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1.
Catheter Cardiovasc Interv ; 103(6): 1023-1034, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38639143

RESUMO

BACKGROUND: The clinical efficacy and safety of alcohol septal ablation (ASA) for obstructive hypertrophic cardiomyopathy (HCM) have been well-established; however, less is known about outcomes in patients undergoing preemptive ASA before transcatheter mitral valve replacement (TMVR). AIMS: The goal of this study is to characterize the procedural characteristics and examine the clinical outcomes of ASA in both HCM and pre-TMVR. METHODS: This retrospective study compared procedural characteristics and outcomes in patient who underwent ASA for HCM and TMVR. RESULTS: In total, 137 patients were included, 86 in the HCM group and 51 in the TMVR group. The intraventricular septal thickness (mean 1.8 vs. 1.2 cm; p < 0.0001) and the pre-ASA LVOT gradient (73.6 vs. 33.8 mmHg; p ≤ 0.001) were higher in the HCM group vs the TMVR group. The mean volume of ethanol injected was higher (mean 2.4 vs. 1.7 cc; p < 0.0001). The average neo-left ventricular outflow tract area increased significantly after ASA in the patients undergoing TMVR (99.2 ± 83.37 mm2 vs. 196.5 ± 114.55 mm2; p = <0.0001). The HCM group had a greater reduction in the LVOT gradient after ASA vs the TMVR group (49.3 vs. 18 mmHg; p = 0.0040). The primary composite endpoint was higher in the TMVR group versus the HCM group (50.9% vs. 25.6%; p = 0.0404) and had a higher incidence of new permanent pacemaker (PPM) (25.5% vs. 18.6%; p = 0.3402). The TMVR group had a higher rate of all-cause mortality (9.8% vs. 1.2%; p = 0.0268). CONCLUSIONS: Preemptive ASA before TMVR was performed in patients with higher degree of clinical comorbidities, and correspondingly is associated with worse short-term clinical outcomes in comparison to ASA for HCM patients. ASA before TMVR enabled percutaneous mitral interventions in a small but significant minority of patients that would have otherwise been excluded. The degree of LVOT and neoLVOT area increase is significant and predictable.


Assuntos
Técnicas de Ablação , Cateterismo Cardíaco , Cardiomiopatia Hipertrófica , Etanol , Implante de Prótese de Valva Cardíaca , Valva Mitral , Humanos , Estudos Retrospectivos , Masculino , Etanol/administração & dosagem , Etanol/efeitos adversos , Cardiomiopatia Hipertrófica/diagnóstico por imagem , Cardiomiopatia Hipertrófica/mortalidade , Cardiomiopatia Hipertrófica/terapia , Cardiomiopatia Hipertrófica/cirurgia , Cardiomiopatia Hipertrófica/fisiopatologia , Feminino , Resultado do Tratamento , Técnicas de Ablação/efeitos adversos , Técnicas de Ablação/mortalidade , Idoso , Cateterismo Cardíaco/efeitos adversos , Cateterismo Cardíaco/mortalidade , Cateterismo Cardíaco/instrumentação , Pessoa de Meia-Idade , Fatores de Risco , Implante de Prótese de Valva Cardíaca/efeitos adversos , Implante de Prótese de Valva Cardíaca/instrumentação , Implante de Prótese de Valva Cardíaca/mortalidade , Fatores de Tempo , Valva Mitral/diagnóstico por imagem , Valva Mitral/fisiopatologia , Valva Mitral/cirurgia , Recuperação de Função Fisiológica , Idoso de 80 Anos ou mais , Septos Cardíacos/diagnóstico por imagem , Septos Cardíacos/cirurgia , Insuficiência da Valva Mitral/diagnóstico por imagem , Insuficiência da Valva Mitral/fisiopatologia , Insuficiência da Valva Mitral/cirurgia , Insuficiência da Valva Mitral/mortalidade
3.
Heart Fail Rev ; 29(3): 663-674, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38308002

RESUMO

Over the last years, there has been a growing interest in the clinical manifestations and outcomes of cardiomyopathies in women. Peripartum cardiomyopathy is the only women-specific cardiomyopathy. In cardiomyopathies with X-linked transmission, women are not simply healthy carriers of the disorder, but can show a wide spectrum of clinical manifestations ranging from mild to severe manifestations because of heterogeneous patterns of X-chromosome inactivation. In mitochondrial disorders with a matrilinear transmission, cardiomyopathy is part of a systemic disorder affecting both men and women. Even some inherited cardiomyopathies with autosomal transmission display phenotypic and prognostic differences between men and women. Notably, female hormones seem to exert a protective role in hypertrophic cardiomyopathy (HCM) and variant transthyretin amyloidosis until the menopausal period. Women with cardiomyopathies holding high-risk features should be referred to a third-level center and evaluated on an individual basis. Cardiomyopathies can have a detrimental impact on pregnancy and childbirth because of the associated hemodynamic derangements. Genetic counselling and a tailored cardiological evaluation are essential to evaluate the likelihood of transmitting the disease to the children and the possibility of a prenatal or early post-natal diagnosis, as well as to estimate the risk associated with pregnancy and delivery, and the optimal management strategies.


Assuntos
Cardiomiopatias , Humanos , Feminino , Cardiomiopatias/terapia , Cardiomiopatias/diagnóstico , Cardiomiopatias/fisiopatologia , Cardiomiopatias/genética , Gravidez , Complicações Cardiovasculares na Gravidez/terapia , Complicações Cardiovasculares na Gravidez/fisiopatologia , Complicações Cardiovasculares na Gravidez/diagnóstico , Complicações Cardiovasculares na Gravidez/genética , Cardiomiopatia Hipertrófica/terapia , Cardiomiopatia Hipertrófica/genética , Cardiomiopatia Hipertrófica/diagnóstico , Cardiomiopatia Hipertrófica/fisiopatologia , Aconselhamento Genético/métodos , Gerenciamento Clínico
5.
Am J Cardiol ; 212S: S64-S76, 2024 Feb 01.
Artigo em Inglês | MEDLINE | ID: mdl-38368038

RESUMO

Hypertrophic cardiomyopathy (HCM) is a relatively common and, often, inherited cardiac disease, once regarded as largely untreatable with ominous prognosis and, perhaps, most visibly as a common cause of sudden cardiac death (SCD) in the young. However, HCM is now more accurately considered a treatable disease with management options that significantly alter its clinical course. This is particularly true for SCD because the penetration of implantable cardioverter-defibrillators into HCM practice enables primary prevention device therapy that reliably terminates potentially lethal ventricular tachyarrhythmias (3% to 4%/year). This therapeutic advance is largely responsible for >10-fold decrease in the overall disease-related mortality to 0.5%/year, independent of patient age. A guideline-based clinical risk stratification algorithm has evolved, which included variables identifiable with cardiac magnetic resonance: ≥1 risk markers judged major within the clinical profile of an individual patient, associated with a measure of physician judgment and shared decision-making, can be sufficient to consider the recommendation of a prophylactic defibrillator implant. Implantable cardioverter-defibrillator decisions using the American College of Cardiology and the American Heart Association traditional major risk marker strategy are associated with a 95% sensitivity for identifying those patients who subsequently experience appropriate therapy, albeit often 5 to 10+ years after implant but without heart failure deterioration or death after a device intervention. A mathematical SCD risk score proposed by European Society of Cardiology is associated with a relatively low sensitivity (33%) for predicting and preventing SCD events but with potential for less device overtreatment.


Assuntos
Cardiomiopatia Hipertrófica , Desfibriladores Implantáveis , Humanos , Fatores de Risco , Desfibriladores Implantáveis/efeitos adversos , Morte Súbita Cardíaca/etiologia , Morte Súbita Cardíaca/prevenção & controle , Prognóstico , Cardiomiopatia Hipertrófica/complicações , Cardiomiopatia Hipertrófica/terapia , Medição de Risco
6.
Am J Cardiol ; 212S: S77-S82, 2024 Feb 01.
Artigo em Inglês | MEDLINE | ID: mdl-38368039

RESUMO

Hypertrophic cardiomyopathy (HCM) is a relatively common often inherited heart disease encumbered throughout much of its almost 60-year history by the expectation of an unfavorable outcome with shortened longevity. However, it is notable that in 2023, most patients affected with HCM can now achieve normal or extended life expectancy without major disability because of a comprehensive constellation of management strategies that have evolved largely over the last 20 years. Distinct adverse disease pathways dictate high-benefit low-risk personalized treatments, without reliance on genomics and sarcomere mutations, including: primary prevention implantable defibrillators for sudden cardiac death prevention, surgical myectomy and percutaneous alcohol septal ablation to reverse heart failure symptoms, anticoagulation to prevent embolic stroke associated with concomitant atrial fibrillation, external defibrillation and hypothermia for out-of-hospital cardiac arrest, and heart transplant in a small patient subgroup with end-stage disease. Large cohort studies using these contemporary management strategies achieved remarkably low HCM-related mortality (0.5%/year) across all age groups, which is lower than in the other cardiac or noncardiac risks of living, and largely confined to nonobstructive patients with progressive heart failure, including those awaiting heart transplant.


Assuntos
Fibrilação Atrial , Cardiomiopatia Hipertrófica , Insuficiência Cardíaca , Transplante de Coração , Doenças Vasculares , Humanos , Cardiomiopatia Hipertrófica/complicações , Cardiomiopatia Hipertrófica/terapia , Cardiomiopatia Hipertrófica/diagnóstico , Estudos de Coortes , Insuficiência Cardíaca/complicações , Transplante de Coração/efeitos adversos , Fibrilação Atrial/complicações , Morte Súbita Cardíaca/epidemiologia , Morte Súbita Cardíaca/etiologia , Morte Súbita Cardíaca/prevenção & controle , Doenças Vasculares/complicações
7.
Am J Cardiol ; 212S: S14-S32, 2024 Feb 01.
Artigo em Inglês | MEDLINE | ID: mdl-38368033

RESUMO

Hypertrophic cardiomyopathy (HCM) is increasingly recognized and may benefit from the recent approval of new, targeted medical therapy. Successful management of HCM is dependent on early and accurate diagnosis. The lack of a definitive diagnostic test, the wide variation in phenotype and the commonness of phenocopy conditions, and the presence of normal or hyperdynamic left ventricular function in most patients makes HCM a condition that is highly dependent on imaging for all aspects of management including, diagnosis, classification, predicting risk of complications, detecting complications, identifying risk for ventricular arrhythmias, evaluating choice of therapy and monitoring therapy, intraprocedural guidance, and screening family members. Although echocardiographic imaging remains the mainstay in the diagnosis and subsequent management of HCM, this disease clearly requires multimethod imaging for various aspects of optimal patient care. Advances in echocardiography hardware and techniques, development and refinement of imaging with computed tomography, magnetic resonance, and nuclear scanning, and the emergence of very focused assessments such as diastology and fibrosis imaging have all advanced the diagnosis and management of HCM. In this review, we discuss the relative utility and evidence support for these imaging approaches to contribute to improve patient outcomes.


Assuntos
Cardiomiopatia Hipertrófica , Humanos , Cardiomiopatia Hipertrófica/diagnóstico por imagem , Cardiomiopatia Hipertrófica/terapia , Imageamento por Ressonância Magnética/métodos , Ecocardiografia , Arritmias Cardíacas/complicações , Função Ventricular Esquerda
8.
Circ Cardiovasc Qual Outcomes ; 17(2): e010243, 2024 02.
Artigo em Inglês | MEDLINE | ID: mdl-38240157

RESUMO

BACKGROUND: Serum troponins and CK-MB (creatine kinase-MB) are readily detectable and reliable cardiac-specific biomarkers of subclinical myocardial injury. This study explores the roles of cTnI (cardiac troponin I) and CK-MB in hypertrophic cardiomyopathy (HCM). METHODS: This study included 1045 patients with HCM who had baseline cTnI and CK-MB measurements at Fuwai Hospital between 1999 and 2019. Patients were excluded if they had undergone percutaneous coronary intervention or coronary artery bypass grafting, or had renal failure. Five end points were studied: all-cause death, cardiovascular death, noncardiovascular death, sudden cardiac death, and other cardiovascular death. Cox regression was used to assess the associations of cTnI and CK-MB levels with outcomes. RESULTS: Nine hundred seventy patients with available follow-up data were finally analyzed (mean age, 49.3 years; 36.4% female). During the median 4.3-year follow-up period, 87 patients reached the end points. Higher cTnI (per 0.05 ng/mL increase) and CK-MB (per 1 IU/L increase) levels were associated with increased risks of all-cause death (cTnI: adjusted hazard ratio [HR], 1.038, P<0.001; CK-MB: adjusted HR, 1.021, P=0.004), cardiovascular death (cTnI: adjusted HR, 1.040, P<0.001; CK-MB: adjusted HR, 1.025, P=0.006), and sudden cardiac death (cTnI: adjusted HR, 1.045, P<0.001; CK-MB: adjusted HR, 1.032, P=0.001). Patients with elevated levels of both cTnI and CK-MB had worse prognoses than patients with an elevated level of either biomarker alone and patients who did not have an elevated level of either biomarker. Addition of the binary indicator elevation of both cTnI and CK-MB significantly improved the discrimination and reclassification abilities of the standard HCM Risk- sudden cardiac death model (C statistics: P=0.002; net reclassification improvement, 0.652; integrated discrimination improvement, 0.064). CONCLUSIONS: Comprehensive evaluations of biomarkers of myocardial injury, cTnI and CK-MB, have considerable value for predicting adverse outcomes among patients with HCM. Routine cTnI and CK-MB assessments may help to guide implantable cardioverter defibrillator implantation for primary prevention in HCM.


Assuntos
Cardiomiopatia Hipertrófica , Humanos , Feminino , Pessoa de Meia-Idade , Masculino , Biomarcadores , Creatina Quinase Forma MB , Prognóstico , Cardiomiopatia Hipertrófica/complicações , Cardiomiopatia Hipertrófica/diagnóstico , Cardiomiopatia Hipertrófica/terapia , Morte Súbita Cardíaca/etiologia , Morte Súbita Cardíaca/prevenção & controle
9.
J Cardiovasc Electrophysiol ; 35(4): 654-663, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38288888

RESUMO

INTRODUCTION: In hypertrophic cardiomyopathy (HCM), atrial fibrillation (AF) has historically been regarded to have a deleterious impact on clinical course, strongly associated with progressive heart failure (HF) symptoms. However, there is a paucity of information regarding the impact of AF on HCM employing validated quality of life (QoL) surveys. Therefore, we evaluated the impact of AF on QoL utilizing patient reported outcome measures (PROMs). METHODS: 218 consecutive HCM patients with or without AF at the Lahey HCM center in 2022 completed PROMs at their most recent visit evaluating HF (Kansas City Cardiomyopathy Questionnaire [KCCQ]) and AF symptoms (AF Effect on QoL [AFEQT]). RESULTS: Among the 218 patients, 50 (23%) had a history of AF and comprise the primary study cohort. AF was diagnosed at 55 ± 10 years of age, median of 5.5 years before PROM, with 66% of patients treated with a rhythm control strategy with antiarrhythmic drug and/or AF ablation. AFEQT indicated that 52% of patients experienced no or minimal AF-related disability, mild to moderate in 22%, and severe in 26%. There was no substantial difference in HCM phenotype in patients with no or minimal AF disability compared to those with severe disability. HF symptoms for most HCM patients with prior AF history was consistent with no or minimal (59%) or only mild (27%) disability as measured by KCCQ overall summary scores. In addition, with multivariate analysis, AF history was associated with less HF symptoms and improved QoL (OR 0.4, p = 0.02). CONCLUSION: In contrast to prior perceptions, HCM patients with prior AF history were less likely to incur HF symptoms impairing QoL compared to HCM patients without AF. After treatment, prior history of AF did not substantially impact current QoL. These data provide a realistic appraisal for the impact that AF has on HCM patients and also offers a measure of reassurance for this patient subgroup.


Assuntos
Fibrilação Atrial , Cardiomiopatia Hipertrófica , Doenças Vasculares , Humanos , Qualidade de Vida , Antiarrítmicos/uso terapêutico , Cardiomiopatia Hipertrófica/complicações , Cardiomiopatia Hipertrófica/diagnóstico , Cardiomiopatia Hipertrófica/terapia , Doenças Vasculares/complicações
12.
Mayo Clin Proc ; 99(2): 218-228, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-38180395

RESUMO

OBJECTIVE: To describe the potential clinical cardiotoxicity of oncological treatments in a cohort of consecutive patients with hypertrophic cardiomyopathy (HCM), systematically followed-up at two national referral centers for HCM. Cardiotoxicity relates to the direct effects of cancer-related treatment on heart function, commonly presenting as left ventricular contractile dysfunction. However, limited data are available regarding cardiotoxic effects on HCM as most studies have not specifically analyzed the effects of oncological treatment in HCM populations. This gap in knowledge may lead to unjustified restriction of HCM patients from receiving curative cancer treatments. METHODS: We retrospectively analyzed clinical and instrumental data of all consecutive HCM patients who underwent oncological treatment between January 2000 and December 2020 collected in a centralized database. RESULTS: Of 3256 HCM patients, 121 (3.7%) had cancer; 110 (90.9%) underwent oncological surgery, 45 (37.2%) received chemotherapy, and 22 (18.2%) received chest radiation therapy (cRT). After a median follow-up of 5.2 years (Q1-Q3: 2-13 years) from oncological diagnosis, 32 patients died. The cumulative survival at 5 years was 79.9%. The cause of death was mainly attributed to the oncological condition, whereas four patients died of sudden cardiac death without receiving previous chemotherapy or cRT. No patient interrupted or reduced the dose of oncological treatment due to cardiac dysfunction. No sustained ventricular tachyarrhythmia was induced by chemotherapy or radiation therapy. CONCLUSION: Cancer treatment was well tolerated in HCM patients. In our consecutive series, none died of cardiovascular complications induced by chemotherapy or cRT and they did not require interruption or substantial treatment tapering due to cardiovascular toxic effects. Although a multidisciplinary evaluation is necessary and regimens must be tailored individually, the diagnosis of HCM per se should not be considered a contraindication to receive optimal curative cancer treatment.


Assuntos
Cardiomiopatia Hipertrófica , Neoplasias , Disfunção Ventricular Esquerda , Humanos , Estudos Retrospectivos , Cardiotoxicidade , Cardiomiopatia Hipertrófica/complicações , Cardiomiopatia Hipertrófica/terapia , Cardiomiopatia Hipertrófica/diagnóstico , Morte Súbita Cardíaca , Neoplasias/complicações , Fatores de Risco
13.
Eur J Prev Cardiol ; 31(1): 3-10, 2024 Jan 05.
Artigo em Inglês | MEDLINE | ID: mdl-37531614

RESUMO

AIMS: Whether pregnancy is a modifier of the long-term course and outcome of women with hypertrophic cardiomyopathy (HCM) is unknown. We assessed the association of pregnancy with long-term outcomes in HCM women. METHODS AND RESULTS: Retrospective evaluation of women with HCM from 1970 to 2021. Only women with pregnancy-related information (pregnancy present or absent) and a follow-up period lasting ≥1 year were included. The peri-partum period was defined as -1 to 6 months after delivery. The primary endpoint was a composite for major adverse cardiovascular events [MACE: cardiovascular death, sudden cardiac death, appropriate defibrillator shock and heart failure (HF) progression]. Overall, 379 (58%) women were included. There were 432 pregnancies in 242 (63%) patients. In 29 (7.6%) cases, pregnancies (n = 39) occurred after HCM diagnosis. Among these, three carrying likely pathogenic sarcomeric variants suffered MACEs in the peri-partum period. At 10 ± 9 years of follow-up, age at diagnosis [hazard ratio (HR) 1.034, 95% confidence interval (CI) 1.018-1.050, P < 0.001] and New York Heart Association (NYHA) class (II vs. I: HR 1.944, 95% CI 0.896-4.218; III vs. I: HR 5.291, 95% CI 2.392-11.705, P < 0.001) were associated with MACE. Conversely, pregnancy was associated with reduced risk (HR 0.605; 95% CI 0.380-0.963, P = 0.034). Among women with pregnancy, multiple occurrences did not modify risk. CONCLUSIONS: Pregnancy is not a modifier of long-term outcome in women with HCM and mostly occurs before a cardiac diagnosis. Most patients tolerate pregnancy well and do not show a survival disadvantage compared to women without. Pregnancy should not be discouraged, except in the presence of severe HF symptoms or high-risk features.


Hypertrophic cardiomyopathy (HCM) is the most common genetic disorder of the myocardium and is characterized by important gender-related differences: women are typically 5 years older than men at diagnosis, over half are diagnosed >50 years of age and consistently show greater propensity than men for heart failure (HF)-related complications and adverse outcome. Whether pregnancy is a modifier of the long-term course and outcome of women with HCM is unknown. In this study, pregnancy was not a modifier of long-term outcome in women with HCM. In particular: At 10 ± 7 years, most patients tolerated pregnancy well and did not show a survival disadvantage compared to women without pregnancies. Only baseline heart failure symptoms and age were associated with adverse outcome.Pregnancy should not be discouraged, except in the presence of severe HF symptoms or high-risk features. Nevertheless, cardio-obstetric counselling and close supervision are key in all instances, particularly in the peri-partum period.


Assuntos
Cardiomiopatia Hipertrófica , Gravidez , Humanos , Feminino , Masculino , Estudos Retrospectivos , Fatores de Risco , Cardiomiopatia Hipertrófica/diagnóstico , Cardiomiopatia Hipertrófica/epidemiologia , Cardiomiopatia Hipertrófica/terapia , Morte Súbita Cardíaca/epidemiologia , Morte Súbita Cardíaca/etiologia , Modelos de Riscos Proporcionais
14.
Herz ; 49(1): 22-32, 2024 Feb.
Artigo em Alemão | MEDLINE | ID: mdl-38051386

RESUMO

The group of cardiomyopathies has received increasing attention over the last few years after some of the causes were identified and they could be characterized more exactly using modern imaging methods. New definitions and classification schemes were regularly provided by national and international cardiac societies. The new guidelines of the European Society of Cardiology (ESC) from 2023 on the management of cardiomyopathies are the first guidelines that comprehensively address all cardiomyopathies in one document. As these are new guidelines most of the recommendations are also new. An exception is the section on hypertrophic cardiomyopathy (HCM), which provides a targeted update of the 2014 ESC guidelines on the diagnosis and treatment of HCM. The main aim of the guidelines is to provide clear guidance for the diagnosis of cardiomyopathies, to highlight general assessment and management problems and to point out the relevant scientific evidence for the recommendations to the readership. Due to the magnitude detailed descriptions and recommendations cannot be provided for each individual cardiomyopathy phenotype; however, reference is made to the relevant literature.


Assuntos
Cardiologia , Cardiomiopatias , Cardiomiopatia Hipertrófica , Sistema Cardiovascular , Humanos , Cardiomiopatias/diagnóstico , Cardiomiopatias/terapia , Cardiomiopatia Hipertrófica/diagnóstico , Cardiomiopatia Hipertrófica/terapia , Coração
15.
Heart Fail Rev ; 29(2): 395-404, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-37865929

RESUMO

Hypertrophic cardiomyopathy (HCM) is an autosomal dominant disorder characterized by left ventricular hypertrophy. Sudden cardiac death (SCD) is a rare but the most catastrophic complication in patients with HCM. Implantable cardioverter-defibrillators (ICDs) are widely recognized as effective preventive measures for SCD. Individualized risk stratification and early intervention in HCM can significantly improve patient prognosis. In this study, we review the latest findings regarding pathogenesis, risk stratification, and prevention of SCD in HCM patients, highlighting the clinic practice of cardiovascular magnetic resonance imaging for SCD management.


Assuntos
Cardiomiopatia Hipertrófica , Desfibriladores Implantáveis , Humanos , Fatores de Risco , Cardiomiopatia Hipertrófica/complicações , Cardiomiopatia Hipertrófica/terapia , Morte Súbita Cardíaca/etiologia , Morte Súbita Cardíaca/prevenção & controle , Coração , Desfibriladores Implantáveis/efeitos adversos , Medição de Risco
16.
Herzschrittmacherther Elektrophysiol ; 34(4): 311-323, 2023 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-37973628

RESUMO

Electrocardiographic findings and arrhythmias are common in cardiomyopathies. Both may be an early indication of a specific diagnosis or may occur due to myocardial fibrosis and/or reduced contractility. Brady- and tachyarrhythmias significantly contribute to increased morbidity and mortality in patients with cardiomyopathies. Antiarrhythmic therapy including risk stratification is often challenging and plays a major role for these patients. Thus, an "electrophysiological" perspective on guidelines on cardiomyopathies may be warranted. As the European Society of Cardiology (ESC) has recently published a new guideline for the management of cardiomyopathies, this overview aims to present key messages of these guidelines. Innovations include a new phenotype-based classification system with emphasis on a multimodal imaging approach for diagnosis and risk stratification. The guideline includes detailed chapters on dilated and hypertrophic cardiomyopathy and their phenocopies, arrhythmogenic right ventricular cardiomyopathy, and restrictive cardiomyopathy as well as syndromic and metabolic cardiomyopathies. Patient pathways guide clinicians from the initial presentation to diagnosis. The role of cardiovascular magnetic resonance imaging and genetic testing during diagnostic work-up is stressed. Concepts of rhythm and rate control for atrial fibrillation have led to new recommendations, and the role of defibrillator therapy in primary prevention is discussed in detail. Whilst providing general guidelines for management, the primary objective of the guideline is to ascertain the disease etiology and disease-specific, individualized management.


Assuntos
Cardiologia , Cardiomiopatias , Cardiomiopatia Hipertrófica , Humanos , Morte Súbita Cardíaca/prevenção & controle , Morte Súbita Cardíaca/etiologia , Cardiomiopatias/diagnóstico , Cardiomiopatias/terapia , Coração , Cardiomiopatia Hipertrófica/diagnóstico , Cardiomiopatia Hipertrófica/terapia , Arritmias Cardíacas
17.
Circulation ; 148(22): 1797-1811, 2023 11 28.
Artigo em Inglês | MEDLINE | ID: mdl-38011245

RESUMO

Atrial fibrillation (AF) is the most common sustained arrhythmia in hypertrophic cardiomyopathy (HCM) with clinical and subclinical episodes occurring in nearly one-half of patients. AF in HCM historically has been characterized as a decisive disease complication associated with substantial risk for thromboembolic stroke and increased morbidity and mortality. However, there have been many advances in treatment strategy resulting in improved outcomes for this patient group. For example, stroke risk in HCM has been greatly reduced by using systemic oral anticoagulation initiated after the first clinical (symptomatic) AF episode, usually with preference given to direct anticoagulants over warfarin. In contrast, stroke risk scoring systems (such as CHA2DS2-VASc score) are not informative in HCM given the substantial potential for stroke events in patients with low scores, and therefore should not be used for anticoagulation decisions in this disease. A novel risk score specifically designed for HCM (HCM-AF score) can reliably identify most patients with HCM at risk for future AF. Although a strategy focused on controlling ventricular rate is effective in asymptomatic (or minimally symptomatic) patients with AF, restoring and maintaining sinus rhythm is required for most patients with marked AF symptom burden and impaired quality of life. Several antiarrhythmic drugs such as sotalol, disopyramide, and amiodarone, can be effective in suppressing AF episodes; albeit safe, long-term efficacy is supported by only limited data. Catheter AF ablation has emerged as an important treatment option for some patients, although freedom from AF after a single ablation is relatively low (35% at 3 years), multiple ablations and the concomitant use of antiarrhythmic drugs can control AF with more than two-thirds of patients maintaining sinus rhythm at 5 years. Surgical AF ablation with biatrial Cox-Maze IV performed as an adjunctive procedure during myectomy can reduce symptomatic AF episodes (70% of patients free from AF at 5 years). For the vast majority of patients who have HCM with AF, the implementation of contemporary therapies has allowed for improved quality of life and low HCM-related mortality.


Assuntos
Fibrilação Atrial , Cardiomiopatia Hipertrófica , Ablação por Cateter , Acidente Vascular Cerebral , Humanos , Fibrilação Atrial/complicações , Fibrilação Atrial/diagnóstico , Fibrilação Atrial/epidemiologia , Antiarrítmicos/uso terapêutico , Qualidade de Vida , Fatores de Risco , Anticoagulantes/uso terapêutico , Acidente Vascular Cerebral/etiologia , Cardiomiopatia Hipertrófica/complicações , Cardiomiopatia Hipertrófica/diagnóstico , Cardiomiopatia Hipertrófica/terapia , Ablação por Cateter/efeitos adversos , Resultado do Tratamento
18.
Rev Med Liege ; 78(11): 619-625, 2023 Nov.
Artigo em Francês | MEDLINE | ID: mdl-37955291

RESUMO

Hypertrophic cardiomyopathy is a disease characterized by left ventricular hypertrophy (with or without right ventricular hypertrophy) not explained by loading conditions, the origin of which may be genetic and whose phenotypic expression is highly variable. The novelties in terms of diagnosis, clinical development, and management have been the subject of an update of the recommendations of the European Society of Cardiology (ESC).


La cardiomyopathie hypertrophique est une maladie caractérisée par une hypertrophie ventriculaire gauche (avec ou sans hypertrophie ventriculaire droite) non expliquée par les conditions de charge, dont l'origine peut être génétique et dont l'expression phénotypique est très variable. Les nouveautés en termes diagnostique, de mise au point, et de prise en charge ont fait l'objet d'une mise à jour des recommandations de la Société Européenne de Cardiologie (ESC).


Assuntos
Cardiologia , Cardiomiopatia Hipertrófica , Humanos , Cardiomiopatia Hipertrófica/diagnóstico , Cardiomiopatia Hipertrófica/genética , Cardiomiopatia Hipertrófica/terapia , Hipertrofia Ventricular Esquerda/diagnóstico , Fenótipo
19.
Europace ; 25(11)2023 11 02.
Artigo em Inglês | MEDLINE | ID: mdl-37995093

RESUMO

AIMS: The validated HCM Risk-Kids model provides accurate individualized estimates of sudden cardiac death risk in children with hypertrophic cardiomyopathy (HCM). A second validated model, PRIMaCY, also provides individualized estimates of risk, but its performance and clinical impact has not been independently investigated. The aim of this study was to investigate the clinical impact of using the PRIMaCY sudden cardiac death (SCD) risk model in childhood HCM. METHODS AND RESULTS: The estimated 5-year SCD risk was calculated for children meeting diagnostic criteria for HCM in a large single-centre cohort using PRIMaCY (clinical and genetic) and HCM Risk-Kids model, and model performance was assessed. Three hundred one patients [median age 10 (interquartile range 4-14)] were followed up for an average of 4.9 (±3.8) years, during which 30 (10.0%) reached the SCD or equivalent event endpoint. Harrell's C-statistic for the clinical and genetic models was 0.66 [95% confidence interval (CI) 0.52-0.8] and 0.66 (95% CI 0.54-0.80) with a calibration slope of 0.19 (95% CI 0.04-0.54) and 0.26 (95% CI -0.03-0.62), respectively. The number needed to treat to potentially treat one life-threatening arrhythmia for the PRIMaCY clinical, PRIMaCY genetic, and HCM Risk-Kids models was 13.7, 14.5, and 9.4, respectively. CONCLUSION: Although PRIMaCY has a similar discriminatory ability to that reported for HCM Risk-Kids, estimated risk estimates did not correlate well with observed risk. A higher proportion of patients met implantable cardioverter-defibrillator thresholds using PRIMaCY model compared with HCM Risk-Kids. This has important clinical implications as these patients will be exposed to a lifetime risk of complications and inappropriate therapies.


Assuntos
Cardiomiopatia Hipertrófica , Desfibriladores Implantáveis , Criança , Humanos , Morte Súbita Cardíaca/epidemiologia , Morte Súbita Cardíaca/etiologia , Morte Súbita Cardíaca/prevenção & controle , Cardiomiopatia Hipertrófica/complicações , Cardiomiopatia Hipertrófica/diagnóstico , Cardiomiopatia Hipertrófica/terapia
20.
Future Cardiol ; 19(13): 661-670, 2023 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-37933625

RESUMO

Hypertrophic cardiomyopathy (HCM) is a phenotypically heterogeneous disease with a genetic basis and variable penetrance. The hallmarks of HCM include dynamic left ventricular outflow tract obstruction, typically caused by asymmetric septal hypertrophy. However, abnormal papillary muscle placement, abnormal mitral valve and subvalvular apparatus and apical hypertrophic forms have also been described. Typical medical treatment has been stagnant for decades, although there have been significant advances in surgical treatment of patients with obstructive HCM. Herein, we describe a new class of drugs targeting the specific pathophysiology of HCM.


Hypertrophic obstructive cardiomyopathy is a genetic condition that leads to increased heart muscle size. This increase in heart muscle can cause blockage of appropriate blood flow out of the heart. In such cases, current medications like ß-blockers, calcium channel blockers and disopyramide can be used, but do not directly target the problem of increased heart muscle size. A new medication class, cardiac myosin inhibitors, decrease the squeezing power of the increased heart muscle to allow for more appropriate blood flow out of the heart. So far, trials have been conducted with mavacamten, with upcoming trials of aficamten (another novel cardiac myosin inhibitor). Recent trials with mavacamten have shown that this medication class has been beneficial for patients in whom other medications have failed. Some trials have also shown that by taking cardiac myosin inhibitors, patients have been able to avoid or delay surgery to correct this problem. Reassuringly, short-term data for this class of medications are positive. However, given that these medications are new, continued monitoring and research is being done to evaluate their long-term effect.


Assuntos
Cardiomiopatia Hipertrófica Familiar , Cardiomiopatia Hipertrófica , Doenças das Valvas Cardíacas , Obstrução da Via de Saída Ventricular Esquerda , Humanos , Cardiomiopatia Hipertrófica/terapia
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