Clinical course of hypertrophic cardiomyopathy with survival to advanced age

Barry J Maron1, Susan A Casey, Robert G Hauser

  • 1Hypertrophic Cardiomyopathy Center, Minneapolis Heart Institute Foundation, 920 E. 28th Street, Suite 60, Minneapolis, MN 55407, USA. hcm.maron@mhif.org

Insights

Hypertrophic cardiomyopathy (HCM) is often well-tolerated in older adults, allowing for normal life expectancy. Many patients experience mild symptoms, with diagnosis and symptom onset often delayed until later in life.

Area of Science:

  • Cardiology
  • Geriatrics
  • Genetics

Background:

  • Adverse outcomes of hypertrophic cardiomyopathy (HCM) are typically emphasized in younger and middle-aged individuals.
  • The clinical course and long-term prognosis of HCM in elderly populations remain less understood.

Purpose of the Study:

  • To elucidate the clinical profile and long-term outcomes of elderly patients diagnosed with hypertrophic cardiomyopathy (HCM).

Main Methods:

  • A community-based cohort of 312 patients with hypertrophic cardiomyopathy (HCM) was assessed to avoid tertiary center referral bias.
  • Long-term outcomes, including life expectancy, symptom severity, and survival probabilities, were analyzed.

Main Results:

  • 23% of patients achieved a normal life expectancy (>=75 years), with 14% living to >=80 years.
  • Most elderly HCM patients (64%) experienced mild symptoms and few clinical consequences, with diagnosis and symptom onset delayed to ages 74 and 70, respectively.
  • Survival probabilities for patients >=50 years at diagnosis did not significantly differ from the general population; elderly women with HCM showed less wall thickness and more frequent basal outflow obstruction.

Conclusions:

  • Hypertrophic cardiomyopathy (HCM) is frequently well-tolerated in older individuals, compatible with normal life expectancy.
  • The disease can remain clinically dormant for extended periods, with diagnosis and symptoms often appearing late in life.
  • These findings offer reassurance to patients, suggesting a potentially less unfavorable and unpredictable course for HCM than previously assumed.
Abstract

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...
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...
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,...
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...
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...
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...