About familial dilated cardiomyopathy (fdc)
What is familial dilated cardiomyopathy (fdc)?
Pediatric cardiomyopathy is a rare heart condition that affects infants and children. Specifically, cardiomyopathy means disease of the heart muscle (myocardium). Several different types of cardiomyopathy exist and the specific symptoms vary from case to case. In some cases, no symptoms may be present (asymptomatic); in many cases, cardiomyopathy is a progressive condition that may result in an impaired ability of the heart to pump blood; fatigue; heart block; irregular heartbeats (tachycardia); and, potentially, heart failure and sudden cardiac death.
Cardiomyopathy may be termed ischemic or nonischemic. Ischemic cardiomyopathy refers to cases that occur due to a lack of blood flow and oxygen (ischemia) to the heart. Such cases often result from hardening of the arteries (coronary artery disease). Nonischemic cardiomyopathy refers to cases that occur due to structural damage or malfunction of the heart muscle. Nearly all cases of pediatric cardiomyopathy are nonischemic. This report deals with nonischemic pediatric cardiomyopathy.
Cardiomyopathy may also be termed primary or secondary. Primary cardiomyopathy refers to cases where cardiomyopathy occurs by itself or for unknown reasons (idiopathic). Secondary cardiomyopathy refers to cases where the disease occurs secondary to a known cause such as heart muscle inflammation (myocarditis) caused by viral or bacterial infections; exposure to certain toxins such as heavy metals or excessive alcohol use; or certain disorders that affect the heart and/or additional organs systems. According to the Pediatric Cardiomyopathy Registry, approximately 79 percent of pediatric cardiomyopathy cases occur for unknown reasons (idiopathic).
Nonischemic cardiomyopathy may be further divided into four subtypes based upon the specific changes within the heart. These subtypes are: dilated, hypertrophic, restrictive and arrhythmogenic right ventricular dysplasia.
What are the symptoms for familial dilated cardiomyopathy (fdc)?
Signs and symptoms of dilated cardiomyopathy may include:
- Shortness of breath (dyspnea) during activity or while lying down
- Reduced ability to exercise
- Swelling (edema) in the legs, ankles, feet or belly (abdomen)
- Chest pain or discomfort
- Fast, fluttering or pounding heartbeat (Palpitations)
What are the causes for familial dilated cardiomyopathy (fdc)?
It may be difficult to determine the cause of dilated cardiomyopathy. However, many things can cause the left ventricle to dilate and weaken, including:
- Certain infections
- Complications of late-stage pregnancy
- Excessive iron in the heart and other organs (hemochromatosis)
- Heart rhythm problems (arrhythmias)
- High blood pressure (hypertension)
- Heart valve disease, such as mitral valve or aortic valve regurgitation
Other possible causes of dilated cardiomyopathy include:
- Alcohol misuse
- Exposure to toxins, such as lead, mercury and cobalt
- Use of certain cancer medications
- Use of illegal drugs, such as cocaine or amphetamines
What are the treatments for familial dilated cardiomyopathy (fdc)?
Treatment of dilated cardiomyopathy depends on the causes. The goals of treatment are to reduce symptoms, improve blood flow and prevent further heart damage. Dilated cardiomyopathy treatment may include medications or surgery to implant a medical device that helps the heart beat or pump blood.
A combination of medications may be used to treat dilated cardiomyopathy and prevent any complications. Medications are used to:
- Control the heart's rhythm
- Help the heart pump better
- Lower blood pressure
- Prevent blood clots
- Reduce fluid from the body
Drugs that are used to treat heart failure and dilated cardiomyopathy include:
- Blood pressure medications. Different types of drugs may be used to lower blood pressure, improve blood flow and reduce the strain on the heart. Such medications include beta-blockers, angiotensin-converting enzyme (ACE) inhibitors and angiotensin II receptor blockers (ARBs).
- Sacubitril/valsartan (Entresto). This drug combines an ARB with another type of medicine to help the heart better pump blood to the rest of the body. It's used to treat those with chronic heart failure.
- Water pills (diuretics). A diuretic removes excess fluid and salt from the body. Too much fluid in the body strains the heart and can make it difficult to breathe.
- Digoxin (Lanoxin). This drug can strengthen heart muscle contractions. It also tends to slow the heartbeat. Digoxin may reduce heart failure symptoms and make it easier to be active.
- Ivabradine (Corlanor). Rarely, this drug may be used to manage heart failure caused by dilated cardiomyopathy.
- Blood-thinners (anticoagulants). These drugs help prevent blood clots.
Surgeries or other procedures
Surgery may be needed to implant a device to control the heart's rhythm or help the heart pump blood. Type of devices used to treat dilated cardiomyopathy include:
- Biventricular pacemaker. This device is for people who have heart failure and irregular heartbeats. A biventricular pacemaker stimulates both of the lower heart chambers (the right and left ventricles) to make the heart beat better.
- Implantable cardioverter-defibrillators (ICD). An ICD doesn't treat cardiomyopathy itself. It monitors the heart rhythm and delivers electrical shocks if an irregular heartbeat (arrhythmia) is detected. Cardiomyopathy can cause dangerous arrhythmias, including those that cause the heart to stop.
- Left ventricular assist devices (LVAD). This mechanical device helps a weakened heart pump better. A LVAD usually is considered after less invasive approaches are unsuccessful. It can be used as a long-term treatment or as a short-term treatment while waiting for a heart transplant.
If medications and other treatments for dilated cardiomyopathy no longer work, a heart transplant may be needed.
What are the risk factors for familial dilated cardiomyopathy (fdc)?
Risk factors for dilated cardiomyopathy include:
- Damage to the heart muscle from certain diseases, such as hemochromatosis
- Family history of dilated cardiomyopathy, heart failure or sudden cardiac arrest
- Heart valve disease
- Inflammation of the heart muscle from immune system disorders, such as lupus
- Long-term excessive alcohol or illegal drug use
- Long-term high blood pressure
- Neuromuscular disorders, such as muscular dystrophy