A diagnosis of hypertrophic cardiomyopathy (HCM) often brings a mix of relief and concern. Finally having an explanation for shortness of breath, chest pressure, or unexpected fatigue is validating, but learning that your heart muscle has thickened can be unsettling. For many, the condition remains mild and manageable, but for others, it significantly limits physical activity and creates anxiety about heart health. It is important to know that while HCM is a chronic genetic condition, it is highly treatable.

Treatment aims to improve the heart’s pumping efficiency, relieve symptoms, and prevent complications such as arrhythmias or heart failure. By relaxing the stiff heart muscle and controlling the heart rate, medical therapy helps patients return to active, comfortable lives. Because the severity of the thickening and the obstruction of blood flow vary greatly from person to person, treatment plans are tailored to individual needs (American Heart Association, 2024).

Overview of treatment options for Hypertrophic Cardiomyopathy

The primary goal of HCM treatment is to reduce the workload on the heart. Strategies focus on improving the heart’s ability to fill with blood and, if an obstruction exists, reducing the pressure gradient that blocks blood flow leaving the heart.

Pharmacological therapy is the cornerstone of management for the vast majority of symptomatic patients. The approach is generally conservative, starting with medications that slow the heart rate and relax the muscle. Invasive procedures, such as septal myectomy (surgery) or alcohol septal ablation, are typically reserved for patients who have severe obstruction that does not improve with medication. Therefore, finding the right drug regimen is the first and most critical step in care.

Medications used for Hypertrophic Cardiomyopathy

Beta-blockers are universally considered the first-line treatment for symptomatic HCM. Medications such as metoprolol, atenolol, or bisoprolol are commonly prescribed. These drugs are effective at relieving chest pain and shortness of breath during exertion. Clinical experience suggests that many patients report a noticeable improvement in their exercise tolerance shortly after starting therapy.

For patients who cannot tolerate beta-blockers or do not find relief with them, non-dihydropyridine calcium channel blockers are the standard alternative. Verapamil and diltiazem are the key drugs in this class. Like beta-blockers, they help manage symptoms by targeting heart rate and stiffness.

A significant advancement in HCM treatment is the introduction of cardiac myosin inhibitors. Mavacamten is a newer, targeted medication specifically approved for obstructive HCM. Unlike older drugs that manage symptoms broadly, this medication targets the source of the hyper-contraction.

In some cases where obstruction remains severe despite initial treatment, a drug called disopyramide may be added. This is an antiarrhythmic agent used specifically for its ability to weaken the force of the heart’s contraction (Mayo Clinic, 2023).

How these medications work

Beta-blockers and calcium channel blockers work by “turning down” the body’s adrenaline response. By blocking the effects of adrenaline on the heart, they slow the heart rate and reduce the force of the contraction. This is crucial in HCM because a slower heart rate gives the thickened, stiff ventricles more time to relax and fill with blood before pumping it out.

Cardiac myosin inhibitors like mavacamten work at a molecular level. In HCM, the heart muscle contracts too forcefully because of an excess number of connections (cross-bridges) between muscle proteins. This medication reduces these connections, effectively allowing the heart muscle to relax and reducing the obstruction to blood flow (Food and Drug Administration, 2022).

Side effects and safety considerations

Beta-blockers and calcium channel blockers, while generally safe, can cause fatigue, dizziness, or cold extremities due to slowing the heart and lowering blood pressure; this can cause lightheadedness upon standing up quickly.

Cardiac myosin inhibitors, which weaken heart contraction, require strict safety monitoring (regular echocardiograms) to prevent excessive reduction of the heart’s pumping function.

Patients with HCM typically avoid diuretics (“water pills”) and vasodilators (like nitrates) because they can worsen obstruction by dehydrating or widening blood vessels excessively. Patients must seek immediate care for fainting, rapid/irregular heartbeats, or severe chest pain.

Since everyone’s experience with the condition and its treatments can vary, working closely with a qualified healthcare provider helps ensure safe and effective care.

References

1. American Heart Association. https://www.heart.org
2. Mayo Clinic. https://www.mayoclinic.org
3. Food and Drug Administration. https://www.fda.gov
4. National Heart, Lung, and Blood Institute. https://www.nhlbi.nih.gov