Camzyos 5mg Capsules
WARNING: This drug may cause heart failure. You will need to have your heart function checked before you start this drug and while you take it. Call your doctor right away if you have signs of heart problems like new or worse cough or shortness of breath, swelling of the ankles or legs, abnormal heartbeat, weight gain of more than 5 pounds in 24 hours, dizziness, or passing out.There are many other drugs that must not be taken with this drug. Doing so may raise the risk of heart failure and other side effects. Be sure your doctor and pharmacist know about all the drugs that you take. This includes prescription drugs; OTC drugs like omeprazole, esomeprazole, or cimetidine; natural products; and vitamins. @ COMMON USES: It is used to treat a heart problem called hypertrophic cardiomyopathy (HCM).
Drug Class
Cardiac Myosin Inhibitor
Pharmacologic Class
Selective Cardiac Myosin Inhibitor
Pregnancy Category
Not available
FDA Approved
Apr 2022
DEA Schedule
Not Controlled
Overview
What is this medicine?
Camzyos (mavacamten) is a medication used to treat a heart condition called obstructive hypertrophic cardiomyopathy (oHCM). In oHCM, the heart muscle becomes too thick, making it harder for blood to pump out of the heart. Camzyos works by reducing how strongly your heart muscle contracts, which helps to improve blood flow and reduce symptoms like shortness of breath and chest pain.
How to Use This Medicine
Taking Your Medication Correctly
To get the most benefit from your medication, take it exactly as directed by your doctor. Carefully read all the information provided with your prescription and follow the instructions closely. You can take this medication with or without food, but be sure to swallow the tablet whole. Do not chew, crush, or break the tablet.
Continue taking your medication as prescribed by your doctor or healthcare provider, even if you start to feel better. It's essential to complete the full course of treatment as recommended.
Storing and Disposing of Your Medication
To maintain the quality and safety of your medication, store it at room temperature in a dry location, away from the bathroom. Keep all medications in a secure place, out of the reach of children and pets.
When disposing of unused or expired medication, do not flush it down the toilet or pour it down the drain unless specifically instructed to do so. Instead, consult with your pharmacist for guidance on the best disposal method. You may also want to check if there are any drug take-back programs available in your area.
What to Do If You Miss a Dose
If you miss a dose, take it as soon as you remember. However, if it's close to the time for your next scheduled dose, skip the missed dose and resume your regular dosing schedule. Do not take two doses on the same day to avoid overdosing.
To get the most benefit from your medication, take it exactly as directed by your doctor. Carefully read all the information provided with your prescription and follow the instructions closely. You can take this medication with or without food, but be sure to swallow the tablet whole. Do not chew, crush, or break the tablet.
Continue taking your medication as prescribed by your doctor or healthcare provider, even if you start to feel better. It's essential to complete the full course of treatment as recommended.
Storing and Disposing of Your Medication
To maintain the quality and safety of your medication, store it at room temperature in a dry location, away from the bathroom. Keep all medications in a secure place, out of the reach of children and pets.
When disposing of unused or expired medication, do not flush it down the toilet or pour it down the drain unless specifically instructed to do so. Instead, consult with your pharmacist for guidance on the best disposal method. You may also want to check if there are any drug take-back programs available in your area.
What to Do If You Miss a Dose
If you miss a dose, take it as soon as you remember. However, if it's close to the time for your next scheduled dose, skip the missed dose and resume your regular dosing schedule. Do not take two doses on the same day to avoid overdosing.
Lifestyle & Tips
- Take Camzyos exactly as prescribed by your doctor, usually once a day.
- Do not stop taking Camzyos without talking to your doctor, as this can worsen your condition.
- Attend all scheduled appointments for heart monitoring (echocardiograms) as these are crucial to ensure the medication is working safely and effectively.
- Inform your doctor about all medications, supplements, and herbal products you are taking, especially new ones, as many can interact with Camzyos.
- Avoid grapefruit and grapefruit juice while taking Camzyos.
- Report any new or worsening symptoms of heart failure (e.g., increased shortness of breath, swelling, unusual fatigue) immediately to your doctor.
Available Forms & Alternatives
Available Strengths:
Dosing & Administration
Adult Dosing
Standard Dose:
Initial dose of 5 mg orally once daily. Titrate based on LVEF and Valsalva LVOT gradient at Weeks 4, 8, and 12, and then every 12 weeks. Maximum dose is 15 mg once daily.
Dose Range:
5 - 15 mg
Condition-Specific Dosing:
obstructive_hypertrophic_cardiomyopathy:
Initial 5 mg once daily. Titrate to target LVEF âĨ50% and Valsalva LVOT gradient <30 mmHg. Doses are 5 mg, 10 mg, or 15 mg once daily. If LVEF <50%, interrupt treatment. If LVEF <30%, discontinue treatment.
Pediatric Dosing
Neonatal:
Not established
Infant:
Not established
Child:
Not established
Adolescent:
Not established
Dose Adjustments
Renal Impairment:
Mild:
No dose adjustment required.
Moderate:
No dose adjustment required.
Severe:
No dose adjustment required.
Dialysis:
No dose adjustment required for patients on dialysis. Monitor for adverse reactions.
Hepatic Impairment:
Mild:
No dose adjustment required.
Moderate:
No dose adjustment required.
Severe:
Contraindicated in patients with severe hepatic impairment (Child-Pugh Class C).
Pharmacology
Mechanism of Action
Mavacamten is a selective, allosteric, reversible inhibitor of cardiac myosin. It modulates the number of myosin heads that can enter a force-producing state, thereby reducing contractility. Mavacamten reduces the hypercontractility and improves left ventricular outflow tract (LVOT) obstruction in patients with obstructive hypertrophic cardiomyopathy (oHCM).
Pharmacokinetics
Absorption:
Bioavailability:
Not available (oral absorption is good)
Tmax:
1 to 4 hours
FoodEffect:
A high-fat meal decreases Cmax by 18% and AUC by 16%, but this is not considered clinically significant. Can be taken with or without food.
Distribution:
Vd:
Approximately 1000 L
ProteinBinding:
>99%
CnssPenetration:
Limited
Elimination:
HalfLife:
Approximately 6 to 10 days (terminal half-life)
Clearance:
Approximately 4.4 L/hour (oral clearance)
ExcretionRoute:
Fecal (79%), Urine (16%)
Unchanged:
<1% (in urine)
Pharmacodynamics
OnsetOfAction:
Not precisely defined, but clinical effects (e.g., reduction in LVOT gradient) are observed within weeks of initiation.
PeakEffect:
Steady-state concentrations are reached after approximately 6 weeks.
DurationOfAction:
Due to long half-life, effects persist for several weeks after discontinuation.
Safety & Warnings
BLACK BOX WARNING
HEART FAILURE: Mavacamten reduces left ventricular ejection fraction (LVEF) and can cause heart failure. Echocardiogram assessments of LVEF are required prior to and during treatment with Mavacamten. Initiation of Mavacamten in patients with LVEF <55% is not recommended. Interrupt Mavacamten if LVEF <50% at any visit or if the patient experiences heart failure symptoms or worsening clinical status. Concomitant use of Mavacamten with certain cytochrome P450 (CYP) inhibitors or inducers, or with other negative inotropes, is contraindicated due to the risk of heart failure. Mavacamten is available only through a restricted program called the Camzyos REMS Program.
Side Effects
Urgent Side Effects: Seek Medical Help Right Away
While rare, some people may experience severe and potentially life-threatening side effects when taking this medication. Immediately contact your doctor or seek medical attention if you experience any of the following symptoms:
Signs of an allergic reaction, such as:
+ Rash
+ Hives
+ Itching
+ Red, swollen, blistered, or peeling skin with or without fever
+ Wheezing
+ Tightness in the chest or throat
+ Trouble breathing, swallowing, or talking
+ Unusual hoarseness
+ Swelling of the mouth, face, lips, tongue, or throat
New or worsening chest pain
Severe dizziness or fainting
Feeling extremely tired or weak
Other Possible Side Effects
Like all medications, this drug can cause side effects. Although many people may not experience any side effects or only minor ones, it's essential to discuss any concerns with your doctor. Contact your doctor or seek medical help if you experience:
Dizziness
Any other side effects that bother you or do not go away
Reporting Side Effects
This list is not exhaustive, and you may experience other side effects. If you have questions or concerns, contact your doctor. You can also report side effects to the FDA at 1-800-332-1088 or online at https://www.fda.gov/medwatch. Your doctor can provide medical advice about side effects.
While rare, some people may experience severe and potentially life-threatening side effects when taking this medication. Immediately contact your doctor or seek medical attention if you experience any of the following symptoms:
Signs of an allergic reaction, such as:
+ Rash
+ Hives
+ Itching
+ Red, swollen, blistered, or peeling skin with or without fever
+ Wheezing
+ Tightness in the chest or throat
+ Trouble breathing, swallowing, or talking
+ Unusual hoarseness
+ Swelling of the mouth, face, lips, tongue, or throat
New or worsening chest pain
Severe dizziness or fainting
Feeling extremely tired or weak
Other Possible Side Effects
Like all medications, this drug can cause side effects. Although many people may not experience any side effects or only minor ones, it's essential to discuss any concerns with your doctor. Contact your doctor or seek medical help if you experience:
Dizziness
Any other side effects that bother you or do not go away
Reporting Side Effects
This list is not exhaustive, and you may experience other side effects. If you have questions or concerns, contact your doctor. You can also report side effects to the FDA at 1-800-332-1088 or online at https://www.fda.gov/medwatch. Your doctor can provide medical advice about side effects.
Seek Immediate Medical Attention If You Experience:
- New or worsening shortness of breath
- Swelling in your legs, ankles, or feet
- Unusual fatigue or weakness
- Rapid or irregular heartbeat
- Dizziness or lightheadedness
- Fainting (syncope)
- Chest pain
Before Using This Medicine
Before Taking This Medication: Important Information to Share with Your Doctor
It is essential to inform your doctor about the following:
Any allergies you have, including allergies to this medication, its components, or other substances, such as foods or drugs. Describe the allergic reaction you experienced, including any symptoms that occurred.
If you have a history of heart failure, also known as a weak heart.
If you are currently experiencing any of the following health issues:
+ An infection
+ An abnormal heartbeat, such as atrial fibrillation (Afib)
If you are taking any of the following medications:
+ Diltiazem
+ Disopyramide
+ Ranolazine
+ Verapamil
Please note that this is not an exhaustive list of all potential interactions. To ensure your safety, it is crucial to discuss all of your medications (including prescription, over-the-counter, natural products, and vitamins) and health problems with your doctor and pharmacist. They will help you determine whether it is safe to take this medication in conjunction with your other medications and health conditions. Never start, stop, or adjust the dosage of any medication without first consulting your doctor.
It is essential to inform your doctor about the following:
Any allergies you have, including allergies to this medication, its components, or other substances, such as foods or drugs. Describe the allergic reaction you experienced, including any symptoms that occurred.
If you have a history of heart failure, also known as a weak heart.
If you are currently experiencing any of the following health issues:
+ An infection
+ An abnormal heartbeat, such as atrial fibrillation (Afib)
If you are taking any of the following medications:
+ Diltiazem
+ Disopyramide
+ Ranolazine
+ Verapamil
Please note that this is not an exhaustive list of all potential interactions. To ensure your safety, it is crucial to discuss all of your medications (including prescription, over-the-counter, natural products, and vitamins) and health problems with your doctor and pharmacist. They will help you determine whether it is safe to take this medication in conjunction with your other medications and health conditions. Never start, stop, or adjust the dosage of any medication without first consulting your doctor.
Precautions & Cautions
It is essential to inform all your healthcare providers, including doctors, nurses, pharmacists, and dentists, that you are taking this medication.
To avoid potential interactions, do not consume grapefruit or grapefruit juice while taking this drug.
This medication can cause harm to an unborn baby. Therefore, a pregnancy test will be conducted before you start taking this drug to confirm that you are not pregnant. If you are capable of becoming pregnant, it is crucial to use effective birth control during treatment and for a certain period after the last dose. Birth control products containing ethinyl estradiol and norethindrone can be used with this medication. However, other hormone-based birth control methods may not be as effective in preventing pregnancy while taking this drug. Consult your doctor to discuss suitable birth control options and the duration of use. If you become pregnant, notify your doctor immediately.
If you are breastfeeding, inform your doctor, as you will need to discuss the potential risks to your baby.
To avoid potential interactions, do not consume grapefruit or grapefruit juice while taking this drug.
This medication can cause harm to an unborn baby. Therefore, a pregnancy test will be conducted before you start taking this drug to confirm that you are not pregnant. If you are capable of becoming pregnant, it is crucial to use effective birth control during treatment and for a certain period after the last dose. Birth control products containing ethinyl estradiol and norethindrone can be used with this medication. However, other hormone-based birth control methods may not be as effective in preventing pregnancy while taking this drug. Consult your doctor to discuss suitable birth control options and the duration of use. If you become pregnant, notify your doctor immediately.
If you are breastfeeding, inform your doctor, as you will need to discuss the potential risks to your baby.
Overdose Information
Overdose Symptoms:
- Excessive reduction in LVEF
- Symptoms of heart failure (e.g., severe dyspnea, hypotension, cardiogenic shock)
What to Do:
There is no specific antidote for mavacamten overdose. Treatment should be supportive and directed at managing symptoms. Monitor LVEF and hemodynamic status. Consider measures to support cardiac function. Contact a poison control center (e.g., 1-800-222-1222) or seek emergency medical attention immediately.
Drug Interactions
Contraindicated Interactions
- Moderate to strong CYP2C19 inhibitors (e.g., fluconazole, fluvoxamine, ticlopidine)
- Moderate to strong CYP3A4 inhibitors (e.g., clarithromycin, itraconazole, ketoconazole, ritonavir, grapefruit juice)
- Strong CYP3A4 inducers (e.g., rifampin, carbamazepine, phenytoin, St. John's Wort)
- Disopyramide (due to additive negative inotropic effects)
- Verapamil (due to additive negative inotropic effects)
- Diltiazem (due to additive negative inotropic effects)
- Beta-blockers (unless dose adjusted and closely monitored due to additive negative inotropic effects)
Major Interactions
- P-glycoprotein (P-gp) inhibitors (e.g., cyclosporine, amiodarone, verapamil, diltiazem) - potential for increased mavacamten exposure.
- Other negative inotropes (e.g., other calcium channel blockers, other beta-blockers) - increased risk of heart failure.
Moderate Interactions
- Weak CYP2C19 inhibitors (e.g., omeprazole, esomeprazole)
- Weak CYP3A4 inhibitors (e.g., cimetidine, erythromycin)
- Moderate CYP3A4 inducers (e.g., efavirenz, bosentan)
Monitoring
Baseline Monitoring
Left Ventricular Ejection Fraction (LVEF)
Rationale: To establish baseline cardiac function and guide initial dosing.
Timing: Prior to initiation of therapy.
Valsalva Left Ventricular Outflow Tract (LVOT) Gradient
Rationale: To establish baseline obstruction severity and guide initial dosing.
Timing: Prior to initiation of therapy.
Echocardiogram
Rationale: For LVEF and LVOT gradient assessment.
Timing: Prior to initiation of therapy.
Review of Concomitant Medications
Rationale: To identify potential drug-drug interactions, especially with CYP inhibitors/inducers and negative inotropes.
Timing: Prior to initiation of therapy.
Routine Monitoring
Left Ventricular Ejection Fraction (LVEF)
Frequency: At Weeks 4, 8, and 12 after initiation or dose adjustment, then every 12 weeks.
Target: âĨ50%
Action Threshold: If LVEF <50%, interrupt treatment. If LVEF <30%, discontinue treatment.
Valsalva Left Ventricular Outflow Tract (LVOT) Gradient
Frequency: At Weeks 4, 8, and 12 after initiation or dose adjustment, then every 12 weeks.
Target: <30 mmHg
Action Threshold: Used in conjunction with LVEF to guide dose titration.
Echocardiogram
Frequency: At Weeks 4, 8, and 12 after initiation or dose adjustment, then every 12 weeks.
Target: Not applicable
Action Threshold: Used for LVEF and LVOT gradient assessment.
Symptoms of Heart Failure
Frequency: Continuously
Target: Absence or improvement of symptoms
Action Threshold: Worsening symptoms may indicate excessive cardiac depression and require LVEF assessment and dose adjustment/interruption.
Symptom Monitoring
- Dyspnea (shortness of breath)
- Fatigue
- Edema (swelling)
- Palpitations
- Chest pain
- Syncope (fainting)
- Presyncope (lightheadedness)
Special Patient Groups
Pregnancy
Mavacamten can cause embryo-fetal toxicity. Based on animal studies, it may cause adverse developmental outcomes. Females of reproductive potential should use effective contraception during treatment with mavacamten and for at least 6 months after the last dose. A pregnancy test is recommended prior to initiation.
Trimester-Specific Risks:
First Trimester:
Potential for embryo-fetal toxicity, including cardiovascular malformations, based on animal data.
Second Trimester:
Potential for embryo-fetal toxicity, including cardiovascular malformations, based on animal data.
Third Trimester:
Potential for embryo-fetal toxicity, including cardiovascular malformations, based on animal data.
Lactation
It is not known if mavacamten is excreted in human milk. Due to the potential for serious adverse reactions in breastfed infants, breastfeeding is not recommended during treatment with mavacamten and for at least 4 months after the last dose.
Infant Risk:
Potential for serious adverse reactions (e.g., cardiac depression) in breastfed infants.
Pediatric Use
The safety and effectiveness of mavacamten in pediatric patients have not been established.
Geriatric Use
No overall differences in safety or effectiveness were observed between elderly patients (âĨ65 years) and younger patients. No specific dose adjustment is required based on age, but monitor for adverse reactions as with all patients.
Clinical Information
Clinical Pearls
- Mavacamten is the first cardiac myosin inhibitor approved for oHCM, offering a novel mechanism of action.
- Strict adherence to the Camzyos REMS program is mandatory due to the risk of heart failure. This includes regular LVEF monitoring.
- Careful medication reconciliation is critical before and during treatment due to numerous significant drug-drug interactions, especially with CYP inhibitors/inducers and other negative inotropes.
- Patients must be educated on the importance of reporting any new or worsening heart failure symptoms immediately.
- Dose titration is guided by LVEF and Valsalva LVOT gradient, requiring close collaboration between the prescriber and echocardiography lab.
- Due to its long half-life, effects (both therapeutic and adverse) can persist for several weeks after discontinuation.
Alternative Therapies
- Beta-blockers (e.g., metoprolol, propranolol)
- Calcium channel blockers (e.g., verapamil, diltiazem)
- Disopyramide
- Septal myectomy (surgical intervention)
- Alcohol septal ablation (interventional cardiology procedure)
Cost & Coverage
Average Cost:
Highly variable, typically >$10,000 per 30 capsules
Insurance Coverage:
Specialty Tier (requires prior authorization and often step therapy)
General Drug Facts
If your symptoms or health issues persist or worsen, it's essential to contact your doctor promptly. To ensure safe use, never share your medication with others or take someone else's medication. This medication is accompanied by a Medication Guide, which provides crucial information. Please read this guide carefully and review it again whenever you receive a refill. If you have any questions or concerns about this medication, consult your doctor, pharmacist, or healthcare provider. In the event of a suspected overdose, immediately call your local poison control center or seek emergency medical attention. Be prepared to provide details about the overdose, including the substance taken, the amount, and the time it occurred.