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Published on: August 8, 2022
Restrictive cardiomyopathies
Petros Nihoyannopoulos1, David Dawson
1Cardiology Department, NHLI, Hammersmith Hospital, Imperial College London, London W12 0NN, UK. petros@imperial.ac.uk
Insights
Restrictive cardiomyopathies are diverse heart muscle diseases causing heart failure. Echocardiography is key for diagnosis, often revealing diastolic dysfunction, and identifying underlying systemic causes is crucial.
Area of Science:
- Cardiology
- Internal Medicine
- Pathology
Background:
- Restrictive cardiomyopathies (RCM) are a heterogeneous group of heart muscle diseases characterized by impaired ventricular filling.
- Diastolic dysfunction with preserved systolic function is a common echocardiographic finding, though systolic dysfunction can occur in advanced stages.
Purpose of the Study:
- To summarize the heterogeneous nature of restrictive cardiomyopathies.
- To highlight diagnostic approaches and the importance of identifying underlying systemic causes.
Main Methods:
- Review of medical history and physical examination findings.
- Utilizing diagnostic tests including blood tests, electrocardiogram, chest X-ray, echocardiography, and magnetic resonance imaging.
- Consideration of endomyocardial biopsies and post-mortem examination for idiopathic cases.
Main Results:
- The majority of RCM cases are secondary to systemic disorders like amyloidosis, sarcoidosis, or radiation.
- Echocardiography is a primary tool for identifying left ventricular dysfunction.
- Differential diagnosis from constrictive pericarditis may be necessary.
Conclusions:
- Restrictive cardiomyopathy diagnosis relies on a combination of clinical evaluation and imaging.
- Early and expert echocardiography is vital for identifying RCM and guiding etiological investigation.
- Distinguishing RCM from other conditions, like constrictive pericarditis, is essential for appropriate management.
Abstract:
Restrictive cardiomyopathies constitute a heterogenous group of heart muscle conditions that all have, in common, the symptoms of heart failure. Diastolic dysfunction with preserved systolic function is often the only echocardiographic abnormality that may be noted, although systolic dysfunction may also be an integral part of some specific pathologies, particularly in the most advanced cases such as amyloid infiltration of the heart. By far, the majority of restrictive cardiomyopathies are secondary to a systemic disorder such as amyloidosis, sarcoidosis, scleroderma, haemochromatosis, eosinophilic heart disease, or as a result of radiation treatment. The much more rare diagnosis of idiopathic restrictive cardiomyopathy is supported only by the absence of specific pathology on either endomyocardial biopsies or at post-mortem. Restrictive cardiomyopathy is diagnosed based on medical history, physical examination, and tests: such as blood tests, electrocardiogram, chest X-ray, echocardiography, and magnetic resonance imaging. With its wide availability, echocardiography is probably the most important investigation to identify the left ventricular dysfunction and should be performed early and by groups that are familiar with the wide variety of aetiologies. Finally, on rare occasions, the differential diagnosis from constrictive pericarditis may be necessary.
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