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Restrictive Cardiomyopathy - The Heart that just doesn’t want to give in

So far, we’ve seen a large, flabby heart that doesn’t know how to fill itself, and another large muscular heart that didn’t know when to stop hitting the gym. Next, we’ll take a look at a heart that just doesn’t want to be compliant…

In Restrictive Cardiomyopathy (RCM), the compliance of the myocardium is drastically reduced usually due to fibrosis. It can often be confused with HCM or even pericarditis. Now why does this occur?

Amyloid deposition, leading to restriction of the myocardium is one cause. Loeffler’s syndrome, where there is an eosinophillic infiltrate in several organs is another cause. Here, eosinophils release major basic protein, which causes necrosis and…

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 Restrictive Cardiomyopathy - Stiff Ventricles

Most patients present as congestive failure and right-sided failure dominates the clinical picture.

Articles of Interest

Cardiac Amyloidosis

Cardiac amyloidosis is one of the leading causes of restrictive cardiomyopathy. It typically presents with rapidly progressive diastolic dysfunction in a non-dilated ventricle. It is one of the under-diagnosed disease entities. The diagnosis of cardiac amyloidosis requires a high degree of suspicion, with cardiovascular imaging being pivotal in reaching the diagnosis.

My Approach to the Evaluation of Restrictive Cardiomyopathy

Restrictive cardiomyopathy (RCM) was defined as a myocardial disease of unknown origin based on the WHO classification of 1980... RCM should not be considered a futile diagnosis.

Overview of Restrictive Cardiomyopathies

Restrictive cardiomyopathy (RCM) includes a heterogeneous group of diseases that cause increased myocardial stiffness, leading to impaired ventricular relaxation and severe diastolic dysfunction. Given that it is the least common type of cardiomyopathy, it can be a diagnostic challenge due to its varied pathogenesis, clinical presentation, and diagnostic evaluation.

Restrictive Cardiomyopathy: An overview

Restrictive cardiomyopathy is one of the least common forms of cardiomyopathy and accounts for almost 5% of the cases for cardiomyopathy registered worldwide.

Spectrum of Restrictive and Infiltrative Cardiomyopathies: Part 1

Restrictive cardiomyopathies are the least common form of heart muscle disease. They are characterized as infiltrative and noninfiltrative, storage diseases, and endomyocardial disorders. Genetic diseases commonly present during childhood or adolescence. However, a growing percentage of elderly patients with heart failure with preserved ejection fraction are being recognized as having forms of restrictive cardiomyopathy, particularly cardiac amyloidosis.

Resources

Children's Cardiomyopathy Foundation

Restrictive cardiomyopathy (RCM) is a less common type of cardiomyopathy; only 3 to 5 percent of children diagnosed with cardiomyopathy have RCM. According to the Pediatric Cardiomyopathy Registry, the average age of diagnosis is 5 to 6 years. Some studies suggest that RCM may be slightly more common in girls than boys.

Definition and Diagnosis of Restrictive Cardiomyopathy: Key Points

Diagnostic workup for etiology of RCM includes the electrocardiogram (low voltage for infiltrative cardiomyopathies) and an echocardiogram. Cardiac magnetic resonance imaging should be performed when possible with consideration for genetic testing.

Radiopaedia

It is predominantly a disease of diastolic dysfunction where the systolic (contractile) of the myocardium is usually unaffected.

Restrictive Cardiomyopathies: The Importance of Noninvasive Cardiac Imaging Modalities in Diagnosis and Treatment—A Systematic Review

Restrictive cardiomyopathy (RCM) is the least common among cardiomyopathies. It can be idiopathic, familial, or secondary to systematic disorders. Marked increase in left and/or right ventricular filling pressures causes symptoms and signs of congestive heart failure. Electrocardiographic findings are nonspecific and include atrioventricular conduction and QRS complex abnormalities and supraventricular and ventricular arrhythmias.

Restrictive Cardiomyopathy

Restrictive cardiomyopathy is the least common form of cardiomyopathy. It occurs in the advanced stages of myocardial infiltrative disease — e.g. due to haemochromatosis, amyloidosis or sarcoidosis.

StatPearls

Restrictive cardiomyopathy (RCM) is characterized by diastolic dysfunction of a non-dilated ventricle. Multiple types of restrictive cardiomyopathies exist and vary in their pathogenesis, clinical presentation, diagnostic evaluation, treatment, and prognosis. Three of the leading causes of RCM are cardiac amyloidosis, cardiac sarcoidosis, and cardiac hemochromatosis.