Camzyos (mavacamten) is a prescription medicine used to treat symptomatic obstructive hypertrophic cardiomyopathy (HCM), helping improve shortness of breath and the ability to be active.  Camzyos is a cardiac myosin inhibitor that relaxes heart muscles so the heart can fill with more blood and pump blood around your body more effectively.

In obstructive hypertrophic cardiomyopathy (HCM), the walls of the heart become excessively thickened and stiff. This reduces blood flow from the heart to your body, which causes symptoms of shortness of breath, rapid heartbeat, feeling light-headed, chest pain, fainting, and tiredness. Camzyos relaxes the heart muscles and reduces heart obstruction, which improves your ability to be active and other symptoms.

How does Camzyos work?

The heart pumps blood around the body as heart muscles contract and release. Heart muscles contract when two proteins called myosin and actin join together, and then muscles relax when myosin and actin are not joined. In obstructive HCM, too many myosin and actin connect, making the heart contract too much, thickening the heart walls, reducing blood flow from the heart, and causing symptoms.

Camzyos’s mechanism of action is a cardiac myosin inhibitor that works by binding to the myosin protein, which stops the actin proteins from binding to it. As a result, the heart muscle relaxes, the heart fills with more blood, and it can pump more effectively. Camzyos is an allosteric and reversible inhibitor selective for cardiac myosin.

Camzyos Indications

Camzyos is indicated for adults with symptomatic obstructive hypertrophic cardiomyopathy (HCM) to help improve functional capacity and symptoms. It can be used in patients with New York Heart Association (NYHA) class II-III obstructive HCM. Camzyos became an FDA-approved medicine on April 28, 2022.

Camzyos side effects

Common Camzyos side effects

Common Camzyos side effects include dizziness (27%) or fainting (6%).

Serious Camzyos side effects

Get emergency medical help if you have signs of an allergic reaction: hives; difficulty breathing; swelling of your face, lips, tongue, or throat.

Camzyos may cause serious side effects. Call your doctor at once if you have:

• heart failure symptoms, such as shortness of breath, chest pain, tiredness, swelling in your legs, rapid weight gain, or racing or pounding heartbeats.

This is not a complete list of side effects, and others may occur. Call your doctor for medical advice about side effects.

Camzyos REMS program

Camzyos is only available through the Camzyos REMS program, which is required by the FDA to monitor the safe use of this medication. The REMS  (Risk Evaluation and Mitigation Strategy) program is required as this medicine can cause the heart muscles to become too relaxed, which increases the risk of heart failure.  Your doctor will review and discuss the REMS Patient Brochure with you and talk to you about how to enroll in the program.

Warnings

Camzyos may cause serious side effects, and carries a Boxed Warning for a risk of heart failure.

Heart failure:

Heart failure is a condition where the heart cannot pump with enough force. Heart failure is a serious condition that can lead to death. You must have echocardiograms before you take the first dose and also during your treatment with this medicine. This is important to help your healthcare provider understand how your heart is responding to this medicine. People who develop a serious infection or irregular heartbeat have a greater risk of heart failure during treatment.

Call your doctor at once if you have shortness of breath, chest pain, tiredness, swelling in your legs, rapid weight gain, or racing or pounding heartbeats.

The risk of heart failure is also increased when Camzyos is taken with certain other medicines. Tell your healthcare provider about the medicines you take, both prescribed and obtained over-the-counter, before and during treatment.

Because of the serious risk of heart failure, this medicine is only available through a restricted program called the Camzyos Risk Evaluation and Mitigation Strategy (REMS) Program.

See Camzyos side effects for information about side effects.

It is not known if Camzyos is safe and effective in children.

Before taking this medicine

Your treatment plan may change if you also use certain other medications such as:

• nefazodone;
• St. John’s wort;
• an antibiotic eg.clarithromycin, rifabutin, rifampin, rifapentine, telithromycin;
• antifungal medicine eg. itraconazole, ketoconazole;
• antiviral medicine for HIV or hepatitis C eg. boceprevir, cobicistat, dasabuvir, elvitegravir, indinavir, lopinavir/ritonavir, nelfinavir, ombitasvir, paritaprevir saquinavir, telaprevir, tipranavir;
• cancer medicine eg. apalutamide, enzalutamide, mitotane;
• seizure medicine eg. carbamazepine, oxcarbazepine, phenytoin, primidone;
• steroid medicine eg. dexamethasone, prednisone;

Tell your doctor if you have ever had:

• an irregular heartbeat;
• heart failure; or
• if you have an infection.

Pregnancy

Camzyos may harm an unborn baby. You may need a pregnancy test to make sure you are not pregnant before using this medicine. Use effective birth control while using this medicine and for at least 4 months after your last dose. Tell your doctor if you become pregnant.

Hormonal contraceptives containing a combination of ethinyl estradiol and norethindrone may be used with this medicine, but it can make other birth control pills less effective. Ask your doctor about all birth control options, such as an injection, implant, skin patch, vaginal ring, condom, diaphragm, cervical cap, or contraceptive sponge.

Breastfeeding

Ask a doctor if it is safe to breastfeed while using this medicine.

How should I take Camzyos?

Take Camzyos once a day.

Swallow the capsule whole. Do not break, open, or chew the capsule.

You will start with an initial dose, and over weeks, your Doctor will increase your dose depending on how you respond to the medicine.

Your healthcare provider may change your dose, temporarily stop, or permanently stop your treatment if you have certain side effects.

Take Camzyos capsules exactly as your healthcare provider tells you to take them.

Do not change your dose without talking to your healthcare provider first.

What happens if I miss a dose?

If you miss a dose, take it as soon as possible and take your next dose at your regularly scheduled time the next day. Do not take 2 doses on the same day to make up for a missed dose.

What happens if I overdose?

If you take too much or overdose, call your healthcare provider or go to the nearest hospital emergency room right away.

Dosing information

Usual Adult Dosage for Hypertrophic Cardiomyopathy

Starting dose: 5 mg orally once a day

• Reduce starting dosage to 2.5 mg/day in those taking moderate CYP2C19 inhibitors or a strong CYP3A4 inhibitor.
• See the Camzyos Prescribing Information for complete dosing information and instructions for regular left ventricular ejection fraction (LVEF) and Valsalva left ventricular outflow tract (LVOT) gradient assessment.

Allowable subsequent doses with titration: 2.5, 5, 10, or 15 mg orally once a day.

Available Strengths 

• 2.5mg, 5mg, 10mg, 15mg capsules.

What other drugs will affect Camzyos?

Tell your doctor about all your other medicines, especially:

• moderate to strong CYP2C19 inhibitors or strong CYP3A4 inhibitors
• moderate to strong CYP2C19 inducers or moderate to strong CYP3A4 inducers
• heart medications such as disopyramide, ranolazine, or verapamil that are taken with a beta blocker, or diltiazem with a beta blocker, as these medications and combinations increase the risk of left ventricular systolic dysfunction and heart failure symptoms
• other medicines to treat obstructive hypertrophic cardiomyopathy; or
• omeprazole, esomeprazole, or cimetidine.

Camzyos is an inducer of CYP3A4, CYP2C9, and CYP2C19. Concomitant use with CYP3A4, CYP2C9, or CYP2C19 substrates may reduce the plasma concentration of these drugs. Ask your healthcare provider if you are taking one of these medications. 

Progestin and ethinyl estradiol are CYP3A4 substrates. Concomitant use of Camzyos may decrease blood levels of certain progestins, which may lead to contraceptive failure. Combined hormonal contraceptives (CHCs) containing a combination of ethinyl estradiol and norethindrone may be used, but if other CHCs are used, patients should add nonhormonal contraception (such as condoms) during concomitant use and for 4 months after the last dose of Camzyos.

What is ivabradine?

Ivabradine works by affecting your heart’s electrical activity in order to slow the heart rate.

Ivabradine is used in adults with chronic heart failure, to help lower the risk of needing to be hospitalized when symptoms get worse.

Ivabradine is also used in children at least 6 months old who have stable heart failure caused by an enlarged heart.

Ivabradine may also be used for purposes not listed in this medication guide.

Ivabradine side effects

Get emergency medical help if you have signs of an allergic reaction: hives; difficult breathing; swelling of your face, lips, tongue, or throat.

Ivabradine may cause serious side effects. Call your doctor at once if you have:

• fast or pounding heartbeats, fluttering in your chest, shortness of breath, and sudden dizziness (like you might pass out);
• very slow heartbeats;
• severe headache, blurred vision, pounding in your neck or ears;
• racing heartbeats with dizziness, tiredness, or a lack of energy;
• chest tightness; or
• shortness of breath that is worse than usual.

If your baby is taking ivabradine, watch for symptoms of feeding problems, trouble breathing, or turning blue.

Ivabradine can cause a temporary brightness in your vision, especially during the first 2 months of treatment. This can make you see halos around lights, see colors within lights, or see multiple images while looking at an object. You may also see kaleidoscope colors or flashes of movement in certain parts of your vision. Sudden bright light can make these vision changes more noticeable. These effects usually go away as you continue taking ivabradine or after you stop taking it.

Common side effects of ivabradine may include:

• slow or irregular heartbeats;
• high blood pressure; or
• your eyes may be more sensitive to light.

This is not a complete list of side effects and others may occur. Call your doctor for medical advice about side effects. You may report side effects to FDA

Warnings

You should not use ivabradine if you have severe liver disease, very low blood pressure, a slow resting heart rate, a serious heart condition such as “sick sinus syndrome” or 3rd-degree “AV block” (unless you have a pacemaker), or if you depend on a pacemaker to control your heart rate.

Tell your doctor about all your current medicines and any you start or stop using. Many drugs can interact, and some drugs should not be used together.

Ivabradine may harm an unborn baby. Use effective birth control to prevent pregnancy, and tell your doctor if you become pregnant.

Ivabradine can cause serious heart rhythm problems. Call your doctor at once if you have chest pressure, racing or pounding heartbeats, very slow heartbeats, weakness, tiredness, severe dizziness, or shortness of breath that is worse than usual.

If your baby is taking ivabradine, watch for symptoms of feeding problems, trouble breathing, or turning blue.

Before taking this medicine

You should not use ivabradine if you are allergic to it, or if you have:

• a serious heart condition such as “sick sinus syndrome” or 3rd-degree “AV block” (unless you have a pacemaker);
• heart failure symptoms that recently got worse;
• very low blood pressure;
• a slow resting heart rate;
• severe liver disease; or
• if you depend on a pacemaker to control your heart rate.

Some medicines can cause unwanted or dangerous effects when used with ivabradine. Your doctor may need to change your treatment plan if you use any of the following drugs:

• an antibiotic or antifungal medicine;
• an antidepressant;
• antiviral medicine to treat hepatitis C or HIV/AIDS; or
• other heart medicine or blood pressure medication (such as amiodarone, digoxin, diltiazem, or verapamil).

Tell your doctor if you have any other heart problems not being treated with ivabradine.

Taking ivabradine during pregnancy may harm an unborn baby or lead to premature birth. Use effective birth control to prevent pregnancy, and tell your doctor if you become pregnant.

You should not breastfeed while using ivabradine.

How should I take ivabradine?

Follow all directions on your prescription label and read all medication guides or instruction sheets. Your doctor may occasionally change your dose. Use the medicine exactly as directed.

Ivabradine is usually taken 2 times per day with food. Avoid grapefruit or grapefruit juice.

Tell your doctor if you have trouble swallowing a ivabradine tablet.

Measure liquid medicine carefully. Use the dosing syringe provided, or use a medicine dose-measuring device (not a kitchen spoon). Each plastic ampule is for one use only. Throw it away after one use, even if there is still medicine left inside.

If a child taking ivabradine spits outs the medicine shortly after taking, do not give another dose. Wait until the next scheduled dose time to give the medicine again.

You may also need to take another medicine called a beta-blocker. Use all medications as directed and read all medication guides you receive. Do not stop taking your medication or change your doses without your doctor’s advice.

Your heart function may need to be checked using an electrocardiograph or ECG (sometimes called an EKG).

Store at room temperature away from moisture, heat, and light. Keep the ampules of liquid ivabradine in the foil pouch and use the medicine right away after opening.

Ivabradine dosing information

Usual Adult Dose for Congestive Heart Failure:

Initial dose: 5 mg orally twice a day with meals
Maximum dose: 7.5 mg orally twice a day

Comments:
-In patients with a history of conduction defects or in patients whom bradycardia could lead to hemodynamic compromise, start with 2.5 mg orally twice a day.
-Assess after 2 weeks and adjust dose to maintain tolerability and achieve resting heart rate between 50 and 60 beats per minute (bpm); if resting heart rate is greater than 60 bpm, increase by 2.5 mg twice daily to a maximum of 7.5 mg twice daily; if resting heart rate is less than 50 bpm or bradycardia signs and symptoms occur, decrease by 2.5 mg twice daily (discontinue if current dose is 2.5 mg orally twice a day).

Use: To reduce worsening heart failure hospitalization risk in patients with stable, symptomatic chronic heart failure and left ventricular ejection fraction (LVEF) at or below 35%, who are in sinus rhythm with resting heart rate at or above 70 bpm and are either unable to tolerate or have a contraindication to beta-blockers.

Usual Pediatric Dose for Congestive Heart Failure:

6 Months or Older:
Less than 40 kg (oral solution):
-Initial dose: 0.05 mg/kg orally twice a day with food; assess patient at 2-week intervals and adjust dose by 0.05 mg/kg to target a heart rate reduction of at least 20%, based on tolerability.
-Maximum dose: 6 months to less than 1 year old: 0.2 mg/kg orally twice a day, up to a total of 7.5 mg orally twice a day; 1 year or older: 0.3 mg/kg orally twice a day, up to a total of 7.5 mg orally twice a day.

40 kg or more (tablets):
-Initial dose: 2.5 mg orally twice a day with food; assess patient at 2-week intervals and adjust dose by 2.5 mg to target a heart rate reduction of at least 20%, based on tolerability.
-Maximum dose: 7.5 mg orally twice a day

Use: Treatment of stable symptomatic heart failure due to dilated cardiomyopathy (DCM) in pediatric patients 6 months or older who are in sinus rhythm with an elevated heart rate.

What happens if I miss a dose?

Skip the missed dose and use your next dose at the regular time. Do not use two doses at one time.

What happens if I overdose?

Seek emergency medical attention or call the Poison Help line .

What should I avoid while taking ivabradine?

Grapefruit may interact with ivabradine and lead to unwanted side effects. Avoid the use of grapefruit products.

Avoid taking an herbal supplement containing St. John’s wort.

Avoid driving or hazardous activity until you know how ivabradine will affect you. Your reactions could be impaired.

Avoid sudden changes in the intensity of light around you, such as going outside in sunlight after being in a dark place. Allow your eyes time to adjust slowly to the light.

What is midodrine?

Midodrine is used to treat low blood pressure (hypotension) that causes severe dizziness or a light-headed feeling, like you might pass out. midodrine is for use only when low blood pressure affects daily life. Midodrine may not improve your ability to perform daily activities

Midodrine may also be used for purposes not listed in this medication guide.

Midodrine side effects

Get emergency medical help if you have signs of an allergic reaction: hives, difficult breathing, swelling of your face, lips, tongue, or throat.

Midodrine may cause serious side effects. Call your doctor at once if you have:

• severely slowed heart rate–weak pulse, severe dizziness or light-headed feeling; or
• dangerously high blood pressure–severe headache, pounding sensation in your ears (“hearing” your heartbeats), blurred vision, buzzing in your ears, anxiety, confusion, chest pain, shortness of breath, uneven heartbeats, seizure.

Common side effects of midodrine may include:

• increased blood pressure when sitting or lying down;
• chills, goosebumps;
• numbness, tingling, or itching (especially in your scalp); or
• increased urination, painful or difficult urination, or sudden urge to urinate.

This is not a complete list of side effects and others may occur. Call your doctor for medical advice about side effects. You may report side effects to FDA .

Warnings

You should not use midodrine if you have severe heart disease, overactive thyroid, an adrenal gland tumor, kidney disease, if you are unable to urinate, or if your blood pressure is high even while lying down.

Midodrine can increase blood pressure even when you are at rest. midodrine should be used only if you have severely low blood pressure that affects your daily life. Midodrine may not improve your ability to perform daily activities.

Before taking this medicine

You should not use midodrine if you are allergic to it, or if you have:

• severe heart disease;
• if you are unable to urinate;
• pheochromocytoma (tumor of the adrenal gland);
• overactive thyroid;
• high blood pressure (even while lying down); or
• kidney disease.

To make sure midodrine is safe for you, tell your doctor if you have or have ever had:

• diabetes;
• glaucoma or a history of vision problems; or
• liver disease.

It is not known if midodrine will harm an unborn baby. Tell your doctor if you are pregnant or plan to become pregnant.

Ask a doctor if it is safe to breastfeed while using this medicine.

How should I take midodrine?

Follow all directions on your prescription label and read all medication guides or instruction sheets. Use the medicine exactly as directed.

Midodrine is usually taken 3 times per day, with doses spaced at least 3 hours apart. Take your last dose of the day before evening meal or 4 hours before bedtime.

You may take midodrine with or without food.

Take midodrine during your normal waking hours, when your are most likely to be upright and not lying down or napping. Ask your doctor about how to take this medicine if you normally lie down during the day.

Midodrine can increase your blood pressure even while you are lying down or sleeping (when blood pressure is usually lowest). Long-term high blood pressure (hypertension) can lead to serious medical problems.

Follow your doctor’s instructions about the best way to position your body while you are laying down or sleeping. You may need to keep your head elevated to help prevent high blood pressure.

Your blood pressure will need to be checked before and during treatment with midodrine. Check your blood pressure while you are lying down, and check it again with your head elevated.

Your kidney and liver function may also need to be checked.

Midodrine is only part of a treatment program that may also include lifestyle changes, wearing support stockings on your legs, and possibly special medical care. Follow your doctor’s instructions very carefully.

Store at room temperature away from moisture and heat.

Midodrine dosing information

Usual Adult Dose for Hypotension:

10 mg orally 3 times a day during daytime hours when the patient needs to be upright

Comments:
-The three daily doses may be given at 3-hour intervals, if needed, but not more frequently.
-This drug should not be given after the evening meal or less than 4 hours before bedtime.
-This drug should only be continued in patients who appear to attain symptomatic improvement during initial treatment.

Use: Treatment of symptomatic orthostatic hypotension (OH) in patients whose lives are considerably impaired despite standard clinical care, including non-pharmacologic treatment such as support stockings, fluid expansion, and lifestyle alterations.

What happens if I miss a dose?

Take the medicine as soon as you can, but skip the missed dose if it is almost time for your next dose. Do not take two doses at one time.

You may need to skip a dose if you will be resting or lying down for a long period of time during your normal waking hours. Talk to your doctor about how to adjust your dose schedule if needed.

What happens if I overdose?

Seek emergency medical attention or call the Poison Help line .

Overdose symptoms include increased blood pressure (flushing, headache, pounding heartbeat, blurred vision), goosebumps, feeling cold, or trouble urinating.

What should I avoid while taking midodrine?

Avoid taking a dose if it is less than 4 hours before going to bed.

Ask a doctor or pharmacist before using any over-the-counter diet or cough/cold medicine containing phenylephrine or pseudoephedrine.