1. What is Noncompaction Cardiomyopathy?
Noncompaction cardiomyopathy is a rare heart condition that is a congenital birth defect. In noncompaction cardiomyopathy, the growth of portions of the heart are arrested during pregnancy. Portions of the heart remain spongy and have mesh like fibers because they fail to become compact. The healthy portion of the heart becomes overworked and enlarged. This congenital heart defect may occur as a singular defect or may be accompanied by other congenital defects. There may be a family history of noncompaction cardiomyopathy or it may occur spontaneously.
2. What are the Symptoms?
The most common symptoms of noncompaction cardiomyopathy are the symptoms of heart failure. Symptoms of heart failure include difficulty breathing, shortness of breath, swelling of the lower extremities (edema), fatigue and distortion of the jugular vein. A chest x-ray usually shows congestion in the lungs from excess fluid build up. Heart failure is a chronic condition that does not indicate noncompaction cardiomyopathy unless accompanied by other symptoms. The additional symptoms that may indicate noncompaction cardiomyopathy include heart rhythm disorders (arrhythmias), a very fast heart beat (tachycardia) and an incidence of clots (embolisms). A person can experience these symptoms from birth or not have any symptoms until later in life, even though noncompaction cardiomyopathy is a congenital defect.
3. How is it Diagnosed?
The doctor will first do a complete history and physical exam to begin the diagnosis of noncompaction cardiomyopathy. The history will include questions about family history, previous symptoms or episodes of heart failure. During the physical exam, the doctor will listen to your lungs to check for congestion and to check your heart for irregular rhythms. An echocardiogram will be done to visualize how well your heart works. An echocardiogram can also be done in 3-D, which can show noncompaction cardiomyopathy as well. In order for doctors to diagnose noncompaction cardiomyopathy, an echocardiogram is required. Most doctors agree that a history of heart failure and positive results from an echocardiogram are the most reliable ways to diagnose noncompaction cardiomyopathy.
4. How is it Treated?
The treatment for noncompaction cardiomyopathy begins with treating the symptoms of heart failure. Heart failure is treated with medication to reduce edema (diuretics) and medication to reduce high blood pressure. A low sodium diet may also be prescribed to reduce edema. Medications to treat embolisms and arrhythmias may also be prescribed. The arrhythmias may also be treated by the implantation of a defibrillator to regulate heart rhythm. In some cases of noncompaction cardiomyopathy, a heart transplant may be needed.
