- Symptoms and signs of heart failure with predominant right-sided findings.
- Normal left and right ventricular size and systolic function with dilated atria.
- Diastolic ventricular functional abnormalities suggestive
of reduced ventricular compliance.
- Increased ventricular filling pressure (left > right) and
reduced cardiac output.
Definitions and Terminology
The World Health Organization defines cardiomyopathies as heart muscle diseases of unknown cause; restrictive cardiomyopathy is
one of three forms (the others are dilated cardiomyopathy and hypertrophic
cardiomyopathy). Restrictive cardiomyopathies are classified as primary
(endocardial fibrosis and eosinophilic endomyocardial disease) or
secondary to cardiac infiltrative diseases. Diseases with a defined
cause that produce a dilated cardiomyopathy with restrictive characteristics
are specifically excluded from this classification of cardiomyopathies.
For the clinician, restrictive cardiomyopathy is usually due to infiltrative diseases, such as the cardiomyopathy that occurs in
systemic amyloidosis, hemochromatosis, sarcoidosis, and glycogen
storage diseases (Table 15–1). These
secondary cardiomyopathies are included because the cardiac involvement
typically displays features of restrictive physiology that are pivotal
in the diagnosis. Restrictive cardiomyopathies represent less than
1% of cases of congestive heart failure, and most of these
are of the secondary form.
Table 15–1. Classification of Restrictive Cardiomyopathy. |Favorite Table|Download (.pdf)
Table 15–1. Classification of Restrictive Cardiomyopathy.
|Familial ± skeletal myopathy||Amyloidosis|
|Löffler cardiomyopathy (endocarditis parientalis fibroplastica)|
Glycogen storage diseases
|Tropical endomyocardial fibrosis||Sarcoidosis|
|Chronic allograft rejection|
Also known as diastolic dysfunction and diastolic heart failure, restrictive physiology requires a precise definition, even though clinical methods often yield only indirect evidence of this functional abnormality. It is characterized by elevated filling pressures and impaired ventricular filling from myocardial or endocardial abnormalities in a nondilated ventricle with no significant impairment of systolic performance. Numerous common cardiac diseases can produce
the functional abnormalities of the restrictive or diastolic type, but the diseases are not classified as a restrictive cardiomyopathy.
An alternative term applied to many of these diseases, infiltrative cardiomyopathy emphasizes that the endocardial, interstitial, or
intracellular infiltration of a variety of materials (eg, extensive
collagen bundles, amyloid protein) is the central histologic and
pathophysiologic feature. The infiltration of material with tissue
less compliant than normal myocardium and the increase in the wall
thickness of the cardiac chambers reduce chamber compliance.
This term is sometimes used to describe the reduction in left ventricular chamber volume as the consequence of endocardial fibrosis
and extensive mural thrombus formation.
Abnormalities in Diastolic Function
Although there are several causes of abnormal diastolic function in restrictive cardiomyopathy, loss of ventricular chamber compliance
is the most common. Fibrosis and the presence of amyloid and hemosiderin
all change ...