Hypertrophic cardiomyopathy
Hypertrophic cardiomyopathy (HCM, or HOCM when obstructive) is a condition in which muscle tissues of the heart become thickened without an obvious cause. The parts of the heart most commonly affected are the interventricular septum and the ventricles. This results in the heart being less able to pump blood effectively and also may cause electrical conduction problems. Specifically, within the bundle branches that conduct impulses through the interventricular septum and into the Purkinje fibers, as these are responsible for the depolarization of contractile cells of both ventricles.
| Hypertrophic cardiomyopathy | |
|---|---|
| Other names | Asymmetric septal hypertrophy; idiopathic hypertrophic subaortic stenosis; hypertrophic obstructive cardiomyopathy (HOCM) |
| Specialty | Cardiology |
| Symptoms | Feeling tired, leg swelling, shortness of breath, chest pain, fainting |
| Complications | Heart failure, irregular heartbeat, sudden cardiac death |
| Causes | Genetics, Fabry disease, Friedreich's ataxia, certain medications |
| Diagnostic method | Electrocardiogram, echocardiogram, stress testing, genetic testing |
| Differential diagnosis | Hypertensive heart disease, aortic stenosis, athlete's heart |
| Treatment | Medications, implantable cardiac defibrillator, surgery |
| Medication | Beta blockers, verapamil, disopyramide |
| Prognosis | Less than 1% per year risk of death (with treatment) |
| Frequency | Up to 1 in 200 people |
People who have HCM may have a range of symptoms. People may be asymptomatic, or may have fatigue, leg swelling, and shortness of breath. It may also result in chest pain or fainting. Symptoms may be worse when the person is dehydrated. Complications may include heart failure, an irregular heartbeat, and sudden cardiac death.
HCM is most commonly inherited in an autosomal dominant pattern. It is often due to mutations in certain genes involved with making heart muscle proteins. Other inherited causes of left ventricular hypertrophy may include Fabry disease, Friedreich's ataxia, and certain medications such as tacrolimus. Other considerations for causes of enlarged heart are athlete's heart and hypertension (high blood pressure). Making the diagnosis of HCM often involves a family history or pedigree, an electrocardiogram, echocardiogram, and stress testing. Genetic testing may also be done. HCM can be distinguished from other inherited causes of cardiomyopathy by its autosomal dominant pattern, whereas Fabry disease is X-linked, and Friedreich's ataxia is inherited in an autosomal recessive pattern.
Treatment may depend on symptoms and other risk factors. Medications may include the use of beta blockers, verapamil or disopyramide. An implantable cardiac defibrillator may be recommended in those with certain types of irregular heartbeat. Surgery, in the form of a septal myectomy or heart transplant, may be done in those who do not improve with other measures. With treatment, the risk of death from the disease is less than one percent per year.
HCM affects up to one in 200 people. People of all ages may be affected. The first modern description of the disease was by Donald Teare in 1958.