Logan Steele
Thickened heart muscle, or hypertrophic cardiomyopathy (HCM), is a complex type of heart disease that causes the heart muscle to thicken over time. This thickening can affect any area of the heart's muscle tissue, but it most commonly affects the septum, the wall that divides the heart into two sides. This can reduce blood flow to the aorta and decrease the amount of blood pumped out of the heart with each heartbeat. HCM is usually genetic and can cause serious health complications. It affects about 1 in 500 people in the US, and treatment options include alcohol septal ablation and cardiothoracic surgery.
| Characteristics | Values |
|---|---|
| Definition | Thickened heart muscle, also known as hypertrophic cardiomyopathy (HCM), is a type of heart disease that thickens the heart muscle. |
| Causes | HCM is usually genetic and caused by abnormal genes in the heart muscle. Other causes include longstanding high blood pressure and other genetic and non-genetic conditions. |
| Symptoms | Reduced blood flow to the aorta and reduced pumping of blood to the body. Some people with HCM may not experience any symptoms, while others may only experience symptoms during exercise or exertion. |
| Treatment | Alcohol septal ablation, septal myectomy, cardiothoracic surgery, valve repair, valve replacement, or other cardiac procedures. |
| Prevalence | Affects about 1 in 500 people in the U.S. |
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What You'll Learn
Hypertrophic cardiomyopathy (HCM)
HCM can manifest as either obstructive or nonobstructive forms. Obstructive HCM occurs when the thickened septal wall blocks or reduces blood flow from the left ventricle to the aorta. This type of HCM accounts for about two-thirds of cases. Nonobstructive HCM, on the other hand, involves thickening in other areas of the heart, such as the bottom of the heart, right ventricle, or entire left ventricle, without obstructing blood flow out of the heart.
The symptoms of HCM vary among individuals. Some people may not experience any symptoms at all, while others may only exhibit symptoms during exercise or exertion. Common symptoms include shortness of breath, chest pain, palpitations, and lightheadedness. HCM can also increase the risk of atrial fibrillation, blood clots, stroke, and other heart-related complications. In rare cases, it may lead to sudden cardiac arrest, particularly in young people and athletes.
Diagnosis of HCM typically involves the use of an echocardiogram to assess the function and thickness of the heart muscle and blood flow patterns. A more specialised type of echocardiogram, called a transesophageal echo (TEE), may be performed under sedation. Other diagnostic procedures include cardiac catheterisation and electrophysiological studies. Treatment options include alcohol septal ablation, septal myectomy, and the use of implantable cardioverter defibrillators (ICDs) and medications like Mavacamten.
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Obstructive HCM
Hypertrophic cardiomyopathy (HCM) is a complex type of heart disease that thickens the heart muscle. The condition is usually genetic and can cause serious health complications. HCM affects about 1 in 500 people in the US, with a large percentage remaining undiagnosed. Of those who are diagnosed, it is estimated that two-thirds have obstructive HCM.
Diagnosis of HCM is typically done by echocardiogram, which checks the function and thickness of the heart muscle and how the blood flows through the heart. A swishing or whooshing sound, called a murmur, detected by a stethoscope may indicate a problem with blood flow through the heart and suggest HCM.
There are a range of surgical and nonsurgical procedures used to treat obstructive HCM. Septal myectomy is open-heart surgery where a surgeon removes part of the thickened septum that is bulging into the left ventricle, eliminating the obstruction and restoring blood flow. Alcohol septal ablation is a nonsurgical procedure where ethanol is injected through a tube into the small artery that supplies blood to the area of thickened heart muscle. The alcohol kills the thickened tissue, causing it to shrink to a more normal size. Another treatment option is Mavacamten, a medication that targets the underlying cause of obstructive HCM.
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Nonobstructive HCM
Hypertrophic cardiomyopathy (HCM) is a type of heart disease that causes the thickening of the heart muscle. This thickening can affect any area of the heart's muscle tissue but most commonly affects the septum—the wall that divides the heart into two sides. This thickening reduces the space in the left ventricle, which can decrease the amount of blood pumped out to the body. HCM is usually genetic and can cause serious health complications. It is a lifelong condition and is considered the most common cause of sudden cardiac death in young people and competitive athletes in North America.
In obstructive HCM, the thickened part of the heart muscle blocks or reduces blood flow from the left ventricle to the aorta. This type of HCM accounts for about two-thirds of all cases. On the other hand, nonobstructive HCM (NO-HCM) is characterised by the thickening of the heart muscle in areas other than the septal wall, such as the bottom of the heart, right ventricle, or entire left ventricle. This thickening does not obstruct blood flow out of the heart. NO-HCM accounts for about one-third of all HCM cases.
While NO-HCM does not have a direct impact on survival compared to the general population, it can still lead to adverse disease complications. Patients with NO-HCM may experience severe drug-refractory end-stage heart failure, sudden death, or embolic stroke associated with paroxysmal atrial fibrillation. Additionally, people with HCM, including NO-HCM, are at a higher risk of developing atrial fibrillation, which can result in blood clots, stroke, and other heart-related complications.
The diagnosis of HCM, including NO-HCM, is typically made by a cardiologist or paediatric cardiologist based on medical history, family history, a physical examination, and diagnostic test results. Genetic testing, however, does not play a role in predicting the clinical course or outcome of the disease. Exercise echocardiography and orthostatism are important tools in the evaluation of symptomatic NO-HCM, as they can induce left ventricular outflow tract obstruction, which may not be detected at rest.
The treatment options for HCM aim to protect the heart and overall health. In cases of obstructive HCM, procedures such as septal myectomy or alcohol septal ablation may be performed to eliminate the obstruction and restore blood flow. For NO-HCM, contemporary management options, including implantable defibrillators, surgical myectomy, and heart transplant, have been shown to improve survival rates.
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Alcohol septal ablation
Hypertrophic cardiomyopathy (HCM) is a type of heart disease that causes the heart muscle to thicken. This thickening can affect any area of the heart's muscle tissue but most commonly affects the septum—the wall that divides the heart into two sides. Obstructive HCM occurs when the thickened septum blocks or reduces blood flow from the left ventricle to the aorta. This places extra pressure on the heart and can lead to symptoms such as fatigue and shortness of breath.
The procedure is often favoured over septal myectomy, a type of open-heart surgery that uses a larger incision and requires a longer recovery period. Septal myectomy may be recommended for patients with other heart abnormalities that need to be addressed surgically. In some cases, an implantable cardioverter defibrillator (ICD) may be considered as an alternative treatment option for patients at risk of life-threatening arrhythmias or sudden cardiac death.
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Cardiothoracic surgery
Hypertrophic cardiomyopathy (HCM) is a type of heart disease that causes the heart muscle to thicken. This thickening of the heart muscle can lead to a reduction in blood flow to the aorta and impaired pumping of the heart, resulting in severe tiredness, chest pain, and shortness of breath. In some cases, HCM can also cause irregular heart rhythms and even sudden cardiac death (SCD).
Another option for treating HCM is alcohol septal ablation, also known as nonsurgical septal reduction therapy. This procedure involves injecting ethanol (a type of alcohol) through a tube into the small artery that supplies blood to the thickened heart muscle. The alcohol causes the thickened cells to die and shrink, improving blood flow. Alcohol septal ablation is less invasive than septal myectomy and may be preferred in older patients or those with other medical conditions.
In some cases, patients with HCM may also benefit from the placement of an implantable cardioverter-defibrillator (ICD). This small device is placed just under the skin and constantly monitors the heart rhythm. ICDs can help protect against life-threatening arrhythmias or sudden cardiac death.
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Frequently asked questions
Thickened heart muscle is a condition known as hypertrophic cardiomyopathy (HCM).
HCM is a complex type of heart disease that causes the heart muscle to thicken over time.
HCM is usually genetic and caused by abnormal genes in the heart muscle. However, it can also be caused by other conditions that increase pressure on the heart, such as longstanding high blood pressure.
HCM affects about 1 in 500 people in the US, but many people don't know they have it.
Treatment options for HCM include alcohol septal ablation, where alcohol is injected into the thickened heart muscle to shrink it, and cardiothoracic surgery, where the thickened muscle is shaved away to improve blood flow.
