- HCM: the disease
- What is Hypertrophic Cardiomyopathy (HCM)?
- How common is Hypertrophic Cardiomyopathy?
- History and other names
- How does Hypertrophic Cardiomyopathy affect the heart?
- When Does Hypertrophic Cardiomyopathy Develop?
- What is "obstruction"in HCM?
- Atrial fibrillation in HCM
- How is Hypertrophic Cardiomyopathy Diagnosed?
- Heart Failure in HCM
- What Symptoms Does Hypertrophic Cardiomyopathy Cause?
- How to screen for HCM
- Treatment Options
- HCM: Genetics
- Is a Cure Available?
- After a Loss
- HCM Spectrum Disorders
In some cases a number of specific complications can occur:
Arrhythmias, irregularities of the heart beat, are a common complication. Symptoms such as palpitation may occur, but not often. Exercise testing or Holter monitoring may detect them. The arrhythmias called ventricular tachycardia (arising from the ventricles) or atrial fibrillation (described below) are particularly important and may require treatment. Atrial Fibrillation, the normal regular rhythm of the heart beat is lost and replaced by an irregular rhythm which may be episodic or persistent. The loss of normal atrial (the top of the heart) contraction produces a risk of clot formation in the atria. Anticoagulants and drugs to slow the heart rate are required.
This is an infection of the heart which occurs rarely in Hypertrophic Cardiomyopathy. Bacteria in the bloodstream can stick to the inside of the heart where it has been roughened by turbulent blood flow. **As uncommon as this is, the Founder of the HCMA fell victim to this. The HCMA advises extreme caution when undergoing any “invasive” medical procedure**
The normal electrical signal may travel down to the ventricles slowly or may even be completely blocked, “heart block”. If this occurs, a pacemaker is implanted (see “other forms of therapy”).
Overall, in patients with Hypertrophic Cardiomyopathy there is an increased risk of premature death, which can occur with little or no warning. Sudden death can strike at any age. In the past the risk of sudden death was thought to be much higher than we believe it to be today. It is estimated that the risk of sudden death is between 1 and 2 % in the HCM population. There are members of the HCM population at a higher risk for sudden cardiac arrest, SCA, or sudden cardiac death, SCDand for those at higher risk it is advised that they consult with their health care provider about receiving an implantable defibrillator.
Fluid Congestion: if the heart becomes less efficient as a pump, the body will try to compensate for it. One way it attempts to do this is by using hormones and nerve signals to increase blood volume (by water retention in the kidneys). A drop in blood flow to the kidneys will also lead to fluid retention. Blood and fluid pressure backed up behind the heart result in excess salt water entering the lungs and other body tissues. However, it is important to note that not all swelling due to fluid retention is a reflection of heart failure. Clinical symptoms due to fluid congestion: shortness of breath and edema (pooling of fluid in lungs and body)
Reduced Blood Flow to the Body: The heart’s inability to pump blood to the muscles and organs isn’t always apparent in early stages of heart failure. Often times, it is unmasked only during increases in physical activity. In advanced heart failure, many tissues and organs may not even receive the oxygen they require for functioning at rest. Clinical symptoms due to poor blood flow to the body: difficulty exercising, fatigue and dizziness (due to low blood pressure).
“Burnt out” or “End Stage” HCM
The terms associated with this part of the HCM disease process are misleading and rather depressing. It is our goal to help readers gain a better understanding of this rare but serious potential consequence of HCM, occurring in approximately 3% of the total HCM population. It is poorly understood at this time why this occurs. Some theories suggest it is the individual genetic mutation that determines the progression of the disease.
While “End stage”/”Burnt out” is not precisely the same it is remarkably similar to the dilated form of cardiomyopathy. In dilated cardiomyopathy (often referred to as congestive cardiomyopathy), the heart reaches a point of generalized weakening and thinning of the muscle walls with dilated chambers, particularly the left ventricle. While the walls may have previously been normal or thickened from overwork through trying to compensate for an inadequate ability to pump, (or in the case of HCM), previously “thick” muscle now changes to a different form of damaged tissue. The dilation and thinning of the cardiac chambers, especially of the left ventricle, is often referred to as “remodeling”. This weakening and dilation of the heart muscle eventually leads to heart failure. previously normal or in the case of HCM, previously “thick” heart muscle changes to a different form of damaged tissue, leading to a generalized weakening of the walls of the cardiac chambers. To compensate for the weakening of their muscular walls, the cardiac chambers dilate. The ejection fraction will drop going below 45% in some cases down to the teens. These patients may move on to heart transplantation.
Diastolic heart failure
A less understood form of heart failure in HCM is diastolic heart failure or dysfunction occurs when signs and symptoms of heart failure are present but the left ventricular systolic function is preserved (normally with ejection fractions of 45% or greater but often less than 60%). The total % of patients in this classification is not clear. Diastolic dysfunction raises the pressure gradient of blood in the pulmonary vessels, which causes fluid to leak from these vessels into the lung alveoli, causing pulmonary edema. This condition impairs oxygenation of blood in the lungs, causing shortness of breath and even death if the condition is not detected and treated promptly. Patients with this form of heart failure are managed closely and several medications may be used to alleviate symptoms. Patients with diastolic heart failure in HCM may quality for heart transplant if other treatments have failed.
Maron, B.J. and Salberg, L. Hypertrophic Cardiomyopathy: For patients, their families and interested physicians. Blackwell Futura: 1st edition 2001,81 pages; 2nd edition 2006, 113 pages; 3rd edition pending publication 2014
Gersh, B.J., Maron, B.J., Bonow, R.O., Dearani, J.A., Fifer, M.A., Link, M.S., et al. (2011). 2011 ACCF/AHA guidelines for the diagnosis and treatment of hypertrophic cardiomyopathy. A report of the American College of Cardiology Foundation/American Heart Asociation Task Force on practice guidelines. Journal of the American College of Cardiology and Circulation, 58, e212-260.