IDIOPATHIC HYPERTROPHIC SUBAORTIC STENOSIS (IHSS)
DESCRIPTIONIdiopathic hypertrophic subaortic stenosis is a chronic heart condition that produces an enlarged heart muscle, restricting the amount of blood the heart pumps. Only the heart is involved.
Appropriate health care includes:
Physician's monitoring of general condition and medications, 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.
SIGNS & SYMPTOMSChest pain (angina pectoris).
Shortness of breath.
Swollen feet and ankles.
Distended neck veins.
Enlarged and tender liver (under the rib cage).
Heart murmur (See Glossary).
The underlying cause is unknown. The effects cause thickening of the left chamber (ventricle) of the heart. This obstructs the flow of blood, and the heart may be unable to pump enough blood during exertion. In some cases, this condition may be inherited as a dominant genetic trait.
Family history of IHSS.
PREVENTING COMPLICATIONS OR RECURRENCEIf you have a family history of IHSS, obtain genetic counseling before starting a family.
Your own observation of symptoms.
Medical history and physical exam by a doctor.
Laboratory studies, such as cardiac catheterization to measure blood flow through the heart chambers.
X-rays of the heart.
EKG and echocardiogram (See Glossary for both) of the heart.
Special studies that may include ultrasonography, CAT or CT scan, MRI, and radionuclide scan (See Glossary for all).
POSSIBLE COMPLICATIONSFatal heartbeat irregularity.
Usually controllable with medication or surgery.
Keep your child under close medical supervision.
Urge your child to wear a Medic-Alert bracelet or pendant (See Glossary).
Family members, close friends, and business acquaintances should learn cardiopulmonary resuscitation (CPR), in case cardiac arrest occurs.
MEDICATIONBeta-adrenergic blockers or calcium-channel blockers to prevent heartbeat irregularities.
Don't give your child nitroglycerin for angina pain. It dilates arteries, which may be harmful.
If digitalis is prescribed for your child, discuss the risks with your doctor. It may trigger heartbeat irregularities.
See Medications section for information regarding medicines your doctor may prescribe.
Your doctor may prescribe:
Your doctor should guide you and your child about how much physical activity is ideal. The ability to increase activity is dependent on the response to therapy. Don't regard your child as an invalid.
DIET & FLUIDS
A low-salt diet (See Appendix 29) may be necessary, if your child has fluid accumulation (a possible sign of congestive heart failure).
OK TO GO TO SCHOOL?When appetite has returned and alertness, strength, and feeling of well-being will allow.
CALL YOUR DOCTOR IF
Your child has symptoms of IHSS, or symptoms worsen during treatment.
New, unexplained symptoms develop. Drugs used in treatment may produce side effects.