización del IMVI en el 98% de los pacientes con EF, independientemente de las mismas covariables. Conclusión : Nuestros resultados están en línea con la bibliografía previa de poblaciones comparables y, pro bablemente, representan el primer estudio de este tipo en Argentina. Se destaca la importancia de la estabilidad morfométrica cardíaca como resultado positivo de la TRE.

Left ventricular hypertrophy: hypertensive or hypertrophic cardiomyopathy? What a dilemma! A case report / Hipertrofia ventricular izquierda: ¿cardiopatía hipertensiva o miocardiopatía hipertrófica? ¡Vaya dilema! Reporte de caso

Abstract In the presence of the left ventricle hypertrophy (LVH), the differential diagnosis with hypertrophic cardiomyopathy (HCM) or some phenocopy must be always considered, which can be easily suspected when the hypertrophy is markedly asymmetric. However, when the hypertrophy is homogeneous, especially if the patient has concomitant hypertension, it may be a challenge to distinguish between hypertensive and HCM, although some clinical features may help us to suspect it. In addition, patients with HCM may present with exertional angina due to microcirculation involvement in the setting of the hypertrophy itself or dynamic obstruction in the left ventricular outflow tract, but in some cases, the presence of concomitant coronary artery disease must be suspected as the cause of angina, especially if the patient has an intermediate or high-risk probability of having ischemic heart disease. We present the case of a 46-year-old Afro-American man with poorly controlled hypertension who was found to have severe LVH, and who presented with symptoms of exertional angina during follow-up. We will review the clinical features that can help us in the differential diagnosis in this context.

Resumen Ante la presencia de hipertrofia del ventrículo izquierdo (HVI), siempre se debe considerar el diagnóstico diferencial con la miocardiopatía hipertrófica (MCH) o alguna fenocopia, que puede sospecharse fácilmente cuando la hipertrofia es marcadamente asimétrica. Además, los pacientes con MCH pueden presentar angina de esfuerzo debido a la afectación de la microcirculación en el contexto de la propia hipertrofia o si ésta condiciona obstrucción dinámica al tracto de salida del ventrículo izquierdo, pero en algunos casos debe sospecharse la presencia de enfermedad coronaria concomitante como causa de la angina, especialmente si el paciente tiene una probabilidad de riesgo intermedio o alto de padecer cardiopatía isquémica. Presentamos el caso de un varón de 46 años de afroamericana con hipertensión arterial mal controlada a quien se le detectó una HVI severa, y que durante el seguimiento presentó síntomas de angina de esfuerzo. Revisaremos las características clínicas que nos pueden ayudar en el diagnóstico diferencial en este contexto.

Diferenças entre Duas Condições Cardíacas Hipertróficas Distintas: Doença de Fabry Versus Cardiomiopatia Hipertrófica / Differences Between Two Distinct Hypertrophic Cardiac Conditions: Fabry Disease versus Hypertrophic Cardiomyopathy

Resumo Fundamento A cardiomiopatia hipertrófica (CMH) e a doença de Fabry (DF) são doenças herdadas geneticamente com características fenotípicas de hipertrofia ventricular esquerda (HVE) que causam resultados cardíacos adversos. Objetivos Investigar as diferenças demográficas, clínicas, bioquímicas, eletrocardiográficas (ECG) e ecocardiográficas (ECO) entre CMH e DF. Métodos 60 pacientes com CMH e 40 pacientes com DF foram analisados retrospectivamente como uma subanálise do "estudo LVH-TR" após exclusão de pacientes com fibrilação atrial, ritmo de estimulação, bloqueios de ramo e bloqueios atrioventriculares (AV) de segundo e terceiro graus. O nível de significância foi aceito como <0,05. Resultados O sexo masculino (p=0,048) e a creatinina (p=0,010) são significativamente maiores a favor da DF; entretanto, infradesnivelamento do segmento ST (p=0,028), duração do QT (p=0,041), espessura do septo interventricular (SIVd) (p=0,003), espessura da parede posterior (PWd) (p=0,009), insuficiência mitral moderada a grave (IM) (p=0,013) e o índice de massa ventricular esquerda (IMVE) (p=0,041) são significativamente maiores a favor da CMH nas análises univariadas. Na análise multivariada, a significância estatística apenas permanece na creatinina (p=0,018) e na duração do intervalo QT (0,045). A DF foi positivamente correlacionada com a creatinina (rho=0,287, p=0,004) e a CMH foi positivamente correlacionada com o PWd (rho=0,306, p=0,002), IVSd (rho=0,395, p<0,001), IM moderada-grave (rho= 0,276, p<0,005), IMVE (rho=0,300, p=0,002), espessura relativa da parede (ERP) (rho=0,271, p=0,006), duração do QT (rho=0,213, p=0,034) e depressão do segmento ST (rho =0,222, p=0,026). Conclusão Características bioquímicas, ECG e ECO específicas podem auxiliar na diferenciação e no diagnóstico precoce da CMH e da DF.

Abstract Background Hypertrophic cardiomyopathy (HCM) and Fabry disease (FD) are genetically inherited diseases with left ventricular hypertrophy (LVH) phenotype characteristics that cause adverse cardiac outcomes. Objectives To investigate the demographic, clinical, biochemical, electrocardiographic (ECG), and echocardiographic (ECHO) differences between HCM and FD. Methods 60 HCM and 40 FD patients were analyzed retrospectively as a subanalysis of the 'LVH-TR study' after excluding patients with atrial fibrillation, pace rhythm, bundle branch blocks, and second and third-degree atrioventricular (AV) blocks. The significance level was accepted as <0.05. Results Male gender (p=0.048) and creatinine (p=0.010) are significantly higher in favor of FD; however, ST depression (p=0.028), QT duration (p=0.041), interventricular septum thickness (IVSd) (p=0.003), posterior wall thickness (PWd) (p=0.009), moderate-severe mitral regurgitation (MR) (p=0.013), and LV mass index (LVMI) (p=0.041) are significantly higher in favor of HCM in the univariate analyses. In multivariate analysis, statistical significance only continues in creatinine (p=0.018) and QT duration (0.045). FD was positively correlated with creatinine (rho=0.287, p=0.004) and HCM was positively correlated with PWd (rho=0.306, p=0.002), IVSd (rho=0.395, p<0.001), moderate-severe MR (rho=0.276, p<0.005), LVMI (rho=0.300, p=0.002), relative wall thickness (RWT) (rho=0.271, p=0.006), QT duration (rho=0.213, p=0.034) and ST depression (rho=0.222, p=0.026). Conclusion Specific biochemical, ECG, and ECHO characteristics can aid in the differentiation and early diagnosis of HCM and FD.

Characteristics of Early Cardiac Involvement in 45 Patients With Fabry Disease Monitored by Ultrasonic Cardiogram / 中山大学学报(医学科学版)

ObjectiveTo evaluate the changes in cardiac structure and ventricular function in patients with Anderson-Fabry Disease （AFD） by two-dimensional speckle tracking echocardiography （2D-STE） and to explore the characteristics of their early cardiac involvement. MethodsAll 45 patients diagnosed with AFD in this observational study underwent routine ultrasonic cardiogram （UCG） examination and 2D-STE. The patients were divided into 2 groups based on UCG measurements： with left ventricular hypertrophy （interventricular septum or posterior left ventricular wall thickness ≥12 mm） and without left ventricular hypertrophy. TomTec software was used to analyze the echocardiographic images， then the baseline data， UCG routine parameters and myocardial strain of the two groups were compared. ResultsThe study included 27 males （60.0%） and 18 females （40.0%）， with an average age of （32.33±16.11）， 17 cases （37.78%） with left ventricular hypertrophy and 28 cases （62.22%） without left ventricular hypertrophy. All patients had normal left ventricular ejection fraction （LVEF） （> 50%）. Compared with those without left ventricular hypertrophy， patients with left ventricular hypertrophy had significantly more target organ involvement， significantly higher E/A and average E/E' ratios （P < 0.05）. No statistical difference was found in global and segmental longitudinal strain （LS）， circumferential strain （CS） and radial strain （RS） of the endocardium and myocardium between the two groups （all P > 0.05）. There were lower absolute values of global and segmental LS and CS in the myocardium than in the endocardium （all P < 0.05）， and higher absolute values of LS and RS in the mid segment than in the basal and apical segments （all P < 0.05）. ConclusionsThere is no significant association between early systolic dysfunction and left ventricular wall thickness. 2D-STE strain can be used to detect AFD in the early stage. Ventricular wall myocardium exhibits more serious involvement than endocardium and mid segment was less involved than the apical and basal segments.

Valor del electrocardiograma en el diagnóstico de la hipertrofia ventricular izquierda en la hipertensión arterial

Introducción: la hipertrofia ventricular izquierda es un factor de riesgo independiente de enfermedad cardiovascular. Existen diversos criterios electrocardiográficos para el diagnóstico, con distintas sensibilidades y especificidades. Objetivo: determinar el valor diagnóstico de los criterios electrocardiográficos de hipertrofia del ventrículo izquierdo en comparación a la ecocardiografía transtorácica en personas adultas con hipertensión arterial. Material y método: diseño observacional, descriptivo, retrospectivo de corte transversal, tipo prueba diagnóstica, que incluyó a pacientes adultos con diagnóstico de hipertensión arterial internados en las salas de Clínica Médica del Hospital de Clínicas, Paraguay, desde agosto del 2022 a agosto del 2023. Se determinaron las variables demográficas, criterios electrocardiográficos (Sokolov-Lyon, Cornell, Lewis, Peguero Lo Presti) y ecocardiográficos de hipertrofia ventricular izquierda. Resultados: se evaluaron 517 electrocardiogramas y ecocardiografías de pacientes hipertensos. Según criterio de Sokolov-Lyon la sensibilidad fue 16% y la especificidad 70% para el diagnóstico de la hipertrofia ventricular izquierda; por criterios de Cornell la sensibilidad fue 43% y especificidad 87 %; por criterios de Lewis la sensibilidad fue 26% y especificidad 76% y por criterios de Peguero Lo Presti la sensibilidad fue 63% y la especificidad 87%. Conclusión: el criterio por electrocardiograma de hipertrofia ventricular izquierda con mayor sensibilidad fue de Peguero Lo Presti y los de mayor especificidad fueron los de Peguero Lo Presti y Cornell.

Introduction: Left ventricular hypertrophy is an independent risk factor for cardiovascular disease. There are various electrocardiographic criteria for diagnosis, with different sensitivities and specificities. Objective: To determine the diagnostic value of electrocardiographic criteria for left ventricular hypertrophy in comparison to transthoracic echocardiography in adults with arterial hypertension. Material and method: Observational, descriptive, retrospective cross-sectional design, diagnostic test type, which included adult patients with a diagnosis of arterial hypertension admitted to the Medical Clinic rooms of the Hospital de Clínicas, Paraguay, from August 2022 to August 2023. Demographic variables, electrocardiographic criteria (Sokolow-Lyon, Cornell, Lewis, Peguero Lo Presti), and echocardiographic criteria for left ventricular hypertrophy were determined. Results: Five hundred seventeen electrocardiograms and echocardiograms of hypertensive patients were evaluated. According to the Sokolow-Lyon criteria, the sensitivity was 16% and the specificity was 70% for the diagnosis of left ventricular hypertrophy; by Cornell criteria, the sensitivity was 43% and specificity 87%; by Lewis criteria, the sensitivity was 26% and specificity 76% and by Peguero Lo Presti criteria. the sensitivity was 63% and the specificity 87%. Conclusion: The electrocardiogram criterion of left ventricular hypertrophy with the greatest sensitivity was that of Peguero Lo Presti and those with the greatest specificity were those of Peguero Lo Presti and Cornell.

The Prevalence of Isolated Systolic Hypertension at a Tertiary Care Hospital in Eastern India : An Observational Study

Background and Objectives : Hypertension is a significant public health issue. Isolated Systolic Hypertension (ISH) was once considered to be a benign aspect among the aging population, but an association with an increased risk of Cardiovascular Disease is now known. ISH shows an increasing prevalence with increase in age. This study was undertaken to determine the incidence of ISH among adults in Eastern India. Methods : This study evaluated the adult population (aged 18-70 years) attending the NRS Medical College and Hospital, a Tertiary Care Center in East India. The clinical characteristics and echocardiographic findings were also evaluated. Results : A total of 800 patients met the inclusion criteria, of whom 75 (9.37%) had ISH. Blood Pressure increased with age. The most common echocardiographic change observed in ISH patients was increased Left Ventricular Mass Index (LVMI), while concentric Left Ventricular Hypertrophy (LVH) was more common in women than men with isolated Systolic Hypertension. The incidence of LVMI increased as the severity of ISH increased. Furthermore, patients with stage 3 ISH were nearly 4 times more likely to develop Proteinuria. Conclusion : The findings of this study are in line with previous studies evaluating the presence of ISH in the adult Indian population. There is need for effective population screening along with effective treatment for Blood Pressure to reduce morbidity and mortality. Primary prevention strategies may be the need of the hour in the Indian population which is at risk of cardiovascular Disease associated with Hypertension

Left Ventricle Longitudinal Strain Parameters Are Effective for Differentiating Cardiac Amyloidosis from Primary Hypertensive Left Ventricular Hypertrophy / 中山大学学报(医学科学版)

ObjectiveTo clarify the value of the left ventricular longitudinal strain（LVLS）parameters in patients with cardiac amyloidosis （CA） and primary hypertension with left ventricular hypertrophy （HLVH）. MethodsForty-one patients confirmed with CA were selected and assigned to CA with hypertension group （n =14） and pure CA group （n=27） based on the initial diagnosis with or without hypertension. Twenty patients with primary hypertension-induced left ventricular hypertrophy （HLVH group） and twenty healthy controls were also selected， matching for gender， age， and body surface area. Clinical data， conventional echocardiography parameters were collected and LVLS parameters were measured. Within-group variations were compared among the four groups， and pairwise comparisons were conducted between groups. The sensitivity and specificity of each parameter in predicting CA were judged by the receiver operator characteristic （ROC） curvy in CA and HLVH patients with left ventricular ejection fraction （LVEF） preserved. ResultsAmong the conventional echocardiography parameters， LVEF and left ventricular end-diastolic diameter （LVEDD） were lower in the CA with hypertension group and pure CA group compared with the higher values in the HLVH group and control group. Whereas， left ventricular posterior wall thickness （LVPWT）， relative wall thickness （RWT）， and average E/e' were higher in the two CA groups compared with the HLVH group （all P＜0.05）.Among the LVLS parameters， Global longitudinal strain （GLS） was the worst in the CA with hypertension group so as pure CA group， modest in the HLVH group， and highest in the control group. On the contrary， relative longitudinal strain and ejection fraction strain ratio （EFSR） were the highest in the CA with hypertension group so as to pure CA group， modest in the HLVH group， and lowest in the control group （all P＜0.05）. ROC analysis showed that when LVEF was preserved， the absolute value of GLS less than 14.35% and EFSR higher than 4.28 could effectively distinguish CA from HLVH （all AUCs＞0.9，all P＜0.05）； meanwhile GLS showed high sensitivity（100%） and EFSR showed great specificity（95%）. There were not statistically significance in any parameter between CA with hypertension group and pure CA group（all P＞0.05）. ConclusionWhether CA was complicated with hypertension or not， there were statistically significance among routine echocardiography and LVLS parameters compared with HLVH. In particular， GLS and EFSR are accurate in predicting CA in patients with myocardial hypertrophy and preserved LVEF.

Echocardiographic Wall Motion Abnormalities in Hypertensive Patients with Electrocardiographic Left Ventricular Hypertrophy

Background: During the cardiac cycle with the effect on cardiac function, Echocardiographic wall motion abnormalities are kinetic alterations in the cardiac wall motion. It can be characterized as per their grades and their distribution pattern like segmental or global. It can be attributed to a coronary territory or follow a non-coronary distribution. Aim of the study: The aim of the study was to evaluate the patterns of echocardiographic wall motion abnormalities among hypertensive patients with electrocardiographic left ventricular hypertrophy.Material & Methods:This was prospective observational study which was conducted in the Department of Cardiology, Ibrahim Cardiac Hospital & Research Institute, Dhaka, Bangladesh from January 2020 to January 2021. In total 76 hypertensive patients with electrocardiographic left ventricular hypertrophy were included as the study subjects for this study. This study was approved by the ethical committee of the mentioned hospital. A predesigned questioner was used in data collection. All data were collected, processed and analyzed by using MS Office and SPSS version 23 programs as per necessity.Results:In this study, in analyzing the status of wall motion abnormalities (WMA) among the participants we observed that, 22% of the patients were with segmental wall motion abnormalities, 18% were with global dysfunction and 59% were fully free from any type of WMA. Among patients with global dysfunctions, 71% were with mild and 12% were with moderate-to-severe dysfunction whereas this ratio was 47:24 in segmental abnormality patients. On the other hand, in analyzing the severity and location of patients with segmental WMA we observed that 47%, 24%, 53%, 41%, 18%, 24%, 29%, 47%, 35%, 53% and 41% were with mild dysfunction, moderate-to-severe dysfunction, inferior wall dysfunction, antero-septal dysfunction, anterior wall dysfunction, lateral wall dysfunction, posterior wall dysfunction, posterior septal dysfunction, LV base dysfunction, LV mid-cavity dysfunction and LV apical dysfunction respectively.Conclusion:In this study, majority of the participants were found free from any type of wall motion abnormality. ‘Mild dysfunction’, inferior wall dysfunction, anterior wall dysfunction, lateral wall dysfunction, posterior septal dysfunction, LV base dysfunction, LV mid-cavity dysfunction and LV apical dysfunction are found as some more frequent dysfunctions among patients with WMA.

Assessment of Lest Ventricular Hypertrophy and Left Ventricular Diastolic Dysfunction Cardiovascular in Patients of Chronic Renal Failure by Echocardiography

Introduction: Chronic renal failure, regardless of the cause, is the presence of kidney damage or a reduced level of kidney function for three months or longer. It is a group of signs and symptoms brought on by slow and long-term renal damage. The most frequent cardiovascular finding in people on dialysis is LVH. Objective: The study was conducted to estimate the prevalence of left ventricular hypertrophy and left ventricular diastolic dysfunction by echocardiography in patients with chronic renal failure. Method: This was an observational cross-sectional study at the Department of General Medicine among IPD patients, Tertiary Care Hospital, Surat. Result: Left ventricular hypertrophy out of 34 cases 22 (64.71%) cases were show left ventricular hypertrophy with an odd ratio of 3.208 (1.049, 9.81) and a p-value 0.0378 which was statically significant. prevalence of diastolic dysfunction was 79%. comparison of renal function test and echocardiographic change of chronic renal failure. In the renal function test serum, creatinine and EGFR were show a p-value < 0.001 which was statistically significant. Conclusion: Cardiac dysfunction and LVH are frequently noted in individuals with chronic renal failure at the time of commencement of dialysis. cardiovascular abnormalities in the form of LVH and diastolic dysfunction which antedate severe systolic dysfunction are frequently observed in milder degrees of chronic renal failure

Caracterización de la hipertensión arterial en adultos menores de 30 años de edad de un hospital del Paraguay

RESUMEN Introducción: la hipertensión arterial constituye el principal factor de riesgo para el desarrollo de enfermedades cardiovasculares. Su detección en adultos jóvenes obliga a buscar una causa secundaria y potencialmente corregible. Objetivo: determinar las características clínicas de la hipertensión arterial en adultos menores de 30 años de edad que acuden al Hospital Nacional, Itauguá, Paraguay, en el periodo 2019-2021. Sujetos y métodos: estudio descriptivo observacional retrospectivo de corte transversal donde se incluyó 50 pacientes entre 18 y 30 años de edad con hipertensión arterial. La investigación fue aprobada por el Comité de Ética de la Universidad Nacional de Itapúa, Paraguay. Resultados: la edad media fue 23 ± 4 años, en su mayoría eran provenientes de la zona urbana, siendo 58% del sexo masculino y con un bajo nivel educativo. La media de índice de masa corporal fue 23,5 ± 5 k/m2 y 58% no tenía antecedente familiar de hipertensión arterial. La media de cifras de presión arterial fueron 150 mmHg para la sistólica y 100 mmHg para la diastólica. La hipertensión arterial secundaria se detectó en 86% de la muestra. La etiología más frecuente fue la enfermedad renal parenquimatosa (86%), de los cuales 89% padecía enfermedad renal crónica y nefritis lúpica. La frecuencia de daño de órgano blanco fue de 86%, el fondo de ojo era anormal en 8%, 46% tenía hipertrofia ventricular izquierda por electrocardiograma y 58% por ecocardiograma, 78% tenía alteración de la arquitectura normal renal por ecografía, 57% presentaba creatininemia elevada, 76% trazas de proteínas en orina tomada al azar y 80% proteinuria de 24 hs elevada. Conclusión: la forma prevalente de hipertensión arterial en los adultos jóvenes estudiados fue la secundaria, con leve predominio del sexo masculino, con normopeso y sin antecedente familiar. La principal causa fue la enfermedad renal parenquimatosa. Más de la mitad de los casos presentó hipertrofia ventricular izquierda y proteinuria elevada.

ABSTRACT Introduction: High blood pressure is the main risk factor for the development of cardiovascular diseases. Its detection in young adults makes necessary to look for a secondary and potentially correctable cause. Objective: To determine the clinical characteristics of arterial hypertension in adults under 30 years of age who attended the Hospital Nacional of Itauguá, Paraguay, in the period 2019-2021. Subjects and methods: Retrospective observational cross-sectional descriptive study which included 50 patients between 18 and 30 years of age with arterial hypertension. The research was approved by the Ethics Committee of the National University of Itapúa, Paraguay. Results: The mean age was 23±4 years, most patients were from urban areas, 58% was male and had a low educational level. The mean body mass index was 23.5±5 k/m2 and 58% had no family history of arterial hypertension. The mean blood pressure values were 150 mmHg for systolic and 100 mmHg for diastolic. Secondary arterial hypertension was detected in 86% of the sample. The most frequent etiology was parenchymal kidney disease (86%), of which 89% had chronic kidney disease and lupus nephritis. The frequency of target organ damage was 86%, the eye fundus was abnormal in 8%, 46% had left ventricular hypertrophy by electrocardiogram and 58% by echocardiogram, 78% had abnormal renal architecture by ultrasound, 57% had elevated creatininemia, 76% trace protein in randomly collected urine, and 80% elevated 24-hour proteinuria. Conclusion: The prevalent form of arterial hypertension in the young adults studied was secondary, with a slight predominance of males, with normal weight and without family history. The main cause was renal parenchymal disease. More than half of the cases presented left ventricular hypertrophy and high proteinuria.

Morphological study of the spectrum of lesions encountered in the heart and coronaries on autopsy

Background and Objectives: This research study has been carried out to study the various morphological changes in the heart in medicolegal autopsies and to compile data for the frequency of heart diseases found in autopsy cases. Methods: The present study comprised 430 medicolegal autopsy cases. Details of the cases were entered in a proforma prepared for the study. The heart was fixed in 10% formalin, and then, opened along the flow of blood. The findings were based on gross and microscopic examinations. Results: Out of 430 adult hearts, 211 (49.0%) cases showed 253 cardiac lesions; 35 (8.1%) cases of coronary atherosclerosis and 196 (45.5%) aortic atherosclerosis were noted; 7 cases of ischemic lesions (1.6%) were identified; 12 (2.7%) cases of hypertrophy; 2 cases of valve calcification; and 1 of cardiomyopathy were noted. Conclusion: In the present study, among medicolegal autopsies, atherosclerotic disease was the most frequently encountered lesion followed by hypertrophy. The incidence of atherosclerosis was found to be 47.6%. This study shows the high prevalence of atherosclerosis in the population.

Hipertensão arterial sistêmica e hipertrofia ventricular esquerda: correlação entre parâmetros eletro-ecocardiográficos / Hypertension and left ventricle hypertrophy: correlation between ecg and echocardiographic parameters

Fundamento: A Hipertensão Arterial Sistêmica (HAS) acomete mais de 35% da população brasileira e tende a trazer repercussões cardíacas, entre elas a hipertrofia ventricular esquerda (HVE). O eletrocardiograma (ECG) é um exame indicado na investigação de possíveis repercussões cardiológicas em pacientes hipertensos, podendo apresentar alterações sugestivas de HVE. Objetivo: Avaliar sinais da presença de HVE em indivíduos hipertensos através do ECG e comparar com achados de ecocardiograma transtorácico (ETT). Método: Estudo transversal retrospectivo. Foram revisados prontuários eletrônicos de 159 pacientes (65,2±9,8 anos) diagnosticados com HAS, com e sem HVE ao ETT. Os resultados dos exames ECG e ETT foram comparados para avaliar a sensibilidade e especificidade do ECG em relação ao ETT. Nesta análise, foram utilizados os critérios de Sokolow-Lyon e Cornell no ECG e a relação de massa indexada do ventrículo esquerdo (VE) e espessura relativa de parede ao ETT, para avaliação do padrão geométrico do VE (PGV). Resultados: Identificamos 128 pacientes hipertensos com HVE e 31 sem alterações geométricas. A sensibilidade e especificidade para identificar HVE ao ECG foram de 31% e 90%, respectivamente, quando considerado o critério de Sokolow-Lyon conjuntamente com o critério de Cornell. Conclusão: A melhor análise de HVE ao ECG deve considerar a associação dos critérios de Sokolow-Lyon e Cornell. Apesar de apresentar uma sensibilidade menor que o ETT, o ECG possui alta especificidade e continua sendo uma alternativa importante inicial para o diagnóstico de HVE em indivíduos hipertensos

Introduction: Hipertension is a condition that affects more than 35% of the brazilian population and can lead to cardiac repercussions such as left ventricle hypertrophy (LVH). The electrocardiogram (ECG) is used in the investigation of possible cardiac repercussions in hypertensive patients, as it may indicate alterations that suggest LVH. Objective: To evaluate signs of LVH in hypertensive patients through ECG and comparing with findings from transthoracic echocardiogram (TTE). Methods: Transversal retrospective study. One hundred fifty nine electronic medical records of hypertensive patients (65,2 ± 9,8 years), with and without LVH at the TTE, were selected. Results of the ECG and TTE were compared to assess the sensitivity and specificity of the ECG in relation to TTE. Sokolow-Lyon and Cornell criteria were applied on ECG and left ventricular mass index and relative wall thickness on TTE to assess left ventricular geometry. Results: We identified 128 hypertensive patients with abnormal left ventricular geometry and 31 hypertensive patients without geometric abnormalities. The sensitivity and specificity for LVH on ECG were 31% and 90%, respectively, when considering both the Sokolow-Lyon and the Cornell criteria. Conclusion: the best way to evaluate LVH on the ECG is by using both Sokolow-yon and Cornell criteria. Despite having a lower sensitivity than TTE, ECG has high specificity and continues to be an important initial alternative to be used in the assessment of LVH in hypertensive patients

A study on cardiovascular parameters in athlete and non-athlete adult males

Background: An old proverb teaches us “Good things come to those who sweat.” Apart from its philosophical and metaphysical meaning, it is also true by words. Many previous studies also supported this view, but study in the rural part of Bengal in this field had hardly been undertaken before. Aim and Objectives: The objective of the study was to find any difference in cardiovascular parameters, for example, arterial blood pressure, heart rate; to compare the body mass index (BMI), skin fold thickness among athletes and non-athlete adult male volunteers; and also to identify any difference in resting electrocardiogram (ECG) patterns between those two groups. Materials and Methods: The present article is a scientific validation of this statement. In technical terms, this is an institutional ethics committee certified case-control study conducted in Burdwan and Birbhum districts of West Bengal with 75 non-athlete people and 75 athletes. Results: With the help of modern diagnostic tools and rigorous statistical methods, it was found that there are significant differences (P < 0.00001) between these two groups in terms of waist circumference, abdominal skin fold thickness, BMI, diastolic blood pressure (BP), and left ventricular hypertrophy by ECG. From existing medical knowledge-base, this finding can immediately be interpreted as having higher risk factors of coronary heart diseases for non-athlete people. Conclusion: Based on these findings, authors recommended that a sedentary lifestyle should be avoided to get rid of hypertension and obesity and stay away from fatal coronary heart diseases. Regular Physical activity and healthy diet can help us to live a long and disease-free life. This article speaks nothing new and surprising but confirms an age-old truth with modern scientific experiments, methods, and techniques which is a need of the hour.

Mecânica Atrial na Cardiomiopatia Hipertrófica: Discriminando Hipertrofia de Fibrose Ventricular / Atrial Mechanics in Hypertrophic Cardiomyopathy: Discriminating between Ventricular Hypertrophy and Fibrosis

Resumo Fundamento A cardiomiopatia hipertrófica (CMH) e a hipertrofia ventricular esquerda (HVE) secundária à hipertensão arterial sistêmica (HAS) podem estar associadas a anormalidades funcionais do átrio esquerdo (AE). Objetivos Caracterizar a mecânica do AE na CMH e na HAS e avaliar qualquer correlação com a extensão da fibrose ventricular esquerda medida por ressonância magnética cardíaca (RMC) em pacientes com CMH. Métodos A função longitudinal do AE derivada do ecocardiograma bidimensional com speckle tracking foi adquirida a partir de cortes apicais de 60 pacientes com CMH e 34 indivíduos controles, pareados por idade. Pacientes com CMH também foram submetidos à RMC, com medida da extensão do realce tardio por gadolínio. A associação com parâmetros de strain do AE foi analisada. Valores p < 0,05 foram definidos como estatisticamente significativos. Resultados A média da fração de ejeção do ventrículo esquerdo não foi diferente entre os grupos. A razão E/e' estava comprometida no grupo CMH e preservada no grupo controle. A mecânica do AE estava significativamente reduzida na CMH em comparação aos pacientes com HAS. O strain rate do AE nas fases de reservatório (SRrAE) e na fase contrátil (SRctAE) foram os melhores parâmetros de discriminação de CMH com uma área sob a curva (AUC) de 0,8, seguido do strain do AE na fase de reservatório (SrAE) (AUC 0,76). O SRrAE e o SRctAE apresentaram elevada especificidade (89% e 91%, respectivamente), e o SrAE apresentou sensibilidade de 80%. Um decréscimo de 2,79% no strain rate do AE na fase de condução (SRcdAE) foi preditor de um aumento de 1 cm na extensão do RT pelo gadolínio (r2=0,42, β 2,79, p=0,027). Conclusões O SRrAE e o SRctAE foram os melhores fatores de discriminação de HVE secundária à CMH. O SRcdAE foi preditor do grau de fibrose ventricular esquerda avaliada por RMC. Esses achados sugerem que a mecânica do AE pode ser um potencial preditor de gravidade de doença na CMH.

Abstract Background Hypertrophic cardiomyopathy (HCM) and left ventricular hypertrophy (LVH) secondary to systemic hypertension (HTN) may be associated with left atrial (LA) functional abnormalities. Objectives We aimed to characterize LA mechanics in HCM and HTN and determine any correlation with the extent of left ventricular (LV) fibrosis measured by cardiac magnetic resonance (CMR) in HCM patients. Methods Two-dimensional speckle tracking-derived longitudinal LA function was acquired from apical views in 60 HCM patients, 60 HTN patients, and 34 age-matched controls. HCM patients also underwent CMR, with measurement of late gadolinium enhancement (LGE) extension. Association with LA strain parameters was analyzed. Statistical significance was set at p<0.05. Results Mean LV ejection fraction was not different between the groups. The E/e' ratio was impaired in the HCM group and preserved in the control group. LA mechanics was significantly reduced in HCM, compared to the HTN group. LA strain rate in reservoir (LASRr) and in contractile (LASRct) phases were the best discriminators of HCM, with an area under the curve (AUC) of 0.8, followed by LA strain in reservoir phase (LASr) (AUC 0.76). LASRr and LASR-ct had high specificity (89% and 91%, respectively) and LASr had sensitivity of 80%. A decrease in 2.79% of LA strain rate in conduit phase (LASRcd) predicted an increase of 1cm in LGE extension (r2=0.42, β 2.79, p=0.027). Conclusions LASRr and LASRct were the best discriminators for LVH secondary to HCM. LASRcd predicted the degree of LV fibrosis assessed by CMR. These findings suggest that LA mechanics is a potential predictor of disease severity in HCM.

Network Meta-analysis of oral blood-activating and stasis-removing Chinese patent medicines in treatment of hypertensive left ventricular hypertrophy / 中国中药杂志

This study was designed to assess the clinical efficacy of oral blood-activating and stasis-removing Chinese patent medicines in treating hypertensive left ventricular hypertrophy(LVH) based on network Meta-analysis. The clinical randomized controlled trials(RCTs) concerning the treatment of hypertensive LVH with oral blood-activating and stasis-removing Chinese patent medicines were retrieved from CNKI, Wanfang, VIP, SinoMed, PubMed, Web of Science, and Cochrane Library from their inception to September 2021. Two researchers independently completed the literature screening, data extraction, and quality evaluation. The data were then analyzed by RevMan 5.3, Stata 15.1, and ADDIS 1.16.8. Finally, a total of 31 RCTs were included, involving 3 001 patients and four oral blood-activating and stasis-removing Chinese patent medicines. In terms of the alleviation of heart damage, the Chinese patent medicines combined with conventional western medicine groups were superior to the conventional western medicine groups in lo-wering the left ventricular mass index(LVMI). There was no significant difference in LVMI, left ventricular ejection fraction(LVEF), or the ratio of early diastolic peak flow velocity to late diastolic peak flow velocity(E/A) between different Chinese patent medicines combined with conventional western medicine groups. Xinnao Shutong Capsules/Tablets combined with conventional western medicine had the best efficacy in reducing LVMI and elevating LVEF, while Xinkeshu Capsules/Tablets combined with conventional western medicine had the best effect in improving E/A. In the control of blood pressure, when all Chinese patent medicines except for Xinnao Shutong Capsules/Tablets were combined with conventional western medicine, the resulting systolic blood pressure(SBP) and diastolic blood pressure(DBP) were significantly lower than those in the conventional western medicine group. Xinkeshu Capsules/Tablets combined with conventional western medicine produced the best effect in reducing SBP and DBP, followed by Xinnao Shutong Capsules/Tablets. In terms of safety, no serious adverse reactions occurred in all trials. The four oral blood-activating and stasis-removing Chinese patent medicines included in this study exhibited obvious advantages in the treatment of hypertensive LVH when they were combined with conventional western medicine, with the best effects observed in the Xinnao Shutong Capsules/Tablets combined with conventional western medicine group. However, due to the limitation of the quantity and quality of the included articles, the conclusion of this study still needs to be verified by more high-quality, multi-center, and large-sample RCTs.

Clinical study on Tianma Gouteng Decoction combined with amlodipine besylate tablets in the treatment of hypertension of liver-yang hyperactivity with left ventricular hypertrophy / 国际中医中药杂志

Objective:To evaluate the clinical efficacy of Tianma Gouteng Decoction combined with amlodipine besylate tabletsin treating patients with hypertension of liver-yang hyperactivity complicated with left ventricular hypertrophy (LVH).Methods:A total of 80 patients with hypertension of liver-yang hyperactivity complicated with LVH, who met the inclusion criteria and were treated in the hospital between June 2019 and January 2021, were divided into two groups, with 40 in each group according to the random number table method. The control group was treated with amlodipine besylate tablets orally, and the observation group was treated with granules of Tianma Gouteng Decoction on the basis of the control group. Both groups were treated for 2 months. Before and after treatment, the TCM syndromes were scored and the office blood pressure was measured with sphygmomanometer, and color Doppler ultrasonic diagnostic apparatus was adopted to detect the interventricular septum thickness (IVST), left ventricular posterior wall thickness (PWT), left ventricular end diastolic diameter (LVDd) and left ventricular mass index (LVMI), and the levels of homocysteine (Hcy) and Cystatin C (Cys-C) were determined by ELISA and the level of uric acid (UA) was determined by fully automatic biochemical analyzer. The clinical efficacy was evaluated.Results:The total effective rate was 95.0% (38/40) in the observation group and that of the control group was 77.5% (31/40) ( χ2=5.17, P=0.023). After treatment, the scores of dizziness, headache, insomnia and dreaminess and vexation and forgetfulness and total score of the observation group were significantly lower than those in the control group ( t=10.06, 14.47, 15.47, 8.74, 14.50, all Ps<0.001), and the systolic blood pressure and diastolic blood pressure were significantly lower than those in the control group ( t=6.30, 8.79, P<0.001). The IVST [(9.36±1.32) mm vs. (11.23±1.07) mm, t=6.96], PWT [(8.89±1.14)mm vs. (10.03±1.02)mm, t=4.71], LVDd[(40.36±4.29) mm vs. (47.62±4.19) mm, t=7.66] and LVMI [(112.39±22.29) g/m 2vs. (148.26± 21.39) g/m 2, t=7.34] were significantly lower compared to the control group ( P<0.01). The levels of Hcy[(12.87±3.11) μmol/L vs. (19.85±3.67) μmol/L, t=9.18], UA [(276.29±19.56) μmol/L vs. (338.52±17.07) μmol/L, t=16.65] and Cys-C [(0.86±0.15) mg/L vs. (1.10±0.17) mg/L, t=6.70] were significantly lower than those in the control group ( P<0.01). Conclusion:The Tianma Gouteng Decoction can improve the clinical symptoms, control the blood pressure, relieve the left ventricular hypertrophy and improve the renal function of patients with hypertension of liver-yang hyperactivity complicated with LVH.

Prevalence of echocardiographic left ventricular hypertrophy among hypertensives in a tertiary health institution in Nigeria

OBJECTIVE: Systemic hypertension remains an important risk factors for cardiovascular diseases and a major global public health problem. Left ventricular hypertrophy (LVH) is a recognized complication of hypertension and strongly predicts cardiovascular morbidity and mortality. In Nigeria, few studies evaluated the role of echocardiography in the diagnosis of LVHs among hypertensives. This study sets out to determine the prevalence of LVH among hypertensives as determined by echocardiography.MATERIAL AND METHODS: One hundred and seventy-eight hypertensives and eighty-nine age and sex-matched controls were recruited consecutively into the study. They all had echocardiography done to determine which among had LVH. The partition value for LVH for hypertensives was determined using the 97th percentile of the left ventricular mass for controls as a cutoff point. RESULTS:Echocardiographic determined the prevalence of LVH among hypertensives was 32.4%.CONCLUSION:The echocardiographic prevalence of LVH was 32.4% in the study population. This is a significant proportion among the study population considering the clinical impact of LVH among patients with hypertension.

Análise de Custo-Efetividade da Terapia com Evolocumabe em Pacientes com Alto Risco de Eventos Cardiovasculares no Contexto do SUS - Brasil / Cost-Effectiveness Analysis of Evolocumab Therapy in Patients at High Risk of Cardiovascular Events in the Context of the Brazilian Unified Health System

Resumo Fundamento: Em associação às estatinas, os inibidores da pró-proteína convertase subtilisina/kexina tipo 9 (PCSK9) demonstraram ser eficazes na redução de eventos cardiovasculares em pacientes de alto risco. Objetivo: Analisar a custo-efetividade da implementação de evolocumabe para pacientes com alto risco de eventos cardiovasculares no contexto do Sistema Único de Saúde (SUS) no Brasil. Métodos: Um modelo de Markov foi utilizado, baseando-se em uma amostra ambulatorial de pacientes com doença arterial coronariana. Os desfechos primários analisados foram infarto agudo do miocárdio, acidente vascular cerebral isquêmico (AVCi), revascularização do miocárdio e morte cardiovascular. O resultado foi expresso por meio da razão de custo-efetividade incremental (RCEI), considerando-se uma taxa de desconto de 5% ao ano, e uma análise de sensibilidade foi realizada, tendo em vista a imprecisão de valores. Resultados: Selecionaram-se 61 pacientes com risco cardiovascular estimado em 35% em 10 anos, se em uso de atorvastatina 80mg/dia, e em 22,75%, se adicionado o evolocumabe. O custo global por paciente no período de 10 anos foi de R$ 46.522,44 no grupo em monoterapia com atorvastatina versus R$ 236.141,85 na terapia combinada, com uma efetividade global de 0,54 e 0,73, respectivamente. Isso resultou em uma RCEI R$ 1.011.188,07 (R$ 864.498,95 a R$ 1.296.748,43) por desfecho cardiovascular evitado. Conclusões: Apesar de não existirem padrões nacionais para custo-efetividade, os dados encontrados sugerem que a estratégia de associação do evolocumabe à terapia com estatina não é, no momento, custo-efetiva.

Abstract Background: Hypertrophic cardiomyopathy (HCM) and left ventricular hypertrophy (LVH) secondary to systemic hypertension (HTN) may be associated with left atrial (LA) functional abnormalities. Objectives: We aimed to characterize LA mechanics in HCM and HTN and determine any correlation with the extent of left ventricular (LV) fibrosis measured by cardiac magnetic resonance (CMR) in HCM patients. Methods: Two-dimensional speckle tracking-derived longitudinal LA function was acquired from apical views in 60 HCM patients, 60 HTN patients, and 34 age-matched controls. HCM patients also underwent CMR, with measurement of late gadolinium enhancement (LGE) extension. Association with LA strain parameters was analyzed. Statistical significance was set at p<0.05. Results: Mean LV ejection fraction was not different between the groups. The E/e' ratio was impaired in the HCM group and preserved in the control group. LA mechanics was significantly reduced in HCM, compared to the HTN group. LA strain rate in reservoir (LASRr) and in contractile (LASRct) phases were the best discriminators of HCM, with an area under the curve (AUC) of 0.8, followed by LA strain in reservoir phase (LASr) (AUC 0.76). LASRr and LASR-ct had high specificity (89% and 91%, respectively) and LASr had sensitivity of 80%. A decrease in 2.79% of LA strain rate in conduit phase (LASRcd) predicted an increase of 1cm in LGE extension (r2=0.42, β 2.79, p=0.027). Conclusions: LASRr and LASRct were the best discriminators for LVH secondary to HCM. LASRcd predicted the degree of LV fibrosis assessed by CMR. These findings suggest that LA mechanics is a potential predictor of disease severity in HCM.

Circulating MicroRNA-30e Predicts Left Ventricular Hypertrophy In Essential Hypertensive Patients

ABSTRACT Background: The biomarker for left ventricular hypertrophy (LVH) in patients with essential hypertension (EH) remains an unmet clinical need. The microRNA-30 (miR-30) family has been associated with LVH in cellular and animal studies, but not in a clinical setting. Objective: The objective of the study was to investigate the clinical significance of circulating levels of miR-30 family as a biomarker for LVH in EH patients. Methods: A total of 239 EH patients and 239 healthy controls were enrolled in this study. Circulating levels of miR-30 family members, namely, miR-30a, miR-30b, miR-30c-1, miR-30c-2, miR-30d, and miR-30e, were evaluated using real-time polymerase chain reaction assays. Results: The circulating miR-30a, miR-30b, and miR-30e were significantly reduced in EH patients in contrast to controls. EH patients with LVH (EH-LVH) had substantially lower circulating miR-30b and miR-30e levels compared to EH patients without LVH (EH-nLVH). Moreover, the expression levels of miR-30b and miR-30e were positively related to LVMI, respectively. Receiver operating curve analysis showed that circulating miR-30e levels distinguished EH patients from controls, and EH-LVH from EH-nLVH patients. Logistic regression analysis identified the circulating miR-30e as a risk factor for LVH in EH patients. Conclusion: Circulating miR-30e level can be used as a biomarker in distinguishing EH-LVH from EH-nLVH. A further prospective study is warranted to validate this finding.

Analyzing the clinical phenotype of heart disease caused by the double mutation of p.Gly743Arg and p.Glu1389Lys carrying the myosin heavy chain gene / 中华危重病急救医学

Objective:To investigate the relationship between double mutations of myosin heavy chain gene (MYH6) p.Gly743Arg and p.Glu1389Lys and the cardiac phenotype.Methods:Patients carrying double mutations in the MYH6 gene p.Gly743Arg and p.Glu1389Lys were screened from 52 unrelated left ventricular hypertrophy (LVH) who were admitted to the Second Hospital of Chongqing Medical University from 2015 to 2020, and the genetic testing of peripheral blood of patients by second-generation whole-exome sequencing assay technology and genomic DNA of their family members Sanger sequencing was performed to validate the genomic DNA of the family members. The cardiac phenotype was evaluated by electrocardiogram, coronary computed tomography angiography (CTA), echocardiography, and cardiac magnetic resonance imaging (MRI) as adjuncts.Results:All whole-exome gene were detected in 52 unrelated patients with LVH, of which 1 patient (1.9%) had double mutations in MYH6 gene p.Gly743Arg and p.Glu1389Lys (proband). Two members of the maternal line of this patient carried p.Glu1389Lys mutation, but there was no obvious clinical phenotype. Two members of the paternal line carried p.Gly743Arg mutation and had obvious clinical phenotype of bradycardia, but there was no LVH. The male proband, aged 21 years old, presented with LVH and sinus bradycardia but no coronary artery stenosis on CTA before treatment, MRI showed that the left ventricular end diastolic diameter was 58 mm. After treatment with angiotensin receptor-enkephalinase inhibitor (ARNI), electrocardiogram showed that the heart rate increased significantly (from 43 bpm to 72 bpm). Echocardiography showed that the left ventricular end diastolic diameter decreased significantly (from 60 mm to 49 mm).Conclusions:The p.Glu1389Lys mutation of the MYH6 gene may not manifest the phenotype of heart disease. MYH6 gene p.Gly743Arg mutation may be manifested asymptomatic sinus bradycardia, but there is no LVH phenotype. The cardiac disease phenotype caused by the double mutations of p.Gly743Arg and p.Glu1389Lys in the MYH6 gene is more obvious. Asymptomatic LVH and sinus bradycardia can appear in adolescence, but the LVH phenotype can be reversed in a short period of time after ARNI treatment.