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1. Hypertrophic cardiomyopathy

Hypertrophic cardiomyopathy Hypertrophic cardiomyopathy - Symptoms, diagnosis and treatment | BMJ Best Practice You'll need a subscription to access all of BMJ Best Practice Search  Hypertrophic cardiomyopathy Last reviewed: February 2019 Last updated: April 2018 Summary Most common cardiomyopathy and the most frequent cause of sudden cardiac death in young people. Presentation varies from asymptomatic to symptoms of heart failure. Physical examination may be normal at rest. Auscultation along (...) cardiomyopathy (HCM) is a genetic disorder characterised by left ventricular hypertrophy (LVH) without an identifiable cause. It is the most common genetic heart disease as well as the most frequent cause of sudden cardiac death in young people. Maron BJ, Olivotto I, Maron MS. The dilemma of left ventricular outflow tract obstruction and sudden death in hypertrophic cardiomyopathy: do patients with gradients reallydeserve prophylactic defibrillators? Eur Heart J. 2006 Aug;27(16):1895-7. https

2018 BMJ Best Practice

3. Effect of Trimetazidine Dihydrochloride Therapy on Exercise Capacity in Patients With Nonobstructive Hypertrophic Cardiomyopathy: A Randomized Clinical Trial. Full Text available with Trip Pro

Effect of Trimetazidine Dihydrochloride Therapy on Exercise Capacity in Patients With Nonobstructive Hypertrophic Cardiomyopathy: A Randomized Clinical Trial. Hypertrophic cardiomyopathy causes limiting symptoms in patients, mediated partly through inefficient myocardial energy use. There is conflicting evidence for therapy with inhibitors of myocardial fatty acid metabolism in patients with nonobstructive hypertrophic cardiomyopathy.To determine the effect of oral therapy with trimetazidine (...) after adjustment for their baseline walking distance measurements. After adjustment for baseline values, peak oxygen consumption was 1.35 mL/kg per minute lower (95% CI, -2.58 to -0.11 mL/kg per minute; P = .03) in the intervention group after 3 months.In symptomatic patients with nonobstructive hypertrophic cardiomyopathy, trimetazidine therapy does not improve exercise capacity. Pharmacologic therapy for this disease remains limited.ClinicalTrials.gov identifier: NCT01696370.

2019 JAMA cardiology Controlled trial quality: predicted high

4. Influence of centre expertise on the diagnosis and management of hypertrophic cardiomyopathy: A study from the French register of hypertrophic cardiomyopathy (REMY). (Abstract)

Influence of centre expertise on the diagnosis and management of hypertrophic cardiomyopathy: A study from the French register of hypertrophic cardiomyopathy (REMY). Our knowledge of hypertrophic cardiomyopathy (HCM) mainly originates from quarternary centres. The objective is to assess the current management of HCM patients in a large multicentre French register according to the level of expertise.A total of 1431 HCM patients were recruited across 26 (11 expert and 15 non-expert) centres

2018 International journal of cardiology

5. Genetic characterization and genotype-phenotype associations in a large cohort of patients with hypertrophic cardiomyopathy - An ancillary study of the Portuguese registry of hypertrophic cardiomyopathy. (Abstract)

Genetic characterization and genotype-phenotype associations in a large cohort of patients with hypertrophic cardiomyopathy - An ancillary study of the Portuguese registry of hypertrophic cardiomyopathy. We present an ancillary study of the Portuguese Registry of Hypertrophic Cardiomyopathy (PRo-HCM). This is one of the largest HCM genetic studies based on a registry.Collected genetic variants were re-analysed for pathogenicity. Demographic, clinical, imaging and outcome data were analysed

2018 International journal of cardiology

6. Eligibility and Disqualification Recommendations for Competitive Athletes With Cardiovascular Abnormalities: Task Force 3: Hypertrophic Cardiomyopathy, Arrhythmogenic Right Ventricular Cardiomyopathy and Other Cardiomyopathies, and Myocarditis

Eligibility and Disqualification Recommendations for Competitive Athletes With Cardiovascular Abnormalities: Task Force 3: Hypertrophic Cardiomyopathy, Arrhythmogenic Right Ventricular Cardiomyopathy and Other Cardiomyopathies, and Myocarditis Eligibility and Disqualification Recommendations for Competitive Athletes With Cardiovascular Abnormalities: Task Force 3: Hypertrophic Cardiomyopathy, Arrhythmogenic Right Ventricular Cardiomyopathy and Other Cardiomyopathies, and Myocarditis (...) With Cardiovascular Abnormalities: Task Force 3: Hypertrophic Cardiomyopathy, Arrhythmogenic Right Ventricular Cardiomyopathy and Other Cardiomyopathies, and Myocarditis A Scientific Statement From the American Heart Association and American College of Cardiology , MD, FACC, Chair , MD, FAHA, FACC , MD, MS, FAHA, MACC , MD, FAHA, MACC , MD, PhD , MD, FACC , MD, FAHA, FACC , and MD, FACC MD, FACCon behalf of the American Heart Association Electrocardiography and Arrhythmias Committee of the Council on Clinical

2015 American Heart Association

7. Mavacamten Treatment for Obstructive Hypertrophic Cardiomyopathy: A Clinical Trial. (Abstract)

Mavacamten Treatment for Obstructive Hypertrophic Cardiomyopathy: A Clinical Trial. Mavacamten, an orally administered, small-molecule modulator of cardiac myosin, targets underlying biomechanical abnormalities in obstructive hypertrophic cardiomyopathy (oHCM).To characterize the effect of mavacamten on left ventricular outflow tract (LVOT) gradient.Open-label, nonrandomized, phase 2 trial. (ClinicalTrials.gov: NCT02842242).5 academic centers.21 symptomatic patients with oHCM.Patients in cohort

2019 Annals of Internal Medicine

8. Homozygous missense MYBPC3 Pro873His mutation associated with increased risk for heart failure development in hypertrophic cardiomyopathy Full Text available with Trip Pro

Homozygous missense MYBPC3 Pro873His mutation associated with increased risk for heart failure development in hypertrophic cardiomyopathy Hypertrophic cardiomyopathy (HCM) is a primary autosomal-dominant disorder of the myocardium with variable expressivity and penetrance. Occasionally, homozygous sarcomere genetic variants emerge while genotyping HCM patients. In these cases, a more severe HCM phenotype is generally seen. Here, we report a case of HCM that was diagnosed clinically at 39 years

2018 ESC heart failure

9. Left Ventricular Relaxation Half-Time as a Predictor of Cardiac Events in Idiopathic Dilated Cardiomyopathy and Hypertrophic Cardiomyopathy With Left Ventricular Systolic and/or Diastolic Dysfunction. (Abstract)

Left Ventricular Relaxation Half-Time as a Predictor of Cardiac Events in Idiopathic Dilated Cardiomyopathy and Hypertrophic Cardiomyopathy With Left Ventricular Systolic and/or Diastolic Dysfunction. Diastolic dysfunction preceding systolic dysfunction is considered an important interaction in cardiomyopathy with poor prognosis. The aim of this study was to compare left ventricular (LV) isovolumic relaxation with the other parameters as a potential prognostic marker for patients (...) with idiopathic dilated cardiomyopathy (IDC) and hypertrophic cardiomyopathy (HC).A total of 145 patients with IDC and 116 with HC were evaluated for hemodynamic parameters; LV pressure was directly measured by a micromanometer catheter, and relaxation half-time (T1/2) was used to determine LV isovolumic relaxation. The median follow-up period was 4.7 years.The mean ages of the patients with IDC and HC were 52.0 ± 12.0 and 57.1 ± 12.4 years, respectively. Each patient group was further divided into 2 groups

2019 American Journal of Cardiology

10. Defining genotype-phenotype relationships in patients with hypertrophic cardiomyopathy using cardiovascular magnetic resonance imaging. Full Text available with Trip Pro

Defining genotype-phenotype relationships in patients with hypertrophic cardiomyopathy using cardiovascular magnetic resonance imaging. HCM is the most common inherited cardiomyopathy. Historically, there has been poor correlation between genotype and phenotype. However, CMR has the potential to more accurately assess disease phenotype. We characterized phenotype with CMR in a cohort of patients with confirmed HCM and high prevalence of genetic testing.Patients with a diagnosis of HCM, who had

2019 PLoS ONE

11. Association of PET-measured myocardial flow reserve with echocardiography-estimated pulmonary artery systolic pressure in patients with hypertrophic cardiomyopathy. Full Text available with Trip Pro

Association of PET-measured myocardial flow reserve with echocardiography-estimated pulmonary artery systolic pressure in patients with hypertrophic cardiomyopathy. Pulmonary hypertension (PH) is a known complication of HCM and is a strong predictor of mortality. We aim to investigate the relationship between microvascular dysfunction measured by quantitative PET and PH in HCM patients.Eighty-nine symptomatic HCM patients were included in the study. Each patient underwent two 20-min 13N-NH3

2019 PLoS ONE

12. Validation of the 2014 European Society of Cardiology Sudden Cardiac Death Risk Prediction Model Among Various Phenotypes in Japanese Patients With Hypertrophic Cardiomyopathy (Abstract)

Validation of the 2014 European Society of Cardiology Sudden Cardiac Death Risk Prediction Model Among Various Phenotypes in Japanese Patients With Hypertrophic Cardiomyopathy Risk stratification for sudden cardiac death (SCD) is essential in the management of hypertrophic cardiomyopathy (HC). The 2014 European Society of Cardiology SCD risk prediction model (Risk-SCD) is a novel risk scoring system; however, whether it can be applied to Japanese HC and its usefulness among various HC

2018 EvidenceUpdates

13. International External Validation Study of the 2014 European Society of Cardiology Guidelines on Sudden Cardiac Death Prevention in Hypertrophic Cardiomyopathy (EVIDENCE-HCM) Full Text available with Trip Pro

International External Validation Study of the 2014 European Society of Cardiology Guidelines on Sudden Cardiac Death Prevention in Hypertrophic Cardiomyopathy (EVIDENCE-HCM) Identification of people with hypertrophic cardiomyopathy (HCM) who are at risk of sudden cardiac death (SCD) and require a prophylactic implantable cardioverter defibrillator is challenging. In 2014, the European Society of Cardiology proposed a new risk stratification method based on a risk prediction model (HCM Risk-SCD

2018 EvidenceUpdates

14. The demanding grey zone: Sport indices by cardiac magnetic resonance imaging differentiate hypertrophic cardiomyopathy from athlete's heart. Full Text available with Trip Pro

The demanding grey zone: Sport indices by cardiac magnetic resonance imaging differentiate hypertrophic cardiomyopathy from athlete's heart. We aimed to characterize gender specific left ventricular hypertrophy using a novel, accurate and less time demanding cardiac magnetic resonance (CMR) quantification method to differentiate physiological hypertrophy and hypertrophic cardiomyopathy based on a large population of highly trained athletes and hypertrophic cardiomyopathy patients.Elite athletes (...) (n = 150,>18 training hours/week), HCM patients (n = 194) and athletes with hypertrophic cardiomyopathy (n = 10) were examined by CMR. CMR based sport indices such as maximal end-diastolic wall thickness to left ventricular end-diastolic volume index ratio (EDWT/LVEDVi) and left ventricular mass to left ventricular end-diastolic volume ratio (LVM/LVEDV) were calculated, established using both conventional and threshold-based quantification method.Whereas 47.5% of male athletes, only 4.1

2019 PLoS ONE

15. Prognostic significance of anterior mitral valve leaflet length in individuals with a hypertrophic cardiomyopathy gene mutation without hypertrophic changes Full Text available with Trip Pro

Prognostic significance of anterior mitral valve leaflet length in individuals with a hypertrophic cardiomyopathy gene mutation without hypertrophic changes Previous studies suggest that anterior mitral valve leaflet (AMVL) elongation is a primary phenotypic feature in hypertrophic cardiomyopathy (HCM). Our aim was to assess AMVL length in individuals with HCM gene mutations and in healthy controls and to identify predictors of the development of HCM during follow-up.A total of 133 HCM mutation

2018 Journal of ultrasound

16. Genotype and Lifetime Burden of Disease in Hypertrophic Cardiomyopathy: Insights from the Sarcomeric Human Cardiomyopathy Registry (SHaRe). Full Text available with Trip Pro

Genotype and Lifetime Burden of Disease in Hypertrophic Cardiomyopathy: Insights from the Sarcomeric Human Cardiomyopathy Registry (SHaRe). A better understanding of the factors that contribute to heterogeneous outcomes and lifetime disease burden in hypertrophic cardiomyopathy (HCM) is critically needed to improve patient management and outcomes. The Sarcomeric Human Cardiomyopathy Registry (SHaRe) was established to provide the scale of data required to address these issues, aggregating

2018 Circulation

17. Takotsubo Cardiomyopathy in a Patient with Previously Undiagnosed Hypertrophic Cardiomyopathy with Latent Obstruction Full Text available with Trip Pro

Takotsubo Cardiomyopathy in a Patient with Previously Undiagnosed Hypertrophic Cardiomyopathy with Latent Obstruction A 62-year-old woman with takotsubo cardiomyopathy (TCM) accompanied by cardiogenic shock due to the obstruction of left ventricular outflow tract (LVOT) and massive mitral regurgitation (MR) was admitted to the emergency department. After successful treatment with intensive care, dobutamine stress-echocardiography was performed, which reproduced a dynamic LVOT gradient, severe (...) MR and cardiogenic shock. A histological examination obtained from the right ventricular septum demonstrated hypertrophied and bizarre myocytes, with myocyte disarray. Besides TCM, a diagnosis of preexisting hypertrophic cardiomyopathy with latent obstruction was made. She was discharged with medical therapy including a beta-blocker, which would not be routinely employed in the treatment of a patient with TCM.

2018 Internal Medicine

18. Hypertrophic Cardiomyopathy

Hypertrophic Cardiomyopathy The Cardiac Society of Australia and New Zealand Diagnosis and Management of Hypertrophic Cardiomyopathy – Position Statement This standard of practice document briefly outlines the current approach to the diagnosis and management of hypertrophic cardiomyopathy (HCM). The supporting levels of evidence are reported in both the American Heart Association HCM guidelines(1) and the European Society of Cardiology HCM guidelines.(2) There is also a detailed recent review (...) of HCM for further reference.(3) Since the last CSANZ HCM guidelines in 2011, the main changes relate to emerging sudden death risk factors such as the amount of myocardial fibrosis, the development of an HCM Risk Score, and more careful consideration of cascade genetic testing in asymptomatic relatives without an HCM phenotype. 1. CLINICAL CHARACTERISTICS OF HCM 1.1 Definition and prevalence Hypertrophic cardiomyopathy (HCM) is a primary cardiac disorder characterised by hypertrophy, usually

2016 Cardiac Society of Australia and New Zealand

19. Identifying unmet clinical need in hypertrophic cardiomyopathy using national electronic health records. Full Text available with Trip Pro

Identifying unmet clinical need in hypertrophic cardiomyopathy using national electronic health records. To evaluate unmet clinical need in unselected hypertrophic cardiomyopathy (HCM) patients to determine the risk of a wide range of subsequent cardiovascular disease endpoints and safety endpoints relevant for trial design.Population based cohort (CALIBER, linked primary care, hospital and mortality records in England, period 1997-2010), all people diagnosed with HCM were identified (...) and matched by age, sex and general practice with ten randomly selected people without HCM. Random-effects Poisson models were used to assess the associations between HCM and cardiovascular diseases and bleeding.Among 3,290,455 eligible people a diagnosis of hypertrophic cardiomyopathy was found in 4 per 10,000. Forty-one percent of the 1,160 individuals with hypertrophic cardiomyopathy were women and the median age was 57 years. The median follow-up was 4.0 years. Compared to general population controls

2018 PLoS ONE

20. Prevalence of clinically apparent hypertrophic cardiomyopathy in Germany-An analysis of over 5 million patients. Full Text available with Trip Pro

Prevalence of clinically apparent hypertrophic cardiomyopathy in Germany-An analysis of over 5 million patients. Hypertrophic cardiomyopathy (HCM) is the most common inherited heart disease. Reported prevalence rates vary substantially between 1:500 (0.2%) and 1:3,000 (0.03%), which may be attributed to different study designs and population characteristics. Prevalence data for Germany is not available. Consequently, this study aimed (1) to quantify age- and gender-specific clinically diagnosed

2018 PLoS ONE

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