Cuando la genética estaba oculta: miocardiopatía hipertrófica por variante MYBPC3 c. 1102A>T (p.Lys368*) refractaria a manejo médico: reporte de un caso / When genetics were hidden: hypertrophic cardiomyopathy due to MYBPC3 c. 1102A>T (p.Lys368*) variant refractory to medical management: case report / Quando a genética estava oculta: cardiomiopatia hipertrófica por variante MYBPC3 c. 1102A>T (p.Lys368*) refractária à manejo médico: reporte de um caso

La miocardiopatía hipertrófica es cada vez más diagnosticada. Es una condición genética que genera hipertrofia miocárdica, fibrosis, isquemia y apoptosis con obstrucción del tracto de salida del ventrículo izquierdo. Puede generar síncope, falla cardíaca y muerte súbita. El tratamiento es farmacológico y se requiere cirugía si hay refractariedad. Se presenta un caso de miocardiopatía hipertrófica asociada a variante genética patogénica en un paciente no respondedor a manejo médico óptimo. La importancia de este artículo radica en lo determinante que es la genética para el abordaje diagnóstico y el establecimiento del origen y pronóstico de esta enfermedad.

Hypertrophic cardiomyopathy is increasingly diagnosed. It is a genetic condition that leads to myocardial hypertrophy, fibrosis, ischemia, and apoptosis with obstruction of the left ventricular outflow tract. It can result in syncope, heart failure, and sudden death. Treatment is pharmacological, and surgery is required in cases of refractoriness. A case of hypertrophic cardiomyopathy associated with a pathogenic genetic variant is presented in a patient unresponsive to optimal medical management. The importance of this article lies in how crucial genetics is for the proper diagnostic approach and the establishment of the origin and prognosis of this disease.

A miocardiopatia hipertrófica está sendo diagnosticada cada vez mais. É uma condição genética que leva à hipertrofia miocárdica, fibrose, isquemia e apoptose com obstrução do trato de saída do ventrículo esquerdo. Pode resultar em síncope, insuficiência cardíaca e morte súbita. O tratamento é farmacológico e a cirurgia é necessária em casos de refratariedade. Apresenta-se um caso de miocardiopatia hipertrófica associada a uma variante genética patogênica em um paciente não responsivo ao manejo médico ótimo. A importância deste artigo reside na determinante genética para a abordagem diagnóstica adequada e para o estabelecimento da origem e prognóstico desta doença.

Enzima convertidora de angiotensina homóloga (ECA2) y actividad de ROCK en la falla cardíaca experimental / ACE 2 and ROCK activity in experimental congestive heart failure

Antecedentes: La ECA2 ha mostrado ser un regulador esencial de la funcionalidad cardíaca. En un modelo experimental de insuficiencia cardíaca (IC) con Fier, modelo de coartación de aorta (COA), se encontró activación de la vía Rho-kinasa. La inhibición de esta vía con fasudil no mejoró el remodelado cardíaco ni la disfunción sistólica. Se desconoce en este modelo, si el deterioro de la función cardíaca y activación de la vía rho-kinasa se asocia con una disminución de la ECA2 cardíaca y si la inhibición de Rho-kinasa tiene un efecto sobre la expresión de ECA2. Objetivo: Nuestro objetivo es determinar si en la falla cardaca experimental por coartación aórtica, los niveles proteicos de ECA2 en el miocardio se asocian a disfunción sistólica y cual es su interacción con la actividad de ROCK en el miocardio. Métodos: Ratones C57BL6J machos de 7-8 semanas se randomizaron en 3 grupos experimentales. Grupo COA por anudación de la aorta + vehículo; Grupo COA + Fasudil (100 mg/Kg día) por bomba osmótica desde la semana 5 post-cirugía; y grupo control o Sham. Se determinaron las dimensiones y función cardíaca por ecocardiografía. Posterior a la eutanasia, se determinaron los niveles de ECA2 del VI por Western-blot y actividad de la Rho-kinasa Resultados: En los grupos COA+vehículo y COA-FAS hubo deterioro de la función cardíaca, reflejada por la reducción de la FE (47,9 ± 1,53 y 45,5 ± 2,10, p < 0,05, respectivamente) versus SHAM (68,6 ± 1,19). Además, aumentaron las dimensiones cardíacas y hubo desarrollo de hipertrofia (0,53 ± 0,02 / 0,53 ± 0,01, p < 0,05) medida por aumento de la masa cardíaca relativa respecto del grupo SHAM (0,40 ± 0,01). En los grupos COA+vehículo y COA-FAS se encontró una disminución significativa del 35% en la expresión de ECA2 cardíaca respecto al grupo control. Conclusiones: La disfunción sistólica por coartación aórtica se asocia con aumento de la actividad de Rho-kinasa y significativa disminución de la expresión de ECA2. La inhibición de Rho-kinasa no mejoró el remodelado cardíaco, la disfunción sistólica y tampoco modificó los niveles de ECA2 cardíaca.

Background: ACE2 has been described as an essential regulator of cardiac function. In an experimental model of heart failure (HF) and heart failure reduced ejection fraction (HFrEF), the aortic coarctation (COA) model, activation of the Rho-kinase pathway of cardiac remodeling was found. Inhibition of this pathway did not improve cardiac remodeling or systolic ventricular dysfunction. It is unknown in this model whether the impairment of cardiac function and activation of the rho-kinase pathway is associated with a decrease in ACE2 and whether rho-kinase inhibition has an effect on ACE2 expression. Objective: To determine if in experimental heart failure due to aortic coarctation, ACE2 protein levels in the myocardium are associated with systolic dysfunction and what is its interaction with ROCK activity in the myocardium. Methods: Male C57BL6J mice aged 7-8 weeks were divided into 3 groups and anesthetized: One group underwent COA+ vehicle; A second group COA + Fasudil (100 mg/Kg/d) by osmotic pump from week 5 post-surgery and; the third group, control(SHAM). Echocardiograms were performed to determine cardiac dimensions and systolic function. Rats were then euthanized. Ventricular expression of ACE2, activity of the Rho-kinase pathway by MYPT-1 phosphorylation, relative cardiac mass, area and perimeter of cardiomyocytes were determined by Western blot. Results: In both COA+vehicle and COA+FAS groups there was deterioration of cardiac function, reflected in the reduction of EF (47.9 ± 1.53 and 45.5 ± 2.10, p < 0.05, respectively) versus the SHAM group (68.6 ± 1.19). In addition, cardiac dimensions and hypertrophy increased (0.53 ± 0.02 / 0.53 ± 0.01, p < 0.05) due to increased relative cardiac mass compared to the SHAM group (0.40 ± 0.01). In the COA+vehicle and COA+FAS groups a significant decrease of 35% in cardiac ACE2 expression was found compared to the control group. Conclusions: Systolic dysfunction due to aortic coarctation is associated with increased Rhokinase activity and a significant decrease in ACE2 expression. Rho-kinase inhibition did not improve cardiac remodeling, systolic dysfunction, nor did it change cardiac ACE2 levels.

The role of inflammation in heart failure with preserved ejection fraction / 生理学报

Heart failure with preserved ejection fraction (HFpEF) is a type of heart failure characterized by left ventricular diastolic dysfunction with preserved ejection fraction. With the aging of the population and the increasing prevalence of metabolic diseases, such as hypertension, obesity and diabetes, the prevalence of HFpEF is increasing. Compared with heart failure with reduced ejection fraction (HFrEF), conventional anti-heart failure drugs failed to reduce the mortality in HFpEF due to the complex pathophysiological mechanism and multiple comorbidities of HFpEF. It is known that the main changes of cardiac structure of in HFpEF are cardiac hypertrophy, myocardial fibrosis and left ventricular hypertrophy, and HFpEF is commonly associated with obesity, diabetes, hypertension, renal dysfunction and other diseases, but how these comorbidities cause structural and functional damage to the heart is not completely clear. Recent studies have shown that immune inflammatory response plays a vital role in the progression of HFpEF. This review focuses on the latest research progress in the role of inflammation in the process of HFpEF and the potential application of anti-inflammatory therapy in HFpEF, hoping to provide new research ideas and theoretical basis for the clinical prevention and treatment in HFpEF.

Clinical features and risk factors of left ventricular hypertrophy in children with primary hypertension / 中华儿科杂志

Objective: To assess the clinical features and relative factors of left ventricular hypertrophy (LVH) in children with primary hypertension. Methods: In this retrospective cohort study, 430 children diagnosed with primary hypertension in Children's Hospital, Capital Institute of Pediatrics from January 2019 to September 2022 were enrolled. Their clinical data was analyzed and LVH was assessed by echocardiography. According to left ventricular geometry, these children were assigned to the LVH group and normal geometry group. General conditions, laboratory indicators and ambulatory blood pressure parameters between two groups were compared by independent sample t-test or Mann-Whitney U test. Spearman correlation coefficient was used to analyze the correlation between LVH and clinical indicators including blood pressure, biochemical and metabolic indicators. The independent risk factors of LVH were analyzed by multivariable logistic regression. The receiver operating characteristic (ROC) curve was used to explore the value of risk factors in the diagnosis of LVH. Results: Among the 430 children with primary hypertension, 342 (79.5%) were males and 88 (20.5%) females. Their age was (12.6±2.3) years, and 123 children (28.6%) of them had LVH. Body mass index (BMI) ((30.0±5.2) vs. (26.2±4.3) kg/m2), ratio of stage 2 hypertension (75.6% (93/123) vs. 59.6% (183/307)), 24-hour systolic blood pressure (24 h SBP)((131±10) vs. (128±10) mmHg,1 mmHg=0.133 kPa), daytime systolic blood pressure (SBP) ((135±11) vs. (131±11) mmHg), nighttime SBP ((128±11) vs. (123±10) mmHg), cholesterol level ((4.0±0.7) vs. (3.9±0.7) mmol/L), serum uric acid level ((447±81) vs. (426±91) μmol/L) and incidence of hyperinsulinemia (69.9% (86/123) vs.59.0% (181/307)) were significantly elevated in the LVH group compared with those in the normal geometry group (all P<0.05). There were more patients with a disease course over 5 years in the LVH group than in the normal geometry group, with a statistically significant difference (χ2=8.90,P=0.031). Spearman correlation analysis showed that BMI, 24 h SBP, daytime SBP, nighttime SBP, triglyceride, uric acid, and serum sodium level were positively correlated with LVMI (r=0.43, 0.20, 0.18, 0.18, 0.18, 0.16, and 0.12, all P<0.05). BMI, hyperinsulinemia, and cholesterol level were positively correlated with relative wall thickness (RWT) (r=0.22, 0.12, and 0.16, all P<0.05). The multivariate logistic regression analysis showed that BMI (OR=1.17, 95%CI 1.10-1.25) and 24 h SBP (OR=1.04, 95%CI 1.01-1.08) were the independent risk factors for LVH (both P<0.05). The area under the receiver operator characteristic curve, combined with BMI and 24 h SBP, was 0.72 (95%CI 0.67-0.77, P<0.05), with a sensitivity and specificity of 71.5% and 64.8%, respectively. Conclusions: BMI and 24 h SBP are the independent risk factors for LVH in children with primary hypertension, and the combination of BMI and 24 h SBP has an acceptable diagnostic value for LVH. Early monitoring of these indexes is necessary to predict preclinical cardiac damage.

Analysis of etiology and complications in children with stage 5 chronic kidney disease / 中华儿科杂志

Objective: To investigate the etiology, complications, and prognostic factors of stage 5 chronic kidney disease (CKD5) in children. Methods: A case series study was conducted to retrospectively analyze the general situation, clinical manifestations, laboratory tests, genetic testing, and follow-up data (until October 2022) of 174 children with CKD5 who were diagnosed and hospitalized at the Children's Hospital of Chongqing Medical University from April 2012 to April 2021. The characteristics of complications in the children were compared based on age, gender, and etiology. Based on the presence or absence of left ventricular hypertrophy (LVH), patients were divided into LVH group and non LVH group for analyzing the influencing factors of cardiovascular disease. Patients were also divided into death group and survival group, peritoneal dialysis group and hemodialysis group based on the follow-up data for analyzing the prognostic factors. The chi-square test, independent sample t-test, Fisher exact probability test, Mann-Whitney U test and Kruskal Wallis test were used to analyze data among different groups. Multivariate Logistic regression analysis was used to identify the prognostic factors. Results: A total of 174 children with CKD5 were enrolled in the study (96 boys and 78 girls), aged 11.2 (8.2, 13.0) years. Congenital kidney and urinary tract malformations (CAKUT) were the most common causes of the CKD5 (84 cases, 48.3%), followed by glomerular diseases (83 cases, 47.7%), and among which 28 cases (16.1%) were hereditary glomerular diseases. The common complications of CKD5 included anemia (98.2%, 165/168), mineral and bone disorder in chronic kidney disease (CKD-MBD) (97.7%, 170/174), lipid metabolism disorders (87.5%, 63/72), hypertension (81.4%, 127/156) and LVH (57.6%,57/99). The incidences of hypertension in primary glomerular disease were higher than that in CAKUT(93.8%(30/32) vs.73.7%(56/76),χ2=5.59,P<0.05). The incidences of hypertension in secondary glomerular disease were higher than that in CAKUT and that in hereditary kidney disease (100.0%(20/20) vs. 73.7%(56/76), 68.2%(15/22), both P<0.05). The incidence of hypocalcemia in CAKUT, primary glomerular disease, and hereditary kidney disease was higher than that in secondary glomerular disease (82.1%(69/84), 88.2%(30/34), 89.3%(25/28) vs. 47.6%(10/21), χ2=10.21, 10.75, 10.80, all P=0.001); the incidence of secondary hyperparathyroidism in women was higher than that in men (80.0%(64/80) vs. 95.0%(57/60), χ2=6.58, P=0.010). The incidence of LVH in children aged 6-<12 was higher than that in children aged 12-18 (73.5%(25/34) vs. 43.1%(22/51), χ2=7.62, P=0.006). Among 113 follow-up children, the mortality rate was 39.8% (45/113). Compared to the survival group, the children in the death group had lower hemoglobin, higher blood pressure, lower albumin, lower alkaline phosphatase and higher left ventricular mass index ((67±19) vs. (75±20) g/L, 142 (126, 154) vs. 128(113, 145) mmHg(1 mmHg=0.133 kPa), (91±21) vs. (82±22) mmHg, 32 (26, 41) vs. 40 (31, 43) g/L, 151 (82, 214) vs. 215 (129, 37) U/L, 48 (38, 66) vs. 38(32, 50) g/m2.7,t=2.03, Z=2.89, t=2.70, Z=2.49, 2.79, 2.29,all P<0.05), but no independent risk factors were identified (all P>0.05). The peritoneal dialysis group had better alleviation for anemia, low calcium, and high phosphorus than the hemodialysis group ((87±22) vs. (72±16) g/L, (1.9±0.5) vs. (1.7±0.4) mmol/L, (2.2±0.7) vs. (2.8±0.9) mmol/L, t=2.92, 2.29, 2.82, all P<0.05), and the survival rate of the peritoneal dialysis group was significantly higher than that of the hemodialysis group (77.8% (28/36) vs. 48.4% (30/62), χ2=8.14, P=0.004). Conclusions: CAKUT is the most common etiology in children with CKD 5, and anemia is the most common complication. The incidence of complications in children with CKD 5 varies with age, gender and etiology. Anemia, hypertension, hypoalbuminemia, reduced alkaline phosphatase and elevated LVMI may be the prognostic factors in children with CKD5. Peritoneal dialysis may be more beneficial for improving the long-term survival rate.

Clinical characteristics of Danon disease / 中华心血管病杂志

Objective: To review the clinical data of 7 patients with Danon disease and analyze their clinical characteristics. Methods: The medical records of 7 patients with Danon disease, who were hospitalized in Peking Union Medical College Hospital of Chinese Academy of Medical Sciences from April 2008 to July 2021, were reviewed and summarized, of which 6 cases were diagnosed as Danon disease by lysosomal-associated membrane protein-2 (LAMP-2) gene mutation detection and 1 case was diagnosed by clinicopathological features. Clinical manifestations, biochemical indexes, electrocardiogram, echocardiography, skeletal muscle and myocardial biopsy and gene detection results were analyzed, and patients received clinical follow-up after discharge. Results: Six patients were male and average age was (15.4±3.5) years and the average follow-up time was (27.7±17.0) months. The main clinical manifestations were myocardial hypertrophy (6/7), decreased myodynamia (2/7) and poor academic performance (3/7). Electrocardiogram features included pre-excitation syndrome (6/7) and left ventricular hypertrophy (7/7). Echocardiography examination evidenced myocardial hypertrophy (6/7), and left ventricular dilatation and systolic dysfunction during the disease course (1/7). The results of skeletal muscle biopsy in 6 patients were consistent with autophagy vacuolar myopathy. Subendocardial myocardial biopsy was performed in 3 patients, and a large amount of glycogen deposition with autophagosome formation was found in cardiomyocytes. LAMP-2 gene was detected in 6 patients, and missense mutations were found in all these patients. During the follow-up period, implantable cardioverter defibrillator implantation was performed in 1 patient because of high atrioventricular block 4 years after diagnosis, and there was no death or hospitalization for cardiovascular events in the other patients. Conclusion: The main clinical manifestations of Danon disease are cardiomyopathy, myopathy and mental retardation. Pre-excitation syndrome is a common electrocardiographic manifestation. Autophagy vacuoles can be seen in skeletal muscle and myocardial pathological biopsies. LAMP-2 gene mutation analysis is helpful in the diagnose of this disease.

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.

Effect and mechanism of leonurine on pressure overload-induced cardiac hypertrophy in rats / 中国中药杂志

To investigate the effects of leonurine(Leo) on abdominal aortic constriction(AAC)-induced cardiac hypertrophy in rats and its mechanism. A rat model of pressure overload-induced cardiac hypertrophy was established by AAC method. After 27-d intervention with high-dose(30 mg·kg~(-1)) and low-dose(15 mg·kg~(-1)) Leo or positive control drug losartan(5 mg·kg~(-1)), the cardiac function was evaluated by hemodynamic method, followed by the recording of left ventricular systolic pressure(LVSP), left ventricular end-diastolic pressure(LVESP), as well as the maximum rate of increase and decrease in left ventricular pressure(±dp/dt_(max)). The degree of left ventricular hypertrophy was assessed based on heart weight index(HWI) and left ventricular mass index(LVWI). Myocardial tissue changes and the myocardial cell diameter(MD) were measured after hematoxylin-eosin(HE) staining. The contents of angiotensin Ⅱ(AngⅡ) and angiotensin Ⅱ type 1 receptor(AT1 R) in myocardial tissue were detected by ELISA. The level of Ca~(2+) in myocardial tissue was determined by colorimetry. The protein expression levels of phospholipase C(PLC), inositol triphosphate(IP3), AngⅡ, and AT1 R were assayed by Western blot. Real-time quantitative PCR(qRT-PCR) was employed to determine the mRNA expression levels of β-myosin heavy chain(β-MHC), atrial natriuretic factor(ANF), AngⅡ, and AT1 R. Compared with the model group, Leo decreased the LVSP, LVEDP, HWI, LVWI and MD values, but increased ±dp/dt_(max) of the left ventricle. Meanwhile, it improved the pathological morphology of myocardial tissue, reduced cardiac hypertrophy, edema, and inflammatory cell infiltration, decreased the protein expression levels of PLC, IP3, AngⅡ, AT1 R, as well as the mRNA expression levels of β-MHC, ANF, AngⅡ, AT1 R, c-fos, and c-Myc in myocardial tissue. Leo inhibited AAC-induced cardiac hypertrophy possibly by influencing the RAS system.

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.

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.

Cardiomiopatia Hipertrófica: uma revisão pelo olhar da ressonância magnética / Hypertrophic Cardiomyopathy: a review using magnetic resonance imaging

A cardiomiopatia hipertrófica é a cardiopatia genética mais frequente na população geral e é caracterizada por uma hipertrofia ventricular esquerda assimétrica. Entretanto, as alterações fenotípicas desta cardiomiopatia vão muito além da hipertrofia ventricular, e incluem alterações do aparato valvar mitral, dos músculos papilares e do ventrículo direito. Devido à dificuldade no diagnóstico diferencial entre as múltiplas causas de hipertrofia, a ressonância magnética cardíaca vem cumprindo um papel fundamental na avaliação diagnóstica e prognóstica desta cardiomiopatia. A cineressonância magnética na definição da localização e extensão da hipertrofia, o realce tardio, na detecção das áreas de fibrose miocárdica e técnicas mais recentes como o Mapa de T1 que avalia a fibrose intersticial e o volume extracelular; e finalmente o Tissue Tracking na análise da deformação miocárdica.(AU)

Hypertrophic cardiomyopathy, the most common genetic cardiopathy in the general population, is characterized by asymmetric left ventricular hypertrophy. However, the phenotypic changes in this cardiomyopathy extend beyond ventricular hypertrophy and include changes in the mitral valve apparatus, papillary muscles, and right ventricle. Due to the difficult differential diagnosis among multiple causes of hypertrophy, cardiac magnetic resonance has played a fundamental role in its diagnostic and prognostic evaluation; magnetic cine-resonance in defining the location and extent of hypertrophy; late enhancement, in the detection of areas of myocardial fibrosis; more recent techniques such as T1 mapping that assesses interstitial fibrosis and extracellular volume; and finally tissue tracking in the analysis of myocardial deformation. (AU)

A importância do eletrocardiograma no diagnóstico da hipertrofia ventricular esquerda / The importance of the electrocardiogram in diagnosis of left ventricular hypertrophy

A hipertrofia ventricular esquerda (HVE) é um importante fator de risco cardiovascular independente de outras manifestações ou comorbidades. Consequentemente sua detecção por métodos diagnósticos de baixo custo e fácil acesso é extremamente relevante. Em pacientes hipertensos, a HVE é uma das manifestações pré-clínicas de lesão de órgão alvo mais frequente cuja identificação leva a mudança na estratificação do risco e maior agressividade no tratamento. O eletrocardiograma (ECG) é um exame de baixo custo que embora apresente baixa sensibilidade, tem alta especificidade e reprodutibilidade, e por isso é amplamente utilizado. Devido a sua alta disponibilidade do serviço público de saúde é de extrema importância a utilização deste exame como auxílio na triagem de pacientes hipertensos para a detecção HVE. Diversos critérios eletrocardiográficos, com sensibilidade baixa e especificidade alta, podem ser utilizados para a avaliação de uma possível HVE. Existe algum tipo de variabilidade dependendo da população estudada, e o aumento da faixa etária é a que mais influencia no aumento da sensibilidade e diminuição da especificidade (AU).

Left ventricular hypertrophy (LVH) is an important cardiovascular risk factor regardless of other manifestations or comorbidities. Consequently, its detection by low-cost and easily accessible diagnostic methods is extremely relevant. In hypertensive patients, LVH is one of the most frequent preclinical manifestations of target organ damage whose identification leads to a change in risk stratification and more aggressive treatment. The electrocardiogram (ECG) is a low-cost test that, despite having low sensitivity, has high specificity and reproducibility, and therefore is widely used. Due to its high availability in the public health service, it is extremely important to use this test as an aid in the screening of hypertensive patients for LVH. Several electrocardiographic criteria, with low sensitivity and high specificity, can be used to evaluate a possible LVH. There is some type of variability depending on the population studied, and the increase in age is the one that most influences the increase in sensitivity and decrease in specificity (AU).

Importância dos métodos de imagem no diagnóstico diferencial de cardiopatia hipertensiva e cardiomiopatia hipertrófica: relato de caso / The importance of imaging methods in the differential diagnosis of hypertensive cardiopathy and hypertrophic cardiomyopathy: a case report

Caso clínico de homem de 31 anos, branco, casado, cozinheiro, natural do Nepal, procedente de São Paulo há 4 anos, em acompanhamento ambulatorial após encaminhamento do pronto-socorro (PS) por angina atípica e pressão alta sic. Pela análise do prontuário verificou-se que o paciente permaneceu no PS por 48 horas e ao exame físico a ausculta cardíaca era normal e a pressão arterial (PA) 140X85 mm Hg. O eletrocardiograma (ECG) mostrava critérios eletrocardiográficos para hipertrofia ventricular esquerda (HVE) e alterações da repolarização ventricular e os marcadores de necrose miocárdica apresentavam aumentos discretos. Na consulta ambulatorial o paciente não referia queixas, a PA estava normal e foram realizados: monitorização ambulatorial da pressão arterial (MAPA) de 24 horas, ecocardiograma (ECO) e angiotomografia de coronárias. A MAPA mostrou níveis pressóricos normais (vigília e sono respectivamente 112x65 e 106x51 mmHg), o ECO não detectou HVE e a angiotomografia de coronárias não identificou obstruções coronarianas e o escore de cálcio era zero. A ressonância magnética de coração (RMC) mostrou aumento da espessura miocárdica de ventrículo direito (VD), hipertrofia miocárdica assimétrica de predomínio septal acometendo paredes anterior e lateral do ventrículo esquerdo (VE) com maior espessura no segmento inferoseptal medial (25 mm). Detectou-se presença de realce tardio mesocárdico nas inserções superior e inferior do VD na região do septo interventricular, além de realce tardio heterogêneo em segmentos antero-septal e ínfero-septal mediobasal. A massa de fibrose miocárdica foi estimada em 6,4g (2,2% da massa total do VE). Todos esses achados são típicos de cardiomiopatia hipertrófica (CMH) com predomínio septal e envolvimento do VD. Este caso chamou a atenção por se tratar de paciente com apresentação clínica de angina, hipertensão e critérios de HVE pelo ECG não confirmada pelo ECO em que a RMC fez o diagnóstico de CMH. Portanto, a avaliação multimodal com diversas técnicas diagnósticas muitas vezes se faz necessária para a confirmação diagnóstica da CMH.

Clinical case of 31-year-old male patient, white, married, born in Nepal living in São Paulo for 4 years, refered to the emergency room (ER) for atypical angina and high blood pressure sic. He remained under observation for 48 hours, and during this period the physical examination showed normal cardiac auscultation and blood pressure (BP) 140X85 mm Hg. The electrocardiogram (ECG) had criteria for left ventricular hypertrophy (LVH) and ventricular repolarization abnormality. He also had a slight increase of myocardial necrosis markers. As his symptoms improved, he was sent to the cardiac ambulatory. When the patient returned to the ambulatory he had no cardiac complaints, his BP was normal. It was then requested 24-hour ambulatory blood pressure monitoring (ABPM), echocardiography (ECHO), and coronary angiotomography. The ABPM presented normal blood pressure levels (awake and asleep respectively 112x65 and 106x51 mmHg), the ECHO did not show LVH, coronary angiotomography did not detect coronary obstructions and the calcium score was zero. A cardiac magnetic resonance (CMR) of the heart was performed which showed increased myocardial thickness of the right ventricle (RV), asymmetric myocardial hypertrophy of septal interventricular that also affecting anterior and lateral walls of the left ventricle (LV) with greater thickness in the medial inferoseptal segment (25 mm). It also presented signal of late mesocardial enhancement in the superior and inferior RV insertions of the interventricular septum and heterogeneous late enhancement in anteroseptal and inferoseptal mediobasal segments. The myocardial fibrosis mass was estimated in 6.4g (2.2% of the total LV mass). All these findings are typical of septal hypertrophic cardiomyopathy (HCM) with involvement of the RV. This case drew attention because it was a patient with a clinical presentation of angina, hypertension, and criteria for LVH by the ECG not confirmed by ECHO, but the CMR characterized as HCM. Therefore, multimodal evaluation diagnostic techniques in patient with electrocardiographic criteria of LVH without correlation with ECHO imagens were essential to the diagnosis of HCM.

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.

Desempenho do eletrocardiograma no diagnóstico da hipertrofia ventricular esquerda em hipertensos idosos e muito idosos / Performance of the electrocardiogram in the diagnosis of left ventricular hypertrophy in older and very older hypertensive patients

Resumo Fundamento: A hipertrofia ventricular esquerda (HVE) é um importante fator de risco cardiovascular, independente da hipertensão arterial. Apesar da evolução dos exames de imagem, o eletrocardiograma (ECG) ainda é o mais utilizado na avaliação inicial, porém, com baixa sensibilidade. Objetivo: Avaliar o desempenho dos principais critérios eletrocardiográficos para HVE em indivíduos hipertensos idosos e muito idosos. Métodos: Em coorte de hipertensos foram realizados ECGs e EcoDopplercardiogramas (ECO), e separados em três grupos etários: <60 anos, Grupo I; 60-79 anos Grupo II; e ≥80 anos, Grupo III. Os critérios eletrocardiográficos mais utilizados foram aplicados para o diagnóstico da HVE: Perúgia; Peguero-Lo Presti; Gubner-Ungerleider; Narita; (Rm+Sm) x duração; Cornell voltagem; Cornell voltagem duração; Sokolow-Lyon voltagem; R de aVL ≥11 mm; RaVL duração. Na avaliação do desempenho desses critérios, além da sensibilidade (Sen) e especificidade (Esp), foram analisadas as "Odds Ratios diagnóstico" (DOR). Consideramos p-valor <0,05 para as análises, com testes bi-caudais. Resultados: Em 2.458 pacientes, a HVE estava presente pelo ECO em 781 (31,7%). Nos Grupos I e II, os melhores desempenhos foram para os critérios de Narita, Perúgia, (Rm+Sm) x duração, sem diferenças estatísticas entre eles. No Grupo III (muito idosos) os critérios de Perúgia e (Rm+Sm) x duração tiveram os melhores desempenhos: Perúgia [44,7/89,3; (Sen/Esp)] e (Rm+Sm) duração [39,4%/91,3%; (Sen/Esp), p<0,05)], com os melhores resultados de DOR:6,8. Isto sugere que nessa população de muito idosos esses critérios têm maior poder discriminatório para separar pacientes com HVE. Conclusão: Nos hipertensos muito idosos os critérios eletrocardiográficos de Perúgia e (Rm+Sm) x duração apresentaram os melhores desempenhos diagnósticos para HVE.

Abstract Background: Left ventricular hypertrophy (LVH) is an important cardiovascular risk factor, regardless of arterial hypertension. Despite the evolution of imaging tests, the electrocardiogram (ECG) is still the most used in the initial evaluation, however, with low sensitivity. Objective: To evaluate the performance of the main electrocardiographic criteria for LVH in elderly and very elderly hypertensive individuals. Methods: In a cohort of hypertensive patients, ECGs and doppler echocardiographies (ECHO) were performed and separated into three age groups: <60 years, Group I; 60-79 years Group II; and ≥80 years, Group III. The most used electrocardiographic criteria were applied for the diagnosis of LVH: Perugia; Pegaro-Lo Presti; Gubner-Ungerleider; Narita; (Rm+Sm) x duration; Cornell voltage; Cornell voltage duration; Sokolow-Lyon voltage; R of aVL ≥11 mm; RaVL duration. In evaluating the performance of these criteria, in addition to sensitivity (Sen) and specificity (Esp), the "Diagnostic Odds Ratios" (DOR) were analyzed. We considered p-value <0.05 for the analyses, with two-tailed tests. Results: In 2,458 patients, LVH was present by ECHO in 781 (31.7%). In Groups I and II, the best performances were for the criteria of Narita, Perugia, (Rm+Sm) x duration, with no statistical differences between them. In Group III (very elderly) the Perugia criteria and (Rm+Sm) x duration had the best performances: Perugia [44,7/89.3; (Sen/Esp)] and (Rm+Sm) duration [39.4%/91.3%; (Sen/Esp), p<0.05)], with the best PAIN results:6.8. This suggests that in this very elderly population, these criteria have greater discriminatory power to separate patients with LVH. Conclusion: In very elderly hypertensive patients, the Perugia electrocardiographic criteria and (Rm+Sm) x duration showed the best diagnostic performance for LVH.

Blood pressure control of hypertensive patients followed in a high complexity clinic and associated variables / Controle da pressão arterial de hipertensos acompanhados em um ambulatório de alta complexidade e variáveis associadas

Abstract Introduction: Arterial hypertension is a disease that has a high impact on cardiovascular mortality and morbidity; however, it is still insufficiently controlled. Objectives: To assess hypertension control in patients seen at a specialized clinic and to identify associated variables. Method: Cross-sectional study involving the analysis of medical records from 782 patients treated in a highly complex outpatient clinic. Inclusion criteria: age ≥18 years, diagnosed with hypertension, in treatment ≥6 months. Patients with secondary hypertension (104) and incomplete data (64) were excluded. The main outcome was blood pressure control (systolic <140 and diastolic <90 mmHg). The independent variables studied were: sociodemographic and clinical characteristics (use of drugs, comorbidities and laboratory tests). Pearson's χ2 tests, Fisher's test, Student's t and Wilcoxon-Mann-Whitney tests were performed in the bivariate analysis and logistic regression in the multiple analyses, adopting p≤0.05. Results: The prevalence of hypertensive control was 51.1%. It was associated with a lack of control: body mass index (OR = 1.038; 95% CI = 1.008 - 1.071), history of stroke (OR = 0.453; 95% CI = 0.245 - 0.821), left ventricular hypertrophy (OR = 1.765; 95% CI = 1.052 - 3.011), and number of medications (OR = 1.082; 95% CI = 1.033 - 1.136). Conclusion: About half of the hypertensive patients had their blood pressure controlled; clinical variables and target organ damage were associated with the control.

Resumo Introdução: A hipertensão arterial é uma doença com alto impacto na mortalidade e morbidade cardiovascular, contudo ainda demonstra insuficientes taxas de controle. Objetivos: Avaliar o controle da hipertensão em pacientes atendidos em um ambulatório especializado e identificar variáveis associadas. Método: Estudo transversal com análise do prontuário de 782 pacientes atendidos em um ambulatório de alta complexidade. Critérios de inclusão: idade ≥ 18 anos e diagnóstico de hipertensão em tratamento ≥ 6 meses. Foram excluídos hipertensão secundária (104) e dados incompletos (64). O desfecho principal foi o controle da pressão arterial (Sistólica < 140 e diastólica < 90 mmHg). As variáveis independentes estudadas foram: características sociodemográficas e clínicas (uso de medicamentos, comorbidades e exames laboratoriais). Realizou-se testes χ2 de Pearson, teste Fisher, t de Student e Wilcoxon-Mann-Whitney na análise bivariada e Regressão Logística na análise múltipla, adotando p ≤ 0,05. Resultados: A prevalência de controle dos hipertensos foi 51,1%. Associou-se à falta de controle: índice de massa corporal (OR = 1,038; IC95% = 1,008 - 1,071), histórico de acidente vascular encefálico (OR = 0,453; IC95% = 0,245 - 0,821) e hipertrofia ventricular esquerda (OR = 1,765; IC95% = 1,052 - 3,011), e número de medicamentos (OR = 1,082; IC95% = 1,033 - 1,136). Conclusão: Cerca da metade dos hipertensos estava com pressão arterial controlada e variáveis clínicas e lesão em órgão alvo associaram-se ao controle.