RECOMMENDED CARE
The cardiologist will watch for signs of the development of heart failure (symptoms that show that the heart is having difficulty meeting the demands of the body). Cardiologists have several tools at their disposal, including EKG (electrocardiogram), 24-hour Holter monitor (a tiny machine with electrodes that records your EKG for 24 hours), echocardiogram, and cardiac MRI.
Below is the recommended standard care for the heart.
CARDIAC IMAGING
Commonly ordered tests to assess the heart include a cardiac MRI or echocardiogram and electrocardiogram (ECG), which both are used to evaluate cardiac muscle abnormalities or fibrosis (scarring of the heart muscles). The doctor may also order other tests such as a Holter monitor, which is a portable monitor that is worn for 24 hours (or longer). The Holter monitor is used to watch your heart rate and rhythm while you are doing normal activity.
How often should you have your heart checked?
- At diagnosis
Meet with a cardiologist at diagnosis. It is recommended that you have a baseline ECG and echocardiogram.
- At least every year in patients without cardiac symptoms or abnormal imaging
Yearly tests include an ECG, as well as a heart imaging test (such as an echocardiogram or cardiac MRI). It is currently recommended that heart medications be started by age 10, even if your tests are normal and there are no symptoms. Some doctors may start you on medications earlier than age 10.
- If symptoms or abnormal imaging results
Your doctor may recommend more frequent visits in addition to medications.
- Before surgery
It is recommended to have a cardiac evaluation within four months of major surgery, such as scoliosis surgery (to straighten the spine).
cardiac medications
Medications are recommended to help protect the heart muscle and should be started in individuals with Duchenne by age 10 or at first sign of fibrosis. The recommendation timing may vary for individuals with Becker and females with dystrophinopathy as the time of diagnosis may be at a later age. You should always have a conversation with you or your child’s cardiologist about starting medications and understand all side effects.It is important to let your doctor know if you think you may be having side effects from heart medication.
Common heart medications prescribed in dystrophinopathy include:
- ACE-Inhibitors (i.e. Lisinopril, Captopril, Enalapril)
These are typically the first type of medication prescribed by cardiologists in Duchenne. These medications work by helping the blood vessels coming from the heart to relax and open up, making it easier for the heart to pump blood out to the body. ACE inhibitors also lower the amount of water your body retains. This along with widened blood vessels decreases your overall blood pressure. These should be started with the first signs of cardiac fibrosis or dysfunction, or by age 10 even if your tests are normal. Occasionally, patients will develop a cough from ACE inhibitors.
- Angiotensin Receptor Blockers (i.e. Losartan)
ARBs work similarly to ACE inhibitors by helping blood vessels relax and widen, making it easier for the heart to pump blood throughout the body. They help lower blood pressure and reduce strain on the heart. Unlike ACE inhibitors, ARBs are less likely to cause a persistent dry cough and may be recommended if an individual experiences this side effect with an ACE inhibitor. Both medications can lower blood pressure, so regular monitoring of blood pressure and kidney function, as well as blood potassium levels, is important during treatment.
- Beta Blockers (i.e. Propranolol, Metoprolol, Carvedilol)
Beta-blockers help reduce the heart’s workload by slowing the heart rate and decreasing the force of the heart’s contractions. They may be prescribed to help manage certain heart-related changes in individuals with Duchenne. Some people may experience tiredness, dizziness, or a slower heart rate when starting these medications or increasing the dose. Starting at a low dose and increasing it gradually, as directed by the healthcare team, may help reduce side effects. Regular monitoring of heart rate and blood pressure is important to ensure they do not become too low. You should always contact your cardiologist if any concerning symptoms occur.
- Diuretics
These help the body remove extra water, so that there is less blood volume for the heart to pump.
- Antimineralcorticoids (Aldosterone Antagonists) (Eplerenone, Spironolactone)
Eplerenone and spironolactone are medications that block the effects of a hormone called aldosterone, which helps the body regulate salt and water. These medications may be used alongside ACE inhibitors or ARBs to help protect heart muscle in individuals with Duchenne. Research suggests that eplerenone may help slow the development of fibrosis (scarring) in the heart muscle, which can contribute to heart dysfunction over time. The benefits of treatment with spironolactone in Duchenne are also being studied. These medications can affect blood pressure, kidney function, and potassium levels. Regular monitoring through blood pressure checks and blood tests is important during treatment.
