Restrictive Cardiomyopathy (RCM) is a rare form of heart disease that is caused either by inherited genetic disorders or by other diseases such as amyloidosis, sarcoidosis, metabolic disorders or endocrine diseases and, more rarely, by liver disease or pulmonary hypertension.
Restrictive Cardiomyopathy is characterized by increased thickening and hardening of the walls of the heart, especially the ventricles, without concomitant significant hypertrophy (enlargement) of the cardiomyocytes. This stiffness of the heart and its difficulty in receiving the normal amount of blood returning from the body and lungs causes a slowdown in the heart’s working cycle, resulting in poor blood supply to the organs and tissues. Its burden causes heart failure.
The symptoms of Restrictive Cardiomyopathy are common with other cardiomyopathies (Dyspnea, fatigue, edema (swelling) of the legs, ankles or abdomen, tachycardia, chest pain, feeling of weakness). The recognition and diagnosis of this specific condition requires a Cardiologist with knowledge and experience in cardiomyopathies.
The diagnosis of the condition in the Clinic includes: Medical hereditary history, cardiogram, echocardiogram and referral for special hematological genetic tests to identify hereditary causes, if a genetic predisposition is suspected.
From the data that will emerge, if restrictive cardiomyopathy is diagnosed, a cardiac MRI will be performed for a more accurate visualization of the structure and functional characteristics of the heart and cardiac angiography or cardiac catheterization to assess the pressures in the ventricles and evaluate the circulatory systems.
Restrictive Cardiomyopathy is incurable in the sense that there is no way to treat the hardening of the cardiomyocyte tissue. However, because it is usually progressive, the specialized Cardiologist contributes with complete medication to relieve symptoms and improve cardiorespiratory function. Regular monitoring by the Cardiologist and early diagnosis are important for the good management of the condition and the support of the patient. At the same time, the function of other organs is monitored and blood pressure or metabolism is regulated where necessary.
If there is a risk of life-threatening arrhythmias, a defibrillator is placed in the patient.
