It’s amazing the impact a word or a group of words can have on your life. In our case; that 14 letter word was Cardiomyopathy and what that meant for our 4-month-old son.
Up until this point, such words did not exist in my vocabulary, as it's use and impact were for others. Life, however, had different ideas and with little to no warning, my vocabulary expanded suddenly with 14 extra letters.
So, faced with this new found word and fueled with a mixture of emotions, I've set out to educate myself; so as to better understand Cardiomyopathy and its place in my little son's life.
A Good Place to Start
Being the logical, methodical individual that I am; a sensible place to start would be with the first 6 letters - Cardio; or, the heart.
Diagram 1: Heart Chambers and Valves
Basic Function of the Heart
The heart is a muscular organ that pumps blood to all the tissues in our body through a network of blood vessels. The right side of the heart pumps blood through the lungs where it picks up oxygen. The left side of the heart receives the blood containing oxygen and pumps the blood to the rest of the body.
The heart consists of four chambers and four valves that regulate blood flow.
- Left and right atria - Chambers that receive blood returning from your body through your veins.
- Left and right ventricles - Chambers where blood is pumped to your body through your arteries.
- Mitral valve - The mitral valve controls the flow of oxygen-rich blood from the left atrium to the left ventricle.
- Tricuspid valve - The tricuspid valve controls the flow of oxygen-poor blood from the right atrium to the right ventricle.
- Aortic valve - The aortic valve controls the flow of oxygen-rich blood from the left ventricle to the body.
- Pulmonary valve - The Pulmonary valve controls the flow of oxygen-poor blood from the right ventricle to the lungs.
For a more visual illustration, the embedded animation shows how a normal heart pumps blood.
What is Cardiomyopathy?
Information for the bulk of this section has been sourced from the Cardiomyopathy UK website; which I found to be a valuable resource.
Cardiomyopathy is a disease of the heart muscle; which could be hereditary affecting one or several members of a family. It is not a single condition, but a group of conditions; which affect the structure of the heart and reduce its ability to pump blood around the body. Anyone can be affected by the disease and its thought 1 in 500 people within the UK have the condition.
Types of Cardiomyopathy
Depending on how the disease affects the heart muscle, cardiomyopathy can be categorised as one of the following types:
Diagram 2: HCM affected Heart
Hypertrophic Cardiomyopathy (HCM)
This is when the muscle of the ventricle thickens (called ‘hypertrophy’) and makes the ventricle smaller. The thickening means that the heart has to work harder to pump blood around the body. It can also block the flow of blood out of the heart.
For the majority of those living with HCM, their quality and duration of life are not limited by the condition. However, for a minority, there is a risk of sudden death and the experience of significant symptoms.
Who can be affected (HCM) :
Caused by either a change or mutation in one or more genes; children of those with HCM have a 50:50 chance of inheriting the genetic mutation in their family.
Symptoms (HCM) :
Most people with HCM may not show symptoms. For those who do, these might vary based on the individual. For some, symptoms might be brought on by exercise.
Symptoms may include:
- Dizziness and Blackouts - Might happen while exercising or for no apparent reason.
- Arrhythmias - Fast and erratic heart rhythms caused by a disruption in the heart's electrical impulses. As a result, the flow of blood could be disturbed, increasing the risk of stroke.
- Chest pain - Tightness or pain on exertion is common and may occur when the heart muscle is not getting sufficient blood supply.
- Fatigue - This may be due to an obstruction of blood flow from the heart.
- Heart block - This is caused when the electrical impulse travels down the ventricles slowly, or may even be completely blocked.
- Endocarditis - Treatable with antibiotics, but a serious infection of the endocardium; the lining of the heart.
- Cardiac arrest - Within a small group of individuals, HCM could cause an arrhythmia that can cause the heart to stop beating.
Treatment (HCM) :
HCM cannot be cured at present, but most symptoms can be controlled. Medicines most commonly used in the treatment of HCM include:
- Betablockers - For the treatment of HCM, they are typically used to control or prevent abnormal heart rhythms.
- Calcium channel blockers (calcium antagonists) - Due to the thickened heart muscle caused by HCM; the contraction force could be much greater than normal, potentially causing some obstruction to blood flow from the heart. Symptoms of obstructions are reduced by Calcium channel blockers; which lessen the tone of the heart's contraction.
- Anti-arrhythmic medicines - These medicines control the rhythm of the heart.
- Anticoagulants - Within some groups of people HCM could cause blood clots to form; which are prevented by anticoagulants.
- Diuretics - Diuretics, or water tablets, increase the output of water and salt in the urine. They help reduce the workload of the heart and ensure that the body does not hold too much salt or water. Diuretics can also help control blood pressure.
- Pacemakers and other heart devices - Although not required for most people with the disease; such devices could be of use for those with a low heart rate, an irregular heart rhythm or pumping problems.
Diagram 3: DCM affected Heart
Dilated Cardiomyopathy (DCM)
DCM causes the pumping chambers in the heart to become enlarged (dilated). As a result, the heart muscle becomes weak, thin and unable to pump blood around the body efficiently.
This condition can lead to fluid building up in the lungs, ankles, abdomen and other organs of the body, leading to a feeling of being breathless.
Who can be affected (DCM) :
DCM is known to be a genetic condition, meaning that it is caused by a change or mutation in one or more genes and is passed on through families.
Symptoms (DCM) :
Symptoms can vary in severity, depending on the individual. However, they generally respond well to the standardised treatments which are currently available.
Symptoms may include:
- Shortness of breath (dyspnoea) - Usually will occur whilst exercising or being active.
- Palpitations - This is a sensation of an extra or skipped heartbeat. In some cases, palpitations may start suddenly and feel very fast, (known as an arrhythmia) and may be accompanied by sweating or light-headedness.
- Ankle and abdominal swelling - Caused as a result of the heart failing to pump efficiently.
- Tiredness - Due to a reduced blood supply to the body; people may experience tiredness, as well as heavy limbs.
- Arrhythmias - Caused when heart's electrical impulses become disrupted, resulting in fast or erratic heart rhythms.
- Blood clots
- Chest pain - It is believed that chest pains occur due to a high pressure on the dilated wall of the left ventricle.
- Heart murmurs - Usually caused when the heart valves do not close effectively following a beat. These unusual sounds can be heard through a stethoscope.
- Endocarditis - Treatable with antibiotics; but both rare and serious, caused by an infection of the lining of the heart (endocardium).
Treatment (DCM) :
DCM cannot be cured at present, but most symptoms can be controlled. Medicines most commonly used in the treatment of DCM include:
- ACE inhibitors - ACE inhibitors (angiotensin converting enzyme inhibitors) are a very important group of drugs in the treatment of DCM which acts to relax the blood vessels, reducing the pumping workload of the heart, and also reduce the amount of water and salt reabsorbed by the body.
- Angiotensin II receptor blockers (ARBs) - Angiotensin II receptor blockers (ARBs) are a group of drugs that have a similar action to ACE inhibitors, and are often used when people experience side effects, particularly a persistent dry cough when taking ACE inhibitors.
- Beta blockers - Beta blockers are used widely in the treatment of cardiac conditions. In dilated cardiomyopathy they are used in small doses to reduce the effect of hormones, called catecholamines, which are released by the body when the heart is not pumping effectively. When used with ACE inhibitors, (or angiotensin II receptor blockers), they can improve the output from the heart and reduce symptoms.
- Diuretics - Diuretics, or water tablets, increase the output of fluid in the urine. They help reduce the workload of the heart and ensure that the body does not hold too much salt or water.
- Anti-arrhythmic drugs - These medicines control the rhythm of the heart. They include beta blockers and calcium channel blockers.
- Anticoagulants - Some people with DCM develop atrial fibrillation, which carries a risk of blood clots forming. Anticoagulants prevent the clots from forming.
- Ivabradine - Ivabradine slows your heart rate so that your heart doesn’t have to work as hard.
- Pacemakers and other heart devices - Pacemakers and other heart devices can be used to treat cardiomyopathy.