• Overview
• Inheritance
• Symptoms
  • Growth, Bones and Joints
  • Brain and Nerves
  • Physical Appearance
  • Ear, Nose, Throat and Chest
  • Heart
  • Liver, Spleen and Abdomen
  • Eyes
• Testing
• Treatment
• Resources
• Aldurazyme Update

The Heart

Several types of heart abnormalities can be found in people with Hurler syndrome. These abnormalities may lead to heart failure or heart attack (cardiac arrest or myocardial infarction). Serious heart problems may need to be treated with surgery, and/or medication.

Cardiomyopathy
GAG accumulation may result in cardiomyopathy (a weakened heart muscle). This condition causes the heart to pump inefficiently and eventually may cause serious illness or death.

Endocardial fibroelastosis
Storage of GAGs may also cause the heart muscle to stiffen (endocardial fibroelastosis). This condition also causes inefficient pumping of blood and may lead to heart failure.

Coronary artery disease
GAG accumulation in the blood vessels of the heart may narrow the vessels, so that less blood is able to flow through. If blood flow is severely impaired, a heart attack may result.

Heart murmurs
The heart's valves (passages which direct blood flow through the heart's chambers) may also become damaged. If a valve is weakened, some blood may leak back instead of being pushed forward through the heart, and a murmur (an abnormal sound heard when listening to the heart with a stethoscope) may be heard. Murmurs are often harmless, but they may be a sign of a serious heart problem.

Monitoring/Management
Because heart problems occur so often in Hurler syndrome, an electrocardiogram or EKG (a test that measures the electrical current of the heart) and an echocardiogram (an ultrasound of the heart) should be performed periodically to assess the heart's condition.