hypertrophic%20cardiomyopathy
HYPERTROPHIC CARDIOMYOPATHY

Hypertrophic cardiomyopathy is a genetically determined cardiac disorder characterized by hypertrophy, often of the left ventricle (LV), with nondilated ventricular chambers and no other cardiac or systemic disease (eg aortic stenosis, thyroid disease or hypertension) capable of producing the extent of hypertrophy manifested by the patient.

Symptoms can range from patients being asymptomatic to mildly or significantly symptomatic with pre-syncopal or syncopal episodes, palpitations, chest pain, decreased exercise tolerance, symptoms of pulmonary congestion (eg fatigue, dyspnea, orthopnea and paroxysmal nocturnal dyspnea) and symptoms of end-stage heart failure.

Approach to treatment is based on clinical experience and observational data as large randomized trials regarding medical therapy in hypertrophic cardiomyopathy are lacking.

An empiric therapy is often necessary to identify the best possible drug for the patient, starting at a low dose and titrating until clinical improvement or occurrence of side effects.

Treat comorbidities such as hypertension, diabetes, obesity and hyperlipidemia according to existing guidelines due to the significant influence of concomitant coronary artery disease on the survival of patients with hypertrophic cardiomyopathy.

 

Hypertrophic%20cardiomyopathy Management

Counselling

Family Counseling

  • Helps in checking the extent of the condition in a family and in protecting other family members through further counseling and genetic testing
  • It is recommended that all of clinically affected patient’s first-degree relatives be screened for HCM with individually tailored time intervals, typically every 5 years in adults and 12-18 months in children and adolescents
    • Clinical screening is long term but becomes less frequent in older age groups
    • Evaluation will include ECG and echocardiography

Follow Up

  • A lifelong follow-up is generally required in patients with HCM in order to identify changes in symptoms or development of adverse events
  • Frequency of patient’s monitoring is based upon the age, symptoms and disease severity
  • On routine follow-up, it is recommended that patients have:
    • A 12-lead ECG, transthoracic echocardiography and 48-hour Holter monitoring whenever there are new or changed symptoms, every 1-2 years if patient is clinically stable
    • An exercise test annually if there are progressive symptoms, every 2-3 years if patient is clinically stable
    • A cardiac MRI every 2-3 years if there are progressive symptoms, every 5 years if patient is clinically stable
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