Cardiac amyloidosis, also known as rigid heart syndrome, is a rare, very serious disease that affects the heart muscle due to the accumulation of proteins called amyloids in the cardiac walls.
This disease is more common in men over 40 years of age and causes signs of heart failure, such as easy tiredness and difficulty climbing stairs or making small efforts.
The accumulation of proteins can occur only in the atrial septum, as is more common in the elderly, or in the ventricles, which can cause heart failure.
Symptoms
Symptoms of cardiac amyloidosis can be:
- Intense urge to urinate at night; Enlargement of the neck veins, scientifically called jugular stasis; Heart palpitations; Accumulation of fluid in the lungs; Enlarged liver; Low pressure when rising from a chair, for example; Fatigue; Persistent dry cough; Weight loss without apparent cause, without diet or increased exercise; Intolerance to physical efforts; Fainting; Shortness of breath; Swollen legs; Swollen abdomen.
Amyloidosis in the heart is characterized by excess protein in the heart muscle and can be caused by multiple myeloma, be of family origin or can arise with advancing age.
How to know if it is cardiac amyloidosis
Normally, this disease is not suspected in a first visit and, therefore, it is common for doctors to order several tests to screen for other diseases before reaching the diagnosis of cardiac amyloidosis.
The diagnosis is made through observation of symptoms and through tests requested by the cardiologist, such as electrocardiogram, echocardiogram and magnetic resonance imaging, which may detect cardiac arrhythmias, changes in heart functions and disturbances in the electrical conduction of the heart, but the diagnosis of cardiac amyloidosis it can only be proven through a biopsy of the cardiac tissue.
This diagnosis can be reached when the ventricular wall thickness is above 12 mm and when the person does not have high blood pressure, but has one of the following characteristics: dilation of the atria, pericardial effusion or heart failure.
Treatment
For treatment, diuretic and vasodilator remedies can be used to relieve the symptoms of the disease. The use of pacemakers and automatic defibrillators can be used as alternatives to control the disease and in the most severe cases, the most suitable treatment is heart transplantation. See the risks and how is the recovery from heart transplantation by clicking here.
Depending on the severity of the disease, anticoagulants that prevent the formation of blood clots in the heart can be used, decreasing the likelihood of strokes. Chemotherapy can be used when the cause of cardiac amyloidosis is cancer of the multiple myeloma type
The person should avoid salt, prefer diuretic foods and avoid making efforts to save the heart. The family should also avoid giving bad news because intense emotions can lead to major cardiac changes that can lead to a heart attack.
See all types and symptoms caused by Amyloidosis.
Possible Complications of Cardiac Amyloidosis
Complications related to cardiac amyloidosis involve severe cardiac changes such as cardiac conduction disorder, restrictive cardiomyopathy, low cardiac output and isolated atrial impairments.
Cardiac amyloidosis is a progressive disease that has a very poor prognosis. Familial cardiac amyloidosis has a more reserved prognosis and can lead to sudden death. The senile form, which develops with advancing age, may result in severe restrictive cardiomyopathy, with a high risk of death or may result only in isolated atrial involvement, leading to arrhythmia such as atrial fibrillation.
