In-Hospital Management and Long-term Clinical Outcomes and Adherence in Patients With Acute Decompensated Heart Failure: Primary Results of the First Brazilian Registry of Heart Failure (BREATHE).

BACKGROUND: Heart failure (HF), a common cause of hospitalization, is associated with poor short-term clinical outcomes. Little is known about the long-term prognoses of patients with HF in Latin America. METHODS: BREATHE was the first nationwide prospective observational study in Brazil that included patients hospitalized due to acute heart failure (HF). Patients were included during 2 time periods: February 2011-December 2012 and June 2016-July 2018 SUGGESTION FOR REPHRASING: In-hospital management, 12-month clinical outcomes and adherence to evidence-based therapies were evaluated. RESULTS: A total of 3013 patients were enrolled at 71 centers in Brazil. At hospital admission, 83.8% had clear signs of pulmonary congestion. The main cause of decompensation was poor adherence to HF medications (27.8%). Among patients with reduced ejection fraction, concomitant use of beta-blockers, renin-angiotensin-aldosterone inhibitors and spironolactone decreased from 44.5% at hospital discharge to 35.2% at 3 months. The cumulative incidence of mortality at 12 months was 27.7%, with 24.3% readmission at 90 days and 44.4% at 12 months. CONCLUSIONS: In this large national prospective registry of patients hospitalized with acute HF, rates of mortality and readmission were higher than those reported globally. Poor adherence to evidence-based therapies was common at hospital discharge and at 12 months of follow-up.

Heart failure and endothelial nitric oxide synthase G894T gene polymorphism frequency variations within ancestries.

The G894T polymorphism in endothelial nitric oxide synthase enzyme gene plays an important role in heart failure (HF) and its frequency varies among populations. We investigated this association in highly admixed samples in terms of ancestry. The cohort included 210 HF patients and 106 healthy individuals. Self-reported race and NYHA class were analyzed for HF patients. G894T polymorphism was analyzed by polymerase chain reaction (PCR) and by restriction fragment length polymorphism technique. Ancestry was estimated using a PCR reaction containing 46 autosomal ancestry informative markers and an analysis by capillary electrophoresis. The GG homozygous genotype had a higher frequency in HF patients (63.8%) than in healthy individuals (48.1%), showing an increased chance (odds ratio 1.90, 95% confidence interval 1.18-3.05). The ancestry profiles in patients and controls were similar, with a major European contribution (57.1% and 63.2%), followed by African (30.2% and 24.0%) and Native American (12.7% and 12.8%), without a significant difference between both samples (p = 0.28). The GG genotype is associated to HF prognosis, and this association remains present in highly admixed sample groups.

Peak oxygen uptake after the 80s as a survival predictor.

PURPOSE: Peak oxygen uptake (VO2peak) is a crucial health marker, extensively studied in adults for its prognostic value. However, its significance in the older persons, especially octogenarians, remains underexplored due to limited representation in research. This study aims to assess the predictive power of VO2peak for survival in individuals aged 80 and above. METHODS: We included individuals aged 80 or older who underwent cardiopulmonary exercise tests at a single center. Mortality rates were compared based on VO2peak relative to 80% of predicted values (%VO2peak). We employed three multivariate Cox regression models: Model 1 (unadjusted), Model 2 (adjusted for age) and Model 3 (adjusted for age and stroke). RESULTS: Among 188 participants (mean age 83.3 ± 3 years, 68.9% male), 22 (11.7%) passed away during a median follow-up of 494 days. Non-survivors tended to be older with lower VO2peak and %VO2peak. All models demonstrated associations between %VO2peak ≤ 80% and mortality: HR = 3.19 (95% CI: 1.30-7.86, p = 0.011) for M1; HR = 3.12 (95% CI: 1.26-7.74, p = 0.013) for M2 and HR = 2.80 (95% CI: 1.11-7.06, p = 0.028) for M3. CONCLUSION: In the context of an aging population, this study underscores the enduring significance of VO2peak as a survival predictor among the older person, including octogenarians. These findings carry profound implications for tailoring healthcare strategies to address the evolving demographic landscape.

Multifaceted Strategy Based on Automated Text Messaging After a Recent Heart Failure Admission: The MESSAGE-HF Randomized Clinical Trial.

Importance: Readmissions after an index heart failure (HF) hospitalization are a major contemporary health care problem. Objective: To evaluate the feasibility and efficacy of an intensive telemonitoring strategy in the vulnerable period after an HF hospitalization. Design, Setting, and Participants: This randomized clinical trial was conducted in 30 HF clinics in Brazil. Patients with left ventricular ejection fraction less than 40% and access to mobile phones were enrolled up to 30 days after an HF admission. Data were collected from July 2019 to July 2022. Intervention: Participants were randomly assigned to a telemonitoring strategy or standard care. The telemonitoring group received 4 daily short message service text messages to optimize self-care, active engagement, and early intervention. Red flags based on feedback messages triggered automatic diuretic adjustment and/or a telephone call from the health care team. Main Outcomes and Measures: The primary end point was change in N-terminal pro-brain natriuretic peptide (NT-proBNP) from baseline to 180 days. A hierarchical win-ratio analysis incorporating blindly adjudicated clinical events (cardiovascular deaths and HF hospitalization) and variation in NT-proBNP was also performed. Results: Of 699 included patients, 460 (65.8%) were male, and the mean (SD) age was 61.2 (14.5) years. A total of 352 patients were randomly assigned to the telemonitoring strategy and 347 to standard care. Satisfaction with the telemonitoring strategy was excellent (net promoting score at 180 days, 78.5). HF self-care increased significantly in the telemonitoring group compared with the standard care group (score difference at 30 days, -2.21; 95% CI, -3.67 to -0.74; P = .001; score difference at 180 days, -2.08; 95% CI, -3.59 to -0.57; P = .004). Variation of NT-proBNP was similar in the telemonitoring group compared with the standard care group (telemonitoring: baseline, 2593 pg/mL; 95% CI, 2314-2923; 180 days, 1313 pg/mL; 95% CI, 1117-1543; standard care: baseline, 2396 pg/mL; 95% CI, 2122-2721; 180 days, 1319 pg/mL; 95% CI, 1114-1564; ratio of change, 0.92; 95% CI, 0.77-1.11; P = .39). Hierarchical analysis of the composite outcome demonstrated a similar number of wins in both groups (telemonitoring, 49 883 of 122 144 comparisons [40.8%]; standard care, 48 034 of 122 144 comparisons [39.3%]; win ratio, 1.04; 95% CI, 0.86-1.26). Conclusions and Relevance: An intensive telemonitoring strategy applied in the vulnerable period after an HF admission was feasible, well-accepted, and increased scores of HF self-care but did not translate to reductions in NT-proBNP levels nor improvement in a composite hierarchical clinical outcome. Trial Registration: ClinicalTrials.gov Identifier: NCT04062461.

Is the obesity paradox in outpatients with heart failure reduced ejection fraction real?

Background: The obesity occurrence has achieved epidemic levels worldwide and several studies indicate a paradoxical similarity among obesity and the prognosis in heart failure (HF). The primary objective was to understand the association between body mass index (BMI) and heart failure with reduced ejection fraction (HFREF) of ischemic etiology in outpatients, using mortality as a parameter. The secondary objectives were to determine the differences in HF functional class, pharmacological therapy and evaluate the prognostic value of MAGGIC Score in this population. Methods: We analyzed 1,556 medical records from the HF outpatient clinic of a quaternary hospital and 242 were selected according to the criteria. Most were male, average age 62.6 (56-70), BMI 18.5-24.9 = 35.1%, 25-29.9 = 37.2%, 30-34.9 = 17.8%, 35-39.9 = 7%; BMI <18.5 and >40 groups were eliminated from the central analyzes because of scarce testing. Results: BMI 30-34.9 and BMI 18.5-24.9 had the best prognosis, BMI 25-29.9 had an average performance, and BMI -39.9 group provided the worst outcome (p = 0.123). In the subcategory analysis, BMI 30-34.9 group had a better prognosis compared to the BMI 35-39.9 group (p = 0.033). In the multivariate analysis The MAGGIC score was not able to foretell mortality in this population according to BMI. Conclusion: In not hospitalized patients with HFREF of ischemic etiology, obesity was not a protective factor.

Personalized care of patients with heart failure: are we ready for a REWOLUTION? Insights from two international surveys on healthcare professionals' needs and patients' perceptions.

AIMS: Guidelines for the management of heart failure (HF) are evolving, and increasing emphasis is placed on patient-centred care. As part of the REWOLUTION HF (REal WOrLd EdUcaTION in HF) programme, we conducted two international surveys aimed at assessing healthcare professionals' (HCPs) educational needs and patients' perspectives on the care of HF. METHODS AND RESULTS: Anonymous online questionnaires co-developed by HF experts and patients assessed HCPs' educational needs (520 respondents, mostly cardiologists, in 67 countries) and patients' perceptions on HF impact and management (98 respondents in 18 countries). Among HCPs, 62.7% prioritized rapid initiation of all guideline-mandated medications over up-titration of some medications, and 87.7% always or frequently discussed treatment goals with patients. There was good agreement between HCPs and patients on key treatment goals, except for a greater emphasis on reducing hospitalizations among HCPs. The most frequently cited barriers to the provision of guideline-recommended pharmacological therapy were treatment side effects/intolerance, complex treatment regimens, low blood pressure, cost/reimbursement issues, and low estimated glomerular filtration rate. Most patients (81.6%) reported no difficulties taking medications as prescribed, although 21.4% felt they were taking too many pills. Patients wanted more information about HF and its consequences, prognosis, and treatments (70.4%, 74.5% and 76.6%, respectively). Cardiologists were the preferred source of information about HF, followed by general practitioners and HF nurses. CONCLUSIONS: These surveys provide valuable insights into HCPs' needs about personalized care for patients with HF, as well as patients' perceptions, expectations and preferences. These findings will be helpful to develop patient-centred, needs-driven quality improvement programmes.

The impact of the COVID-19 pandemic on heart failure management: Global experience of the OPTIMIZE Heart Failure Care network.

During the COVID-19 pandemic, reductions in heart failure (HF) hospitalizations have been widely reported, and there is an urgent need to understand how HF care has been reorganized in countries with different infection levels, vaccination rates and healthcare services. The OPTIMIZE Heart Failure Care program has a global network of investigators in 42 countries, with first-hand experience of the impact of the pandemic on HF management in different care settings. The national coordinators were surveyed to assess: 1) the challenges of the COVID-19 pandemic for continuity of HF care, from both a hospital and patient perspective; 2) the organizational changes enacted to ensure continued HF care; and 3) lessons learned for the future of HF care. Contributions were obtained from 37 national coordinators in 29 countries. We summarize their input, highlighting the issues raised and using the example of three very different settings (Italy, Brazil, and Taiwan) to illustrate the similarities and differences across the OPTIMIZE program.

Left Atrial Septal Pouch and Acute Thromboembolic Ischemia of the Upper Limb.

BACKGROUND A left atrial septal pouch (LASP) was first described in 2010 as a new anatomical entity with potential for embolic events. The prevalences of left, right, and double septal pouches are 40.8%, 5.1%, and 3.7%, respectively. There is a concern about the risk of embolic events due to formation of thrombi in a LASP (especially stroke). CASE REPORT A 60-year-old man presented with sudden onset of right arm pain associated with sweating and neck pain radiating to his left upper extremity. On physical examination, his right arm was cyanotic and he had pain, paresthesia, and no radial pulse. The patient was diagnosed with acute arterial occlusion of his right upper extremity. An arterial embolectomy was performed with a Fogarty catheter at the level of the brachial artery, which resulted in immediate reperfusion. The patient had an embolic event and after efforts to identify the possible etiology, only an LASP was found. Therefore, we hypothesized that he experienced an embolic event in which a thrombus had formed at the site of the LASP. CONCLUSIONS The present case report is designed to raise awareness of the thrombogenic potential of LASP and the possibility of an embolic event to the upper limb of patients with it. LASP can be the source of a thrombus in a patient with a non-stroke embolic event.

Design of a multifaceted strategy based on automated text messaging in patients with recent heart failure admission.

AIMS: To evaluate a telemonitoring strategy based on automated text messaging and telephone support after heart failure (HF) hospitalization. METHODS AND RESULTS: The MESSAGE-HF study is a prospective multicentre, randomized, nationwide trial enrolling patients from 30 clinics in all regions of Brazil. HF patients with reduced left ventricular ejection fraction (<40%) and access to mobile phones are eligible after an acute decompensated HF hospitalization. Patients meeting eligibility criteria undergo an initial feasibility text messaging assessment and are randomized to usual care or telemonitoring intervention. All patients receive a HF booklet with basic information and recommendations about self-care. Patients in the intervention group receive four daily short text messages (educational and feedback) during the first 30 days of the protocol to optimize self-care; the feedback text messages from patients could trigger diuretic adjustments or a telephone call from the healthcare team. After 30 days, the frequency of text messages can be adjusted. Patients are followed up after 30, 90, and 180 days, with final status ascertained at 365 days by telephone. Our primary endpoint is the change in N-terminal pro-brain natriuretic peptide (NT-proBNP) levels after 180 days. Secondary endpoints include changes in NT-proBNP after 30 days; health-related quality of life, HF self-care, and knowledge scales after 30 and 180 days; and a composite outcome of HF hospitalization and cardiovascular death, adjudicated by a blinded and independent committee. CONCLUSIONS: The MESSAGE-HF trial is evaluating an educational and self-care promotion strategy involving a simple, intensive, and tailored telemonitoring system. If proven effective, it could be applied to a broader population worldwide.

Implications for Clinical Practice from a Multicenter Survey of Heart Failure Management Centers.

OBJECTIVES: This observational, cross-sectional study based aimed to test whether heart failure (HF)-disease management program (DMP) components are influencing care and clinical decision-making in Brazil. METHODS: The survey respondents were cardiologists recommended by experts in the field and invited to participate in the survey via printed form or email. The survey consisted of 29 questions addressing site demographics, public versus private infrastructure, HF baseline data of patients, clinical management of HF, performance indicators, and perceptions about HF treatment. RESULTS: Data were obtained from 98 centers (58% public and 42% private practice) distributed across Brazil. Public HF-DMPs compared to private HF-DMP were associated with a higher percentage of HF-DMP-dedicated services (79% vs 24%; OR: 12, 95% CI: 94-34), multidisciplinary HF (MHF)-DMP [84% vs 65%; OR: 3; 95% CI: 1-8), HF educational programs (49% vs 18%; OR: 4; 95% CI: 1-2), written instructions before hospital discharge (83% vs 76%; OR: 1; 95% CI: 0-5), rehabilitation (69% vs 39%; OR: 3; 95% CI: 1-9), monitoring (44% vs 29%; OR: 2; 95% CI: 1-5), guideline-directed medical therapy-HF use (94% vs 85%; OR: 3; 95% CI: 0-15), and less B-type natriuretic peptide (BNP) dosage (73% vs 88%; OR: 3; 95% CI: 1-9), and key performance indicators (37% vs 60%; OR: 3; 95% CI: 1-7). In comparison to non- MHF-DMP, MHF-DMP was associated with more educational initiatives (42% vs 6%; OR: 12; 95% CI: 1-97), written instructions (83% vs 68%; OR: 2: 95% CI: 1-7), rehabilitation (69% vs 17%; OR: 11; 95% CI: 3-44), monitoring (47% vs 6%; OR: 14; 95% CI: 2-115), GDMT-HF (92% vs 83%; OR: 3; 95% CI: 0-15). In addition, there were less use of BNP as a biomarker (70% vs 84%; OR: 2; 95% CI: 1-8) and key performance indicators (35% vs 51%; OR: 2; 95% CI: 91,6) in the non-MHF group. Physicians considered changing or introducing new medications mostly when patients were hospitalized or when observing worsening disease and/or symptoms. Adherence to drug treatment and non-drug treatment factors were the greatest medical problems associated with HF treatment. CONCLUSION: HF-DMPs are highly heterogeneous. New strategies for HF care should consider the present study highlights and clinical decision-making processes to improve HF patient care.

Experience in a Brazilian Center with Cryoablation for Electric Isolation of the Pulmonary Veins in Paroxysmal and Persistent Atrial Fibrillation - Preliminary Results in Brazil. / Experiência de um Centro Brasileiro com Crioablação para Isolamento Elétrico das Veias Pulmonares na Fibrilação Atrial Paroxística e Persistente ­ Resultados Preliminares no Brasil.

BACKGROUND: Electrical isolation of the pulmonary veins is recognized as the cornerstone of non-pharmacological treatment of Atrial Fibrillation (AF), and therefore, has been recommended as the first step in AF ablation according to all guidelines. Even though the cryoballoon technology is widely used in North America and Europe, this experience is still incipient in many developing countries such as Brazil. OBJECTIVE: To evaluate initial results regarding success and safety of the new technology in patients with persistent and paroxysmal AF. METHODS: One hundred and eight consecutive patients with symptomatic AF refractory to pharmacological treatment were submitted to cryoablation for isolation of the pulmonary veins. Patients were separated into two groups according to AF classification: persistent (AF for over one week); or paroxysmal (shorter episodes). Recurrence and procedural safety data were analyzed respectively as primary and secondary outcomes. The level of significance was 5%. RESULTS: One hundred and eight patients, with mean age 58±13 years, 84 males (77.8%), underwent cryoablation. Sixty-five patients had paroxysmal AF (60.2%) and 43 had persistent AF (39.2%). The mean time of the procedure was 96.5±29.3 minutes and the mean fluoroscopy time was 29.6±11.1 minutes. Five (4.6%) complications were observed, none fatal. Considering a blanking period of 3 months, 21 recurrences (19.4%) were observed in a one-year follow-up period. The recurrence-free survival rates of AF in the paroxysmal and persistent groups were 89.2% and 67.4%, respectively. CONCLUSION: Cryoablation for electrical isolation of the pulmonary veins is a safe and effective method for the treatment of AF. Our results are consistent with other studies suggesting that this technology can be used as an initial technique even in cases of persistent AF.

FUNDAMENTO: O isolamento elétrico das veias pulmonares é reconhecidamente base fundamental para o tratamento não farmacológico da fibrilação atrial (FA) e, portanto, tem sido recomendado como passo inicial na ablação de FA em todas as diretrizes. A técnica com balão de crioenergia, embora amplamente utilizada na América do Norte e Europa, ainda se encontra em fase inicial em muitos países em desenvolvimento, como o Brasil. OBJETIVO: Avaliar o sucesso e a segurança da técnica de crioablação em nosso serviço, em pacientes com FA paroxística e persistente. MÉTODOS: Cento e oito pacientes consecutivos com FA sintomática e refratária ao tratamento farmacológico foram submetidos à crioablação para isolamento das veias pulmonares. Os pacientes foram separados em dois grupos, de acordo com a classificação convencional da FA paroxística (duração de até sete dias) e persistente (FA por mais de sete dias). Dados de recorrência e segurança do procedimento foram analisados respectivamente como desfechos primário e secundário. O nível de significância adotado foi de 5%. RESULTADOS: Cento e oito pacientes, com idade média de 58±13 anos, 84 do sexo masculino (77,8%), foram submetidos ao procedimento de crioablação de FA. Sessenta e cinco pacientes apresentavam FA paroxística (60,2%) e 43, FA persistente (39,2%). O tempo médio do procedimento foi de 96,5±29,3 minutos e o tempo médio de fluoroscopia foi de 29,6±11,1 minutos. Foram observadas cinco (4,6%) complicações, nenhuma fatal. Considerando a evolução após os 3 meses iniciais, foram observadas 21 recorrências (19,4%) em período de um ano de seguimento. As taxas de sobrevivência livre de recorrência nos grupos paroxístico e persistente foram de 89,2% e 67,4%, respectivamente. CONCLUSÃO: A crioablação para isolamento elétrico das veias pulmonares é um método seguro e eficaz para tratamento da FA. Nossos resultados estão consoantes com demais estudos, que sugerem que a tecnologia pode ser utilizada como abordagem inicial, mesmo nos casos de FA persistente. (Arq Bras Cardiol. 2020; [online].ahead print, PP.0-0).

Coronavirus Disease 2019 and the Myocardium. / Doença de Coronavírus-19 e o Miocárdio.

Infection with the coronavirus known as COVID-19 has promoted growing interest on the part of cardiologists, emergency care specialists, intensive care specialists, and researchers, due to the study of myocardial involvement based on different clinical forms resulting from immunoinflammatory and neurohumoral demodulation.Myocardial involvement may be minimal and identifiable only by electrocardiographic changes, mainly increased cardiac troponins, or, on the other side of the spectrum, by forms of fulminant myocarditis and takotsubo syndrome.The description of probable acute myocarditis has been widely supported by the observation of increased troponin in association with dysfunction. Classical definition of myocarditis, supported by endomyocardial biopsy of inflammatory infiltrate, is rare; it has been observed in only one case report to date, and the virus has not been identified inside cardiomyocytes.Thus, the phenomenon that has been documented is acute myocardial injury, making it necessary to rule our obstructive coronary disease based on increased markers of myocardial necrosis, whether or not they are associated with ventricular dysfunction, likely associated with cytokine storms and other factors that may synergistically promote myocardial injury, such as sympathetic hyperactivation, hypoxemia, arterial hypotension, and microvascular thrombotic phenomena.Systemic inflammatory and myocardial phenomena following viral infection have been well documented, and they may progress to cardiac remodeling and myocardial dysfunction. Cardiac monitoring of these patients is, therefore, important in order to monitor the development of the phenotype of dilated myocardiopathy.This review presents the main etiological and physiopathological findings, a description of the taxonomy of these types of cardiac involvement, and their correlation with the main clinical forms of the myocardial component present in patients in the acute phase of COVID-19.

Strain Analysis of Left Ventricular Function in the Association of Hypertrophic Cardiomyopathy and Systemic Arterial Hypertension.

BACKGROUND: Hypertrophic cardiomyopathy (HCM) is the most common heart disease of genetic origin in the world population, with a prevalence of at least 1/500. The association with systemic arterial hypertension (SAH) is not uncommon, as it affects approximately 25% of the world population. Most studies aim at the differential diagnosis between these diseases, but little is known about the magnitude of this association. OBJECTIVE: To compare left ventricular global longitudinal strain (GLS) in HCM patients with and without associated SAH. METHODS: Retrospective cross-sectional study that included 45 patients with HCM and preserved ejection fraction, with diagnosis confirmed by magnetic resonance imaging, including 14 hypertensive patients. Transthoracic echocardiography was performed, with emphasis on left ventricular myocardial strain analysis using GLS. In this study, p < 0.05 was considered statistically significant. RESULTS: Left ventricular strain was significantly lower in hypertensive individuals compared to normotensive individuals (-10.29 ± 2.46 vs. -12.35% ± 3.55%, p = 0.0303), indicating greater impairment of ventricular function in that group. Mean age was also significantly higher in hypertensive patients (56.1 ± 13.9 vs. 40.2 ± 12.7 years, p = 0.0001). Diastolic dysfunction was better characterized in hypertensive patients (p = 0.0242). CONCLUSION: Myocardial strain was significantly lower in the group of patients with HCM and SAH, suggesting greater impairment of ventricular function. This finding may be related to a worse prognosis with early evolution to heart failure. Prospective studies are required to confirm this hypothesis.

Clinical outcomes and mortality in old and very old patients undergoing cardiac resynchronization therapy.

AIM: Cardiac resynchronization therapy (CRT) is a valid therapeutic option for patients with heart failure (HF). However, the elderly population was not well represented in the guidelines. The primary end point was to evaluate the impact of advanced age on clinical response and cardiovascular and total mortality of patients undergoing CRT. The secondary end point was to assess the rate of acute complications related to the procedure. METHODS AND RESULTS: A total of 249 consecutive patients with HF and optimized treatment, QRS ≥ 120 ms, ejection fraction (EF) ≤ 35% and functional class (FC) III/ IV (NYHA) underwent CRT and divided into 3 groups: Group I-< 65 years-88/ 249 (35%); Group II- 65 to 75 years (old)- 72/ 249 (29%); Group III-≥ 75 years (very old)- 89/ 249 (36%). The improvement in FC and increase in EF (>10%) and/ or decrease in the left ventricular end systolic diameter (LVESD) >15% were the criteria of responsiveness. The favorable clinical response (p = ns) and cardiovascular mortality (p = 0.737) was similar in the 3 groups. In the group of very old patients, a significant increase in total mortality was observed (p = 0.03). The rate of acute complications related to the procedure did not differ between the groups (p = ns). CONCLUSION: The response to CRT and cardiovascular mortality were not affected by the advanced age and should not be an exclusion factor of this therapy. The procedure has been shown to be safe in elderly patients due to low rate of acute complications.

Role of Myocardial Fibrosis in Hypertrophic Cardiomyopathy: A Systematic Review and Updated Meta-Analysis of Risk Markers for Sudden Death.

BACKGROUND: Hypertrophic cardiomyopathy (HCM) is associated with sudden death (SD). Myocardial fibrosis is reportedly correlated with SD. OBJECTIVE: We performed a systematic review with meta-analysis, updating the risk markers (RMs) in HCM emphasizing myocardial fibrosis. METHODS: We reviewed HCM studies that addressed severe arrhythmic outcomes and the certain RMs: SD family history, severe ventricular hypertrophy, unexplained syncope, non-sustained ventricular tachycardia (NSVT) on 24-hour Holter monitoring, abnormal blood pressure response to exercise (ABPRE), myocardial fibrosis and left ventricular outflow tract obstruction (LVOTO) in the MEDLINE, LILACS, and SciELO databases. We used relative risks (RRs) as an effect measure and random models for the analysis. The level of significance was set at p < 0.05. RESULTS: Twenty-one studies were selected (14,901 patients aged 45 ± 16 years; men, 62.8%). Myocardial fibrosis was the major RISK MARKER (RR, 3.43; 95% CI, 1.95-6.03). The other RMs, except for LVOTO, were also predictors: SD family history (RR, 1.75; 95% CI, 1.39-2.20), severe ventricular hypertrophy (RR, 1.86; 95% CI, 1.26-2.74), unexplained syncope (RR, 2.27; 95% CI, 1.69-3.07), NSVT (RR, 2.79; 95% CI, 2.29-3.41), and ABPRE (RR, 1.53; 95% CI, 1.12-2.08). CONCLUSIONS: We confirmed the association of myocardial fibrosis and other RMs with severe arrhythmic outcomes in HCM and emphasize the need for new prediction models in managing these patients.