IDIOPATHIC HYPERTROPHIC

General Information

DEFINITION--A chronic heart condition that produces an enlarged heart muscle, restricting the amount of blood the heart pumps. Cardiac output may be low, normal or high depending on whether stenosis is obstructive or nonobstructive. If output is normal, IHSS could go undetected for years.

BODY PARTS INVOLVED--Heart.

SEX OR AGE MOST AFFECTED--Both sexes; all ages.

SIGNS & SYMPTOMS

• Chest pain (angina pectoris).
• Heart-rhythm irregularity.
• Fainting.
• Shortness of breath.
• Swollen feet and ankles.
• Distended neck veins.
• Heart failure.
• Heart murmur.

CAUSES--Thickening of the left chamber (ventricle) of the heart for unknown reason. This obstructs the flow of blood, and the heart may be unable to pump enough blood during exertion. In some cases, this condition is inherited as a dominant genetic trait.

RISK INCREASES WITH--Family history of IHSS.

HOW TO PREVENT--If you have a family history of IHSS, obtain genetic counseling before starting a family.

What To Expect

DIAGNOSTIC MEASURES--

• Your own observation of symptoms.
• Medical history and physical exam by a doctor.
• Laboratory studies, such as cardiac catheterization to measure blood flow through heart chambers.
• X-rays of the heart.
• EKG and echocardiogram (See Glossary for both) of the heart.

APPROPRIATE HEALTH CARE

• Doctor's treatment, including consultation with a cardiologist.
• Surgery to reduce the obstruction, if medication does not control the problem.
• DC electrocardioversion (electric shock to the heart) for treatment of life-threatening heartbeat irregularities and to improve heart output.

POSSIBLE COMPLICATIONS

• Heartbeat irregularity.
• Bacterial infection of the heart valve.
• Sudden death.

PROBABLE OUTCOME--Usually curable with medication or surgery.

How To Treat

GENERAL MEASURES--

• Treatment goals are to relax the ventricle and relieve outflow obstruction. First therapy is usually with medications.
• Stay under close medical supervision.
• Psychological counseling for help in adjusting to emotional effects of chronic illness.

MEDICATION--Your doctor may prescribe:

• Beta-adrenergic blockers (usually propranolol) or calcium-channel blockers to prevent heartbeat irregularities.
• Don't use nitroglycerin for angina pain. It dilates arteries, which may be harmful.

ACTIVITY--

• Instructions will be provided about how much physical activity is ideal. Your ability to increase activity is dependent on your response to therapy. Don't regard yourself as an invalid.
• Strenuous activities and sports are to be avoided because of high risk of sudden death.

DIET--Usually no special diet. A low-salt diet may be recommended, if you have fluid accumulation (a possible sign of congestive heart failure).

Call Your Doctor If

• You have symptoms of IHSS, or symptoms worsen during treatment.
• New, unexplained symptoms develop. Drugs used in treatment may produce side effects.