Prognostic implications of different clinical profiles in hypertrophic cardiomyopathy

Domitilla Russo1, Matteo Sclafani1, Giacomo Tini1

  • 1Unit of Cardiology, Department of Clinical and Molecular Medicine, Faculty of Medicine and Psychology, Sant'Andrea Hospital, Sapienza University, Rome, Italy.

Insights

Hypertrophic cardiomyopathy (HCM) is a common genetic heart condition with varied presentations. This review details distinct clinical subgroups and their specific prognostic implications for better patient management.

Area of Science:

  • Cardiology
  • Genetics
  • Internal Medicine

Background:

  • Hypertrophic cardiomyopathy (HCM) is a prevalent genetic myocardial disorder.
  • HCM exhibits significant heterogeneity in clinical presentation, natural history, and prognosis.
  • Approximately 60% of HCM patients experience a stable clinical course.

Purpose of the Study:

  • To delineate distinct clinical subgroups within the HCM patient population.
  • To describe the unique characteristics of each identified HCM subgroup.
  • To elucidate the prognostic implications associated with each clinical profile.

Main Methods:

  • This review synthesizes existing literature on hypertrophic cardiomyopathy.
  • Clinical profiles and prognostic factors were identified and categorized.
  • Subgroups were defined based on specific HCM-related complications and disease manifestations.

Main Results:

  • HCM patients can be classified into distinct subgroups, including those with outflow tract obstruction, end-stage disease, apical hypertrophy or aneurysm, atrial fibrillation, and high risk for sudden death.
  • Preclinical HCM represents another important clinical profile.
  • Each subgroup is associated with specific and relatively independent prognostic pathways.

Conclusions:

  • Understanding these diverse clinical profiles is crucial for accurate prognostication in HCM.
  • Tailoring management strategies based on specific subgroup characteristics can improve patient outcomes.
  • Further research into the independent pathways of HCM complications may refine therapeutic approaches.

Related Concept Videos

Cardiomyopathy III: Hypertrophic Cardiomyopathy01:29

Cardiomyopathy III: Hypertrophic Cardiomyopathy

Hypertrophic cardiomyopathy, or HCM, is an autosomal dominant genetic disorder characterized by asymmetric left ventricular hypertrophy without ventricular dilation. It is more common in men and is typically diagnosed in young, athletic adults.EtiologyHCM is primarily genetic and is caused by mutations in genes encoding sarcomeric proteins. Researchers have identified over 1400 mutations across at least 11 different genes. Among these, the most frequently occurring mutations are found in the...
94
Cardiomyopathy V: Interprofessional Care01:29

Cardiomyopathy V: Interprofessional Care

Managing cardiomyopathy involves addressing underlying or precipitating causes, treating heart failure with medications, and implementing dietary changes and a balanced exercise and rest regimen.Lifestyle ModificationsCardiomyopathy patients should adopt a low-sodium diet to reduce fluid retention and manage heart failure. A personalized exercise and rest plan helps maintain physical fitness without overstraining the heart. Avoiding alcohol and tobacco is essential to prevent further damage to...
65
Cardiomyopathy I: Introduction and Classification01:25

Cardiomyopathy I: Introduction and Classification

Cardiomyopathy, or CMP, is a group of diseases affecting the myocardial structure, impairing its ability to pump blood effectively. This condition can lead to arrhythmias, heart failure, or sudden cardiac death.Cardiomyopathies are classified into primary and secondary categories:Primary Cardiomyopathy refers to conditions involving only the heart muscle that are often idiopathic (of unknown cause) or genetic. They primarily affect the myocardium without the involvement of other systemic...
123
Pathophysiology of Heart Failure01:17

Pathophysiology of Heart Failure

Heart failure (HF) is a progressive syndrome involving ventricles that leads to inadequate cardiac output. It can be classified based on location and output or ejection fraction. Ejection fraction (EF) is an essential measurement in the diagnosis and surveillance of HF. Reduced EF corresponds to systolic heart failure (HFrEF). However, HF with preserved ejection fraction (HFpEF) is becoming increasingly prevalent. Also known as diastolic HF, this form of HF is related to aging. The...
2.0K
Heart Failure IV: Classification and Diagnostic Evaluation01:30

Heart Failure IV: Classification and Diagnostic Evaluation

Heart failure can be classified in various ways, with the most common classifications based on physical activity limitations, disease progression, severity, and treatment strategies.The Functional Classification of Heart Failure divides patients into four categories based on physical activity limitation due to symptom burden.Class I: Patients in this class have cardiac disease but no physical activity limitations. Ordinary activities like walking, climbing stairs, or routine tasks do not cause...
73
Cardiomyopathy II: Dilated Cardiomyopathy01:30

Cardiomyopathy II: Dilated Cardiomyopathy

Dilated cardiomyopathy, or DCM, is a progressive myocardial disorder characterized by ventricular chamber dilation and contractile dysfunction.EtiologyVarious factors can cause DCM, including hypertension and heavy alcohol intake, which contribute to the weakening and enlargement of the heart muscle. Viral infections, such as Coxsackievirus B, adenoviruses, and influenza, can lead to DCM by causing inflammation and damage to heart tissue. Certain chemotherapeutic agents, including daunorubicin,...
73