“Cardiomyopathy can be caused by various factors, including genetic predisposition, viral infections, alcohol abuse, high blood pressure, autoimmune diseases, and certain medications. Complications of cardiomyopathy can include arrhythmias, heart failure, stroke, heart valve disease, cardiac arrest, and cardiogenic shock. Early detection and management are crucial to prevent these complications.”
National Heart, Lung and Blood Institute (Cardiomyopathy – Causes and Risk Factors | NHLBI, NIH)
There are a number of factors that can increase your risk of cardiomyopathy, including:
Cardiomyopathy can lead to other heart conditions, including:
In many cases, you can’t prevent cardiomyopathy. Let your doctor know if you have a family history of the condition.
You can help reduce your chance of cardiomyopathy and other types of heart disease by living a heart-healthy lifestyle and making lifestyle choices such as:
“Cardiomyopathy (kahr-dee-o-my-OP-uh-thee) is a disease of the heart muscle. It causes the heart to have a harder time pumping blood to the rest of the body, which can lead to symptoms of heart failure. Cardiomyopathy also can lead to some other serious heart conditions.
There are various types of cardiomyopathy in all age groups.”
MAYO CLINIC (Cardiomyopathy – Symptoms and causes – Mayo Clinic)
C
Cardiomyopathy is cardiomyopathy, meaning rather in adult or child the same problems in each age group occur but degree of the disease varies for all, it is case by case. What is cardiomyopathy?
Cardiomyopathy is a disease of the heart muscle characterized by an abnormally large, thick or stiff heart muscle. It may affect only the heart’s lower chambers (ventricles) or both the lower and upper chambers (atria). For an adult there just more wear and tear on the organ due to age and severity of the disease.
Cardiomyopathy causes damage to tissue around the heart, as well as heart muscle cells. In severe cases, the heart becomes so weak that it can’t pump blood properly. This can lead to heart failure or irregular heartbeats (arrhythmias). In some cases, cardiomyopathy also involves a buildup of scar tissue or fat within the heart muscle. In rare cases, the heart muscle can’t relax and blood can’t fill the heart properly.
Remember the natural pacemaker in humans is located in the upper right chamber (Rt. atrium) in the upper right region called the sinus node. The sinus node initiates our impulses from that right atrium across to the Lt. atrium and works the impulse sensation down thru the A-V node-atrioventricular node (site between upper and lower chambers in the heart located at the inferior end of the interatrial septum ending at the top of the ventricles in the heart)). From the A-V node the impulse sensation continues on from A-V node to the end of the lower right and left lower chambers (called the ventricles) of the heart that continues up the ventricles and passing through the bundle of His to the bundle branches/Purkinje fibers (The bundle of His is an important part of the electrical conduction system of the heart, as it transmits impulses from the A-V node, located at the inferior end of the interatrial septum, to the ventricles of the heart). Than the impulse sensation goes located in the inner ventricular walls of the heart radiating over the outside of the ventricles = Purkinje Fibers. This whole process allows the heart beat to occur sounding “LubDub”.
This entire impulse conduction process allows the heart to fill up the chambers with blood first in upper chambers to lower ones to releasing the blood in the ventricles (the Rt Ventricle pumps blood to the heart to get more oxygen from lungs that sends this fresh oxygenated blood to the left side of the heart. The left ventricle pumps the filling of its blood into our blood stream called Cardiac Output in creating the heart to beat. Stroke Volume x Heart Rate=our Cardiac Output (SVxHR=CO). Stroke volume is the amount of blood circulated by the heart with each beat x HR = beat in 60 secs.
Cardiomyopathy is very often a “time-will-tell” disease. Symptoms can vary and the progression of the disease can be unpredictable. There are many forms of cardiomyopathy; just like in the adult.
The main types include:
DCM is the most common type and occurs when the main pumping chamber of the heart muscle is too stretched out (dilated). Dilated cardiomyopathy makes the heart unable to pump blood effectively.
HCM makes the heart muscle too thick. Usually, the thickening occurs in the muscle of the left ventricle in the heart, often involving the wall between the heart’s two ventricles.
Restrictive cardiomyopathy is a rare type of cardiomyopathy that causes the heart muscle to become very rigid or stiff. This makes it difficult for the ventricles of the heart to properly fill with blood.
ARVC is a rare form of cardiomyopathy that affects only one in 5,000 people. It occurs when the muscle of the heart’s right ventricle is replaced by thick or fatty scar tissue. The scarring “scrambles” electrical signals within the heart and can make it difficult for the heart to pump blood.
Remember the sinus node (natural pacemaker) of the heart.
Because the symptoms of cardiomyopathy can be diverse, it is often misdiagnosed as asthma, an infection or a gastrointestinal problem but the tests similar to adults tests being ruled out for this disease. Remember its the same problem at a different age.
In children who have no symptoms, cardiomyopathy is sometimes diagnosed when the child has a chest x-ray that shows an enlarged heart or an echocardiogram for another reason.
If your child’s doctor suspects cardiomyopathy, he or she may order one or more of the following tests, from least invasive to most invasive tests:
Your child’s treatment options will be determined by the type of cardiomyopathy he or she has, as well as the specific symptoms. A child with no symptoms might not need medication or other treatment right away. Instead, the cardiologist will monitor your child to gauge the progression of the disease.
A child with more serious symptoms may need additional tests to give the treatment team more detailed information about how the cardiomyopathy is affecting the heart and the rest of the body. .
Many children with cardiomyopathy do well with medication alone. Others need surgery to improve the function of valves, and in the most severe cases, some children need a heart transplant.
There are several different types of medications for cardiomyopathy, depending on which type your child has and the symptoms.
There are several options for treating cardiomyopathy using surgery or minimally-invasive procedures.
For some children with cardiomyopathy — particularly HCM — an implantable cardioverter-defibrillator (ICD) can be a life-saving option. This tiny instrument, about the size of a deck of cards, is placed in the chest to monitor the child’s heartbeat. If the child has an arrhythmia, the defibrillator will administer a precise electrical pulse to restore normal heart rhythm.
Pacemaker implantation is minimally invasive option for some children with cardiomyopathy. The pacemaker — a small electronic device — is inserted directly under the skin, where it sends electric signals to the child’s heart, controlling and monitoring the heart rate. The procedure can be performed under local anesthesia in a matter of hours.
Radiofrequency ablation is another type of minimally invasive treatment that can be very effective for some children with cardiomyopathy due to arrhythmia. A small, needle-like probe is inserted into the scarred tissue of the heart muscle, sending out radiofrequency waves that burns away the scar tissue and the arrhythmia.
In serious cases of hypertrophic cardiomyopathy, the treatment team may perform a surgery called a septal myectomy or septal myomectomy. This procedure involves the removal of a portion of the thickened muscle in the heart, widening the channel in the heart’s ventricle that leads to the aortic valve. The procedure has a very good success rate, and most children have improved blood flow throughout the heart and body.
In certain types of cardiomyopathy, a ventricular-assist device (VAD) can be used to help recover the heart and normalize the heart’s function. After the heart’s function has returned to normal, the VAD will be removed and the child will be monitored for any further heart problems. This approach using the VAD is called a “bridge to recovery.”
Children with the most severe cases of cardiomyopathy may need a heart transplant if other methods don’t manage symptoms. While your child is waiting for an available heart, a VAD may be used to support the heart. In many cases, children can return to school and other activities while waiting for a transplant.
So similar to how adults are treated also with cardiomyopathy. The age is the pretty much the difference.
“In the United States, approximately 1.1 million people, have some form of cardiomyopathy. In 2021, there were 14,770 hospitalizations for cardiomyopathy as the principal diagnosis, and it accounted for 23% of all heart failures. Some people with cardiomyopathy don’t ever get symptoms. For others, symptoms appear as the condition becomes worse. ”
American Heart Association (2024-Statistics-At-A-Glance-final_2024.pdf)
Cardiomyopathy (kahr-dee-o-my-OP-uh-thee) is a disease of the heart muscle that makes it harder for your heart to pump blood to the rest of your body. Cardiomyopathy can lead to heart failure.
There might be no signs or symptoms in the early stages of cardiomyopathy. But as the condition advances, signs and symptoms usually appear, including:
Signs and symptoms tend to get worse unless treated. In some people, the condition worsens quickly; in others, it might not worsen for a long time.
See your doctor if you have one or more signs or symptoms associated with cardiomyopathy. Call 911 or your local emergency number if you have severe difficulty breathing, fainting or chest pain that lasts for more than a few minutes.
Because some types of cardiomyopathy can be hereditary, if you have it your doctor might advise that your family members be checked.
Often the cause of the cardiomyopathy is unknown. In some people, however, it’s the result of another condition (acquired) or passed on from a parent (inherited).
Contributing factors for acquired cardiomyopathy include:
“Health care providers can treat hemochromatosis safely and effectively by removing blood from the body on a regular basis. This is similar to donating blood. The process is known as phlebotomy.
The goal of phlebotomy is to lower your iron levels. The amount of blood removed and how often it’s removed depend on your age, your overall health and the severity of iron overload.”
MAYO CLINIC (https://www.mayoclinic.org/diseases-conditions/hemochromatosis/diagnosis-treatment/drc-20351448)
“Hemochromatosis is a metabolic disorder in which your organs accumulate excess iron, leading to organ damage. Hereditary hemochromatosis affects one in 300 people in the United States.”
John Hopkins Medicine (https://www.hopkinsmedicine.org/health/conditions-and-diseases/hemochromatosis)
Common causes include falls, car or motorcycle crashes, vehicular accidents involving pedestrians, athletics, and assaults with or without a weapon.
Approximately 1.5 to 2 million adults and children suffer a traumatic brain injury (TBI) each year in the United States. Most people who experience a head injury, about 1.1 million, will have a mild injury that does not require an admission to the hospital. Another 235,000 individuals will be hospitalized with a moderate to severe head injury, and approximately 50,000 will die.
When a person is brought to the emergency room with a head injury, doctors will learn as much as possible about his or her symptoms and how the injury occurred. The person’s condition is assessed quickly to determine the extent of injury.
The Glasgow Coma Score (GCS) is a 15-point test used to grade a patient’s level of consciousness. Doctors assess the patient’s ability to 1) open his or her eyes, 2) ability to respond appropriately to orientation questions, (“What is your name? What is the date today?”), and 3) ability to follow commands (“Hold up two fingers, or give a thumbs up”). If unconscious or unable to follow commands, his or her response to painful stimulation is checked. A number is taken from each category and added together to get the total GCS score. The score ranges from 3 to 15 and helps doctors classify an injury as mild, moderate, or severe. Mild TBI has a score of 13-15. Moderate TBI has a score of 9-12, and severe TBI has a score of 8 and below.
Diagnostic imaging tests will be performed:
Figure 3. CT scan shows a blood clot (hematoma) collecting under the bone (red arrows) and displacing brain (yellow arrow) to the other side of the skull.
- Computed Tomography (CT) is a noninvasive X-ray that provides detailed images of anatomical structures within the brain. A CT scan of the head is taken at the time of injury to quickly identify fractures, bleeding in the brain, blood clots (hematomas) and the extent of injury (Fig. 3). CT scans are used throughout recovery to evaluate the evolution of the injury and to help guide decision-making about the patient’s care.
- Magnetic Resonance Imaging (MRI) is a noninvasive test that uses a magnetic field and radiofrequency waves to give a detailed view of the soft tissues of the brain. A dye (contrast agent) may be injected into the patient’s bloodstream. MRI can detect subtle changes in the brain that cannot be seen on a CT scan.
- Magnetic Resonance Spectroscopy (MRS) gives information about the metabolism of the brain. The numbers generated from this scan provide a general prognosis about the patient’s ability to recover from the injury.
Mild TBI usually requires rest and medication to relieve headache. Moderate to severe TBI require intensive care in a hospital. Bleeding and swelling in the brain can become an emergency that requires surgery. However, there are times when a patient does not require surgery and can be safely monitored by nurses and physicians in the neuroscience intensive care unit (NSICU).
The goals of treatment are to resuscitate and support the critically ill patient, minimize secondary brain injury and complications, and facilitate the patient’s transition to a recovery environment. Despite significant research, doctors only have measures to control brain swelling, but do not have a way to eliminate swelling from occurring.
Neurocritical care
Neurocritical care is the intensive care of patients who have suffered a life-threatening brain injury. Many patients with severe TBI are comatose or paralyzed; they also may have suffered injuries in other parts of the body. Their care is overseen by a neurointensivist, a specialty-trained physician who coordinates the patient’s complex neurological and medical care. Patients are monitored and awakened every hour for nursing assessments of their mental status or brain function.
Figure 4. In the NSICU, the patient is connected to numerous machines, tubes, and monitors. The monitoring equipment provides information about body functions and helps guide care. Some equipment may take over certain functions, such as breathing, nutrition, and urination, until the patient’s body is able to do these things on its own.
Seeing a patient who has suffered a severe TBI can be shocking. It is possible that your loved one’s appearance will be altered because of facial injury and equipment that is used for monitoring. Numerous tubes, lines, and equipment may be used to closely monitor his or her heart rate, blood pressure, and other critical body functions. (Fig. 4)
Figure 5. A brain oxygen and cerebral blood flow monitor is inserted into the brain tissue and secured to the skull with a bolt. A catheter is inserted into the ventricle of the brain to monitor intracranial pressure (ICP). If pressure is too high, the CSF fluid can be drained from the ventricles.
- Intracranial pressure (ICP) monitor. A catheter is placed through a small hole in the skull and positioned inside the ventricle (fluid-filled area deep within the brain) to measure pressure inside the head (Fig. 5). The ICP monitor allows the NSICU team to intervene quickly if the pressure becomes too high. Typical intracranial pressure is less than 20 mmHg. However, there are times when a higher number is safe and acceptable.
- Brain oxygen monitor (Licox). A catheter is placed through a small hole in the skull and positioned within the brain tissue. The Licox measures the oxygen level and temperature within the brain. Adjustments in the amount of oxygen given to the patient are often made to maximize the brain’s oxygen level. A cerebral blood flow monitor, called a Hemedex, is a newer monitor that is placed with the Licox and helps the NSICU team evaluate blood flow through the brain.
- Ventilator. Some patients may require a ventilator, a machine that helps them breathe. The ventilator is connected to the patient by the endotracheal tube, or ET tube. The tube is placed into the patient’s mouth and down into the trachea, or windpipe. The tube allows the machine to push air into and out of the lungs, thereby helping the patient breathe.
- Feeding tube. When patients are on a ventilator or have a decreased level of alertness, they may not be able to eat or get sufficient nutrition to meet their needs. A nasal-gastric feeding tube may be inserted through the patient’s nose and passed down the throat into the stomach. It delivers liquid nutrition as well as any medication that is required.
- Seizures and EEG monitoring. A seizure is an abnormal electrical discharge from the brain. Approximately 24% of patients who suffer a TBI will have a seizure that is undetected unless they are monitored by an electroencephalogram (EEG). Seizures that are not visible to the human eye are referred to as non-convulsive seizures. Because these seizures are serious, all patients with a severe TBI are monitored with continuous EEG for 24 to 72 hours after injury.
Medication
- Sedation and pain. After a head injury it may be necessary to keep the patient sedated with medications. These medications can be turned off quickly in order to awaken the patient and check their mental status. Because patients often have other injuries, pain medication is given to keep them comfortable.
- Controlling intracranial pressure. Hypertonic saline is a medication used to control pressure within the brain. It works by drawing the extra water out of the brain cells into the blood vessels and allowing the kidneys to filter it out of the blood.
- Preventing seizures. Patients who’ve had a moderate to severe traumatic brain injury are at higher risk of having seizures during the first week after their injury. Patients are given an anti-seizure medication (levetiracetam or phenytoin) to prevent seizures from occurring.
- Preventing infection. Although every attempt is made to prevent infection, the risk is always present. Any device placed within the patient has the potential to introduce a microbe. If an infection is suspected, a test will be sent to a laboratory for analysis. If an infection is present, it will be treated with antibiotics.
Surgery
Surgery is sometimes necessary to repair skull fractures, repair bleeding vessels, or remove large blood clots (hematomas). It is also performed to relieve extremely high intracranial pressure.
- Craniotomy involves cutting a hole in the skull to remove a bone flap so that the surgeon can access the brain. The surgeon then repairs the damage (e.g., skull fracture, bleeding vessel, remove large blood clots). The bone flap is replaced in its normal position and secured to the skull with plates and screws.
Figure 6. A large decompressive craniectomy is removed and the dura is opened to allow the brain to expand. Blood clots are removed and bleeding vessels are repaired. The bone flap is frozen and replaced about 6 weeks later.
- Decompressive craniectomy involves removing a large section of bone so that the brain can swell and expand. This is typically performed when extremely high intracranial pressure becomes life threatening. At that time the patient is taken to the operating room where a large portion of the skull is removed to give the brain more room to swell (Fig. 6). A special biologic tissue is placed on top of the exposed brain and the skin is closed. The bone flap is stored in a freezer. One to 3 months after the swelling has resolved and the patient has stabilized from the injury, the bone flap is replaced in another surgery, called cranioplasty.
Other surgical procedures may be performed to aid in the patient’s recovery:
- Tracheotomy involves making a small incision in the neck to insert the breathing tube directly into the windpipe. The ventilator will then be connected to this new location on the neck and the old tube is removed from the mouth.
- Percutaneous Endoscopic Gastrostomy Tube (PEG) is a feeding tube inserted directly into the stomach through the abdominal wall. A small camera is placed down the patient’s throat into the stomach to aid with the procedure and to ensure correct placement of the PEG tube (see Surgical Procedures for Accelerated Recovery).
Clinical trials are research studies in which new treatments—drugs, diagnostics, procedures, and other therapies—are tested in people to see if they are safe and effective. Research is always being conducted to improve the standard of medical care. Information about current clinical trials, including eligibility, protocol, and locations, are found on the Web. Studies can be sponsored by the National Institutes of Health (see clinicaltrials.gov) as well as private industry and pharmaceutical companies (see www.centerwatch.com).
The recovery process varies depending on the severity of the injury, but typically progresses through stages: coma, confusion / amnesia, and recovery.
- When a patient is in a coma, his or her eyes are closed and they show minimal reaction when spoken to or stimulated. Movements that may be seen at this time are basic reflexes or automatic responses to a stimulus. The brain wave activity in a comatose person is very different from that of a sleeping person.
- When a patient begins to awaken, the first natural response is that of bodily protection. Patients at this stage will move away from any stimulus or tend to pull at items attached to them in an attempt to remove anything that is uncomfortable or irritating. His or her eyes may be open more often, but they may not be aware of their behavior or be able to interact in a meaningful way. It is common for a patient to respond to each stimulus (hearing, seeing, or touching) in the same way. Responses may include increased rate of breathing, moaning, moving, sweating, or a rise in blood pressure.
- As the patient continues to wake up, their interactions may become more purposeful. They may look at a person and follow them around the room with their eyes, or follow simple commands such as “Hold up your thumb.” Patients tend to be confused and may have inappropriate or agitated behaviors.
Not all head injuries are the same. Patients recover at different rates and to varying degrees. It is difficult to determine at what point a patient will start understanding and interacting with their caregivers or family in a meaningful way. It is important to have patience; recovery from a brain injury can take weeks, months, or even years.
The Family’s Role
Many family members express feelings of helplessness when their loved one is in the NSICU. You are not alone. Please take care of yourself and use your energy wisely.
Visiting hours are limited in the NSICU. Too much stimulation can agitate the patient and raise his or her blood pressure. You can most effectively convey your concern by sitting quietly and holding your loved one’s hand. Be aware that the patient, though silent, may hear anything you say. Never speak as if the patient were not there.
As patients recover, they need help understanding what has happened to them during this “lost period of time.” Keep in mind that the recovery of consciousness is a gradual process – not just a matter of waking up. Progress is usually tracked in three areas: movement, thinking, and interacting. You can help by keeping a diary of their progress. Family photos may help with regaining memory.
Rehabilitation
Most patients are discharged from the hospital when their condition has stabilized and they no longer require intensive care. A social worker will work closely with the family as preparations are made for a return home or for transfer to a long-term care or rehabilitation center.
- A long-term acute care (LTAC) facility is a place for patients who have stabilized from their initial injury but who still require a ventilator or frequent nursing care. Many patients are discharged to an LTAC to continue being weaned from the ventilator. Once off the ventilator, they can be moved to a rehabilitation or skilled nursing facility.
- A rehabilitation facility is a place for patients who do not require a ventilator but who still require help with basic daily activities. Physical and occupational therapists work with patients to help them achieve their maximum potential for recovery. Rehab facilities are either Acute Inpatient Rehab that require patients to participate in 3 hours or more of rehab a day or a Skilled Nursing Facility (SNF) that provide 1-3 hours of rehab a day depending on what the patient can tolerate.
Recovering from a brain injury relies on the brain’s plasticity—the ability for undamaged areas of the brain to take over functions of the damaged areas. It also relies on regeneration and repair of nerve cells. And most importantly, on the patient’s hard work to relearn and compensate for lost abilities.
- A physical therapist helps patients rebuild and maintain strength, balance, and coordination. They can work with the patient in any facility.
- An occupational therapist helps patients to perform activities of daily living, such as dressing, feeding, bathing, toileting, and transferring themselves from one place to another. They also provide adaptive equipment if a patient has difficultly performing a task.
- A speech therapist helps patients by monitoring their ability to safely swallow food and helping with communication and cognition.
- A neuropsychologist helps patients relearn cognitive functions and develop compensation skills to cope with memory, thinking, and emotional needs.
Tips to reduce the risk for a head injury:
- Always wear your helmet when riding a bicycle, motorcycle, skateboard, or all-terrain vehicle.
- Never drive under the influence of alcohol or drugs.
- Always wear your seat belt and ensure that children are secured in the appropriate child safety seats.
- Avoid falls in the home by keeping unsecured items off the floor, installing safety features such as non-slip mats in the bathtub, handrails on stairways, and keeping items off of stairs.
- Avoid falls by exercising to increase strength, balance, and coordination.
- Store firearms in a locked cabinet with bullets in a separate location.
- Wear protective headgear while playing sports.
Reference for Part I and Part II: Mayfield Brain & Spine at 513-221-1100.
“As advances in CF knowledge and care are potentially able to prolong the life expectancy of many patients, it’s important to keep in mind the complications—beyond lung disease—that will develop and progress as patients age.1,9-11 Monitoring for these complications can help detect their emergence and progression, which can ensure earlier intervention; this has been associated with better outcomes in patients.
Early as in utero and into infancy, inflammation may occur, with the possibility of mucus plugging and bronchiectasis.
Inflammation, lung structure and lung function may progress throughout childhood.
Childhood, adolescence and early adulthood what happens is lower airway inflammation and worsening airway abnormalities including established bronchiectasis may occur, driven by the inflammation in the lungs.
In Adulthood and Aging what happens is airway destruction and complications, including bacterial infections, bronchiectasis with hemoptysis, and pneumothorax, may occur and may lead to progressive respiratory failure, often requiring lung transplant.”
CF Source (Multi-Organ Disease Progression in Cystic Fibrosis (CF)
Heart Enlarged