Restrictive Cardiomyopathy
Cardiology (12%) Core Clinical Conditions
2B: The Physician Associate is able to undertake the day to day management of the patient and condition once the diagnosis and strategic management decisions have been made by another.
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Definition Aetiology Pathophysiology Risk factor Sign and Symptoms Investigations Management
Definition
Restrictive cardiomyopathy is a type of heart disease that impairs the heart muscle's capacity to relax and fill with blood between heartbeats. This reduces the amount of blood the heart is able to pump to the rest of the body. It is referred to as "restrictive" because the heart muscle stiffens and becomes less flexible, limiting its ability to fill with blood.
Aetiology
Genetics: Inherited genetic mutations.
Amyloidosis: a disease that causes abnormal protein buildup in the heart.
Sarcoidosis: a disease that causes scarring or fibrosis of the heart muscle.
Chemotherapy or radiation therapy.
Hemochromatosis: where excess iron builds up in the heart muscle.
Idiopathic.
Pathophysiology
In restrictive cardiomyopathy, the heart muscle stiffens and becomes less flexible, impairing its capacity to relax and fill with blood between heartbeats. The stiffening of the heart muscle can be caused by abnormal protein accumulation, scarring, or abnormal cell infiltration.
As the heart muscle stiffens, it cannot fill with blood as efficiently during diastole, resulting in a decrease in cardiac output.
There is also an elevated intracardiac pressure, particularly in the atria. This may result in the development of atrial fibrillation (a heart rhythm disorder) and congestive heart failure.
Risk factors
Family history.
Age: over 60s are more susceptible.
Some medical conditions: sarcoidosis, amyloidosis, and hemochromatosis.
Chemo and Radiation therapy.
Alcohol abuse.
Obesity.
Idiopathic.
Sign and symptoms
Shortness of breath with exertion or at rest
Fatigue and weakness
Swelling in the legs, ankles, and feet
Abdominal swelling
Tachycardia or irregular heartbeat (arrhythmia)
Chest pain
Dizziness
Syncope
Reduced exercise tolerance
Investigations
Echocardiogram
Electrocardiogram (ECG)
Cardiac MRI: it may display scarring of the heart muscle.
Blood tests: include kidney function, UE, BNP and LFTs
Ventricular Biopsy (gold standard)
Management
Heart transplantation is the treatment of choice for children.
Medication: to manage symptoms such as shortness of breath and edoema, medications such as diuretics, beta-blockers, and calcium channel blockers may be used.
Surgery: heart valve repair or replacement, or heart transplantation, may be necessary.
Lifestyle modifications: such as reducing alcohol consumption, losing weight, and avoiding activities that may exacerbate symptoms (such as vigorous exercise), can help manage symptoms and slow the progression of the disease.
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