The European Society of Cardiology (ESC) has recently published the first international guidelines on cardiomyopathies in the European Heart Journal. This groundbreaking document covers all subtypes of cardiomyopathies and provides specific recommendations for conditions other than hypertrophic cardiomyopathy.
Led by Dr. Elena Arbelo from the Hospital Clinic, University of Barcelona, Spain, and Dr. Juan Pablo Kaski from University College London, UK, the guidelines reflect the advancements in genetics, cardiac imaging, and targeted treatments for different causes of the disease.
Cardiomyopathies are conditions that affect the heart muscle, resulting in structural abnormalities, decreased pumping ability, and irregular heart rhythms. They can occur at any age and have varying risks and complications. Some types of cardiomyopathies are more common than others, and symptoms can range from mild to severe, including fatigue, shortness of breath, chest pain, palpitations, and fainting. Complications such as stroke or sudden death can also occur.
The guidelines emphasize the importance of accurate diagnosis and management across all subtypes of cardiomyopathies. Diagnosis involves evaluating the heart’s structure and function through imaging techniques such as echocardiography and cardiac magnetic resonance imaging. Genetic testing and family history assessment also play a significant role in arriving at an accurate diagnosis.
Inherited cardiomyopathies can have implications on various aspects of a patient’s life, including participation in competitive sports and decisions about family planning. Genetic counseling is strongly recommended to help patients and their families understand the implications of a genetic disease. Psychological support should also be provided to patients who undergo implantable cardioverter defibrillator (ICD) implantation or have a family history of sudden cardiac death.
Management of cardiomyopathies aims to alleviate symptoms, prevent complications, and screen at-risk relatives. Identifying individuals at an increased risk of sudden death is a major focus, and shared decision-making is advised when selecting treatments such as ICDs. Drug therapy and heart transplantation are also viable options for treatment. For inherited forms of cardiomyopathies, early diagnosis and treatment in family members can help prevent disease progression and complications.
The guidelines also address the importance of patient education and provide advice on various aspects of living with a cardiomyopathy. This includes guidance on exercise, diet, alcohol consumption, weight management, reproductive issues, sexual activity, medication, vaccination, driving, employment, holidays and travel insurance, and life insurance.
A significant change brought about by these guidelines is the recommendation for regular low to moderate intensity exercise for all patients with cardiomyopathy who are physically capable. Previously, many patients limited their activity due to fears of sudden cardiac death, missing out on the benefits of exercise in preventing heart disease. Individualized risk assessments should be performed to ensure safe exercise prescription.
The ESC guidelines highlight the need for a multidisciplinary approach to patient care and emphasize the importance of seamless transition from pediatric to adult cardiomyopathy services. Collaboration between specialist cardiomyopathy teams, cardiologists, geneticists, genetic counselors, pathologists, and other healthcare professionals is essential for providing integrated and comprehensive care.
In summary, the publication of the first international guidelines on cardiomyopathies marks a significant milestone in the care of heart disease. By incorporating the latest advancements in genetics, cardiac imaging, and targeted treatments, these guidelines aim to improve diagnosis, management, and overall outcomes for patients with cardiomyopathies.
