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Cardiomyopathies

Randomized Clinical Trial: Aficamten improves peak oxygen uptake in symptomatic obstructive hypertrophic cardiomyopathy – N Engl J Med

27 May, 2024 | 20:24h | UTC

Study Design and Population: This phase 3, double-blind trial randomized 282 adults with symptomatic obstructive hypertrophic cardiomyopathy (HCM) to receive aficamten or placebo for 24 weeks. The primary outcome was the change in peak oxygen uptake, with secondary outcomes including changes in the Kansas City Cardiomyopathy Questionnaire score and New York Heart Association functional class.

Main Findings: Aficamten significantly increased peak oxygen uptake by 1.7 ml/kg/min (95% CI, 1.0 to 2.4; P<0.001) compared to placebo. Improvements were also seen in all secondary outcomes. The incidence of adverse events was similar between the groups.

Implications for Practice: Aficamten shows promise in improving exercise capacity and symptoms in patients with obstructive HCM, potentially offering a new therapeutic option. Further research may confirm its long-term benefits and safety.

 

Reference (link to abstract – $ for full-text):

Maron, M. S., et al. (2024). Aficamten for Symptomatic Obstructive Hypertrophic Cardiomyopathy. New England Journal of Medicine. DOI: 10.1056/NEJMoa2401424.

 


2024 AHA/ACC/AMSSM/HRS/PACES/SCMR Guideline for the Management of Hypertrophic Cardiomyopathy – J Am Coll Cardiol

11 May, 2024 | 14:20h | UTC

In a significant advancement for the treatment of hypertrophic cardiomyopathy (HCM), the American Heart Association and the American College of Cardiology, along with other leading societies, have released updated guidelines to optimize patient care. Here are the essential updates and recommendations for practicing physicians:

1 – Updated Diagnostic Strategies: The guideline emphasizes the use of advanced imaging techniques and genetic testing to enhance diagnostic accuracy, enabling personalized treatment approaches.

2 – Risk Assessment Tools: Revised tools for sudden cardiac death (SCD) risk assessment are detailed, aiding clinicians in making informed decisions regarding the use of implantable cardioverter-defibrillators (ICDs).

3 – Management of Obstructive HCM: New recommendations for the pharmacological treatment of symptomatic obstructive HCM include the use of disopyramide and advanced therapies such as septal reduction when initial medication does not suffice.

4 – Guidelines on Exercise and Lifestyle: The guidelines provide a nuanced approach to physical activity, recognizing the benefits while outlining the risks for patients with HCM. Detailed advice is offered on managing competitive sports involvement and other lifestyle considerations.

5 – Multidisciplinary Approach: The guidelines advocate for a team-based approach involving specialized HCM centers, ensuring patients benefit from comprehensive expertise and the latest treatment modalities.

6 – Innovations in Treatment: Highlighting new therapeutic options like myosin inhibitors, the guidelines underscore their role in managing obstructive symptoms when traditional medications are inadequate.

7 – Special Populations: Detailed sections on the management of HCM in children and pregnant women, addressing the unique challenges these groups face.

These guidelines represent a cornerstone in the evolving landscape of HCM management, embodying a commitment to enhancing outcomes and quality of life for patients through evidence-based practices and collaborative care.

 

Reference (link to free full-text):

Ommen, S. R., Ho, C. Y., Asif, I. M., et al. (2024). 2024 AHA/ACC/AMSSM/HRS/PACES/SCMR Guideline for the Management of Hypertrophic Cardiomyopathy: A report of the American Heart Association/American College of Cardiology Joint Committee on Clinical Practice Guidelines. Journal of the American College of Cardiology. https://doi.org/10.1016/j.jacc.2024.02.014

 


Cohort Study | Younger age at hypertrophic cardiomyopathy diagnosis, male sex among predictors of developing LV systolic dysfunction

11 Aug, 2023 | 15:16h | UTC

Left Ventricular Systolic Dysfunction in Patients Diagnosed With Hypertrophic Cardiomyopathy During Childhood: Insights From the SHaRe Registry – Circulation

 

Commentary on Twitter

 


Phase 1 Trial | NI006 shows potential for cardiac amyloid depletion in transthyretin amyloid cardiomyopathy

1 Aug, 2023 | 14:17h | UTC

Phase 1 Trial of Antibody NI006 for Depletion of Cardiac Transthyretin Amyloid – New England Journal of Medicine (link to abstract – $ for full-text)

Commentaries:

Amyloid Removal Looks Possible in ATTR Cardiomyopathy Imaging Trial – TCTMD

Antibody NI006 for Depletion of Cardiac Transthyretin Amyloid – American College of cardiology

 


AHA Scientific Statement | Treatment strategies for cardiomyopathy in children

12 Jun, 2023 | 13:49h | UTC

Treatment Strategies for Cardiomyopathy in Children: A Scientific Statement From the American Heart Association – Circulation

News Releases:

Identifying the cause of heart muscle disease in children is key to effective treatment – American Heart Association

Children with heart muscle disease need more personalized treatment plans – American Heart Association

Top Things to Know: Treatment Strategies for Cardiomyopathy in Children – American Heart Association

 


Review | Takotsubo syndrome: more frequent in women, more dangerous in men

12 May, 2023 | 13:21h | UTC

Takotsubo syndrome: more frequent in women, more dangerous in men – European Heart Journal Supplements

See all articles in the series here


Guideline | Diagnosis and treatment of myocarditis

23 Mar, 2023 | 12:55h | UTC

JCS 2023 Guideline on the Diagnosis and Treatment of Myocarditis – Circulation Journal (see PDF)

Related: Brazilian Society of Cardiology Guideline on Myocarditis – 2022 – Arquivos Brasileiros de Cardiologia

 


Review | Cardiac sarcoidosis: phenotypes, diagnosis, treatment, and prognosis

20 Mar, 2023 | 13:21h | UTC

Cardiac sarcoidosis: phenotypes, diagnosis, treatment, and prognosis – European Heart Journal

Related: Pathophysiological Gaps, Diagnostic Challenges, and Uncertainties in Cardiac Sarcoidosis – Journal of the American Heart Association

 


Review | Pathophysiological gaps, diagnostic challenges, and uncertainties in cardiac sarcoidosis

17 Mar, 2023 | 12:47h | UTC

Pathophysiological Gaps, Diagnostic Challenges, and Uncertainties in Cardiac Sarcoidosis – Journal of the American Heart Association

 


Phase 2 RCT | Aficamten in patients with obstructive hypertrophic cardiomyopathy

14 Feb, 2023 | 10:37h | UTC

Phase 2 Study of Aficamten in Patients With Obstructive Hypertrophic Cardiomyopathy – Journal of the American College of Cardiology

 


Review | Valvular heart disease: shifting the focus to the myocardium

7 Feb, 2023 | 13:37h | UTC

Valvular heart disease: shifting the focus to the myocardium – European Heart Journal

 

Commentary on Twitter

 


2023 ACC expert consensus decision pathway on comprehensive multidisciplinary care for the patient with cardiac amyloidosis

24 Jan, 2023 | 14:29h | UTC

2023 ACC Expert Consensus Decision Pathway on Comprehensive Multidisciplinary Care for the Patient With Cardiac Amyloidosis: A Report of the American College of Cardiology Solution Set Oversight Committee – Journal of the American College of Cardiology

Key Points: 2023 ACC Expert Consensus on Cardiac Amyloidosis – American College of Cardiology

News Release: ACC Expert Consensus Decision Pathway Focuses on Diagnosis and Management of Cardiac Amyloidosis – American College of Cardiology

 

Commentary on Twitter

 


The year in cardiovascular medicine 2022: the top 10 papers in heart failure and cardiomyopathies

9 Jan, 2023 | 14:10h | UTC

The year in cardiovascular medicine 2022: the top 10 papers in heart failure and cardiomyopathies – European Heart Journal

 

Commentary on Twitter

 


Cohort Study | Prognostic prediction of genotype vs. phenotype in genetic cardiomyopathies.

23 Nov, 2022 | 13:57h | UTC

Prognostic Prediction of Genotype vs Phenotype in Genetic Cardiomyopathies – Journal of the American College of Cardiology

 

Commentary on Twitter

 

Under a Creative Commons license

 


Review | Differing strategies for sudden death prevention in hypertrophic cardiomyopathy.

4 Nov, 2022 | 13:28h | UTC

Differing strategies for sudden death prevention in hypertrophic cardiomyopathy – Heart

 


Consensus Paper | Diagnosis and management of syncope in hypertrophic cardiomyopathy.

31 Oct, 2022 | 13:49h | UTC

Syncope in hypertrophic cardiomyopathy (part II): An expert consensus statement on the diagnosis and management – International Journal of Cardiology

 


Cohort Study | Highly malignant disease in childhood-onset arrhythmogenic right ventricular cardiomyopathy.

24 Oct, 2022 | 14:08h | UTC

Highly malignant disease in childhood-onset arrhythmogenic right ventricular cardiomyopathy – European Heart Journal

Editorial: Arrhythmogenic cardiomyopathies in children: seek and you shall find – European Heart Journal

 

Commentary on Twitter

Under a https://creativecommons.org/licenses/by-nc/4.0/ License

 


Review | Restrictive cardiomyopathy: definition and diagnosis.

24 Oct, 2022 | 13:58h | UTC

Restrictive cardiomyopathy: definition and diagnosis – European Heart Journal

 

Commentary on Twitter

Under a https://creativecommons.org/licenses/by-nc/4.0/ License

 


Review | Cardiac care of children with Duchenne muscular dystrophy (DMD) and females carrying DMD-gene variations.

20 Oct, 2022 | 12:21h | UTC

Cardiac care of children with dystrophinopathy and females carrying DMD-gene variations – openheart

 


Incidence of myocarditis/pericarditis following mRNA COVID-19 vaccination among children and younger adults in the US.

13 Oct, 2022 | 13:51h | UTC

Incidence of Myocarditis/Pericarditis Following mRNA COVID-19 Vaccination Among Children and Younger Adults in the United States – Annals of Internal Medicine

News Release: Incidence of myocarditis/pericarditis following mRNA COVID-19 vaccination among children, younger adults in the US – American College of Physicians

 

Commentary on Twitter (thread – click for more)

 

Commentary on Twitter

 


Guideline | Evaluation and management of myocarditis.

12 Oct, 2022 | 13:56h | UTC

Brazilian Society of Cardiology Guideline on Myocarditis – 2022 – Arquivos Brasileiros de Cardiologia

 


Cohort Study | Cardiovascular MRI phenotypes and long-term outcomes in patients with suspected cardiac sarcoidosis.

30 Sep, 2022 | 12:39h | UTC

Cardiovascular Magnetic Resonance Imaging Phenotypes and Long-term Outcomes in Patients With Suspected Cardiac Sarcoidosis – JAMA Cardiology (link to abstract – $ for full-text)

Commentary: Cardiovascular magnetic resonance imaging phenotype with pathology-frequent late gadolinium enhancement associated with high-risk of ventricular arrhythmic and heart failure events – Physician’s Weekly

 

Commentary on Twitter

 


Clinical risk score to predict pathogenic genotypes in patients with dilated cardiomyopathy.

26 Sep, 2022 | 11:57h | UTC

Clinical Risk Score to Predict Pathogenic Genotypes in Patients With Dilated Cardiomyopathy – Journal of the American College of Cardiology

Editorial: Genetic Testing for Dilated Cardiomyopathy: Old School Is New School – Journal of the American College of Cardiology

News Release: Scientists develop a tool to determine if dilated cardiomyopathy has a genetic origin  – Centro Nacional de Investigaciones Cardiovasculares Carlos III

 

Commentary on Twitter

 


Retrospective Cohort Study | Natural history of MYH7-related dilated cardiomyopathy.

14 Sep, 2022 | 13:06h | UTC

Natural History of MYH7-related Dilated Cardiomyopathy – Journal of the American College of Cardiology

 


20-year follow-up of a RCT trial | Immunosuppressive therapy in virus-negative inflammatory cardiomyopathy.

26 Jul, 2022 | 13:33h | UTC

Immunosuppressive therapy in virus-negative inflammatory cardiomyopathy: 20-year follow-up of the TIMIC trial – European Heart Journal

Commentary: Immunosuppression may benefit long term in virus-negative inflammatory cardiomyopathy – Healio (free registration required)

 

Commentary on Twitter

Under a https://creativecommons.org/licenses/by/4.0/ license

 


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