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Overview of HCM

Hypertrophic cardiomyopathy (HCM) is an inherited equal-opportunity disease that may appear without warning or an obvious family history, making diagnosis often difficult.

It affects 1 in 500 individuals across all age groups, both genders and every race. In general, one half of all offspring of a known HCM patient will inherit the gene, although when and how the disease manifests itself is highly variable.

Characterized by myocardial disarray that creates abnormalities in the heart muscle fibers and makes accurate diagnosis and treatment important, HCM often involves:

Clinical presentation is diverse, ranging from asymptomatic individuals to those with:

  • disabling symptoms of heart failure
  • exercise intolerance and fatigue
  • shortness of breath (dyspnea)
  • chest pain
  • palpitations
  • lightheadedness or passing out (syncope)

HCM is also the most common cause of sudden cardiac death in individuals under 30 who appear otherwise healthy. A conclusive diagnosis, which may include genetic testing, is vital to the prognosis and determination of treatment options.

While diagnosis traditionally involves an echocardiogram, the subtleties of the disease manifestations may be difficult to discern, requiring comprehensive focused echocardiographic evaluations using multiple views. When echocardiogram is inconclusive or suspicious for severe thickening or apical variant, cardiac MRI may be required. Other diagnostic testing may include periodic Holter monitoring, stress testing, and cardiac catheterization.

Although there is no cure, most symptomatic patients can be managed with:

When symptoms persist despite the above measures, surgical myectomy or catheter-based alcohol septal ablation (ASA) may be used to:

Most important, HCM is a family disease, and it is not enough to simply treat the patient. All blood relatives must be evaluated by genetic testing or echocardiogram� the latter at routine intervals regardless of initial results � and all children need to be evaluated by pediatric cardiologists specializing in the disease.

The HCM Center gladly helps patients and families coordinate the prescribed care, and fully expects to forge a lifelong relationship with each patient. We believe that our experience and expertise in treating HCM patients has led to improved outcomes and satisfaction.

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