5. • Restrictive cardiomyopathy is characterized by a nondilated (or small, end-
diastolic volume index <40 mL/m2) left ventricle (LV) with abnormal
ventricular diastolic function
• Hypertrophy is typically absent, although infiltrative disease (such as
amyloidosis) and lysosomal storage diseases (such as Fabry disease) may
cause an increase in LV wall thickness
• Systolic function usually remains normal, at least early in the disease. This
entity is a subset of what has been referred to as heart failure with
preserved ejection fraction (HFpEF)
• Causes of restrictive cardiomyopathy include infiltrative disease, storage
disease, and endomyocardial processes
6. • Restrictive cardiomyopathies are more difficult to diagnose with
echocardiography than dilated or hypertrophic cardiomyopathies, and
may be challenging to distinguish from constrictive pericarditis.
7. • Restrictive cardiomyopathy is characterized by a
• low or normal diastolic volume,
• normal or only mildly reduced LV ejection fraction, atrial
enlargement,
• normal pericardium
• abnormal diastolic function.
• Diastolic dysfunction is frequently restrictive, with an elevated peak
mitral inflow velocity
• rapid early mitral inflow deceleration, and reduced Doppler tissue
imaging early annular velocity.
8. • Echocardiographic findings may help differentiate between restrictive
cardiomyopathy and pericardial constriction among individuals with
HF symptoms and normal LV ejection fraction.
9. Diabetes mellitus
• most common restrictive cardiomyopathy is the small, stiff heart of
diabetes in which diastolic dysfunction is the predominant functional
abnormality
• In the majority of diabetics, this condition is clinically unapparent
• Quantitation of LV function reveals a normal ejection fraction and LV
volumes that are lower than expected.
• This condition is suspected as a predisposing factor when, for
example, diabetics with critical coronary artery stenosis and normal
LV systolic function develop pulmonary congestion in association with
angina or acute myocardial infarction.
10. • Amyloid cardiomyopathy — Amyloid cardiomyopathy can present as
a restrictive cardiomyopathy with increased wall thickness that may
be confused with hypertrophic cardiomyopathy (HCM).
• Diastolic dysfunction is the most common, earliest, and most
important echocardiographic abnormality in cardiac amyloidosis
11. • Echocardiography in patients with overt cardiac amyloidosis
frequently demonstrates symmetric LV wall thickening, typically
involving the interventricular septum, small ventricular chambers,
thickening of the atrial septum, pericardial effusion, and dilated
• Increased right ventricular (RV) wall thickness, when present, may be
associated with RV diastolic dysfunction, which can be demonstrated
by Doppler examination
• Disproportionate RV enlargement may also occur
12.
13. Thickened walls of left ventricle with intensely
reflective myocardium and small LV cavity
14. • Another common echocardiographic finding in cardiac amyloidosis is
a granular, "speckled" appearance of the myocardium, resulting
from the presence of amyloid and collagen nodules in the heart
• This finding alone is relatively nonspecific but the combination of
these refractile echoes, atrial septum thickening, and low ECG voltage
are highly suggestive of cardiac amyloid
15. • Apparent preservation or exaggeration of contractile function in a subgroup
of patients with amyloid cardiomyopathy is explained by very low wall
stress, which is the ultimate expression of afterload and is greatly reduced
by the combination of thickened walls, very small cavity, and low generated
systolic pressure (arterial hypotension).
• When contractile function is preserved, amyloid cardiomyopathy can be
mistaken for HCM, and diagnosis can be delayed.
• A patient with low blood pressure, low ECG voltage, valvular thickening,
pericardial and pleural effusions, and the above features should
immediately arouse suspicion of the advanced stage of this form of
infiltrative cardiomyopathy.
• Early disease may be difficult to differentiate from other forms of cardiac
disease and may only be manifest upon serial echocardiograms.
16. Concentric left ventricular hypertrophy with
reduced lv cavity size
sparkling or ground glassing of myocardium
non collapsible IVC due RV failure
21. Strain echocardiography
• Strain echocardiography may be helpful in diagnosing amyloid
cardiomyopathy.
• A systolic septal longitudinal base-to-apex strain ratio >2.1 has an 88
percent sensitivity and 85 percent specificity in differentiating
amyloid cardiomyopathy from other causes of LV hypertrophy
• Polar maps (so-called "bulls-eye" maps or apical sparing pattern)
facilitate recognition of this unique pattern.
• Relative regional strain ratio with apical sparing has both diagnostic
and prognostic implications in patients with cardiac amyloidosis
22. Endomyocardial fibrosis
• Endomyocardial fibrosis is a cause of restrictive cardiomyopathy in
North Africa and South America.
• The condition is associated with eosinophilia in approximately 50
percent of those afflicted, and may overlap with eosinophilic
endocarditis also known as Loeffler's or Davies disease when
encountered in North Africa.
23. Characteristic echo findings
• There are mass-like apical lesions in the LV, resulting from a
thrombotic fibrocalcific process.
• These lesions are associated with restriction of LV and RV filling due
to obliteration of one or both cardiac
• Mitral and tricuspid valve leaflet tethering may occur, resulting in
regurgitation.
• In addition to the unique appearance of the apices, the atria are
strikingly enlarged.
• As the condition progresses, more and more of the LV cavity is
obliterated, leading to a progressively restrictive physiology
25. EMF should be differentiated from HCM
• Endomyocardial fibrosis can be differentiated from apical HCM by
left-sided echo contrast, which demonstrates obliteration of the
apical cavity in the former and apical hypertrophy in the latter.
26. ARTHYMOGENIC RIGHT VENTRICULAR
CARDIOMYOPATHY
• arhythmogenic right ventricular cardiomyopathy (ARVC), also known
as arrhythmogenic right ventricular dysplasia, is a category of
cardiomyopathy characterized by fibrous or fibro-fatty replacement
of myocardium in the inflow tract, outflow tract, and/or apex of the
RV
• Echocardiography is one of several modalities utilized to aid in the
diagnosis of ARVC although sensitivity of echocardiography may be
limited
27. • In a study using 2D echocardiography, TAPSE and RV fractional area
change predicted adverse outcomes
• A study using 3D echocardiography with Doppler tissue imaging
demonstrated that patients with ARVC had lower RV ejection fraction,
decreased lateral RV and LV systolic annular velocity, and decreased
regional systolic strain compared with age-matched controls
• In a study that included genotypically abnormal (desmosomal gene)
relatives of ARVC probands (without phenotypic abnormality) and
controls, reduced global and regional strain was present in patients
with ARVC mutation but not in controls
28. • UNCLASSIFIED CARDIOMYOPATHYLeft ventricular (LV)
noncompaction and stress (takotsubo) cardiomyopathy lie in this
category.
• Stress cardiomyopathy is an often reversible form of cardiomyopathy
that may present with slight troponin elevation and
electrocardiogram abnormalities.
• Apical ballooning and/or midventricular hypokinesis is usually seen.
29. Left ventricular noncompaction
• Isolated LV noncompaction is a cardiomyopathy characterized by
prominent trabeculae and deep intertrabecular recesses, with a
noncompacted to compacted myocardial ratio of at least 2:1.
• LV morphologic criteria for noncompaction should be applied with
caution since the specificity of imaging criteria may be low in some
populations such as athletes.
• Left-sided contrast with a proper mechanical index can be used to
delineate the endocardial border and improve the diagnosis of LV
noncompaction
30. • LVNC is characterized by the following features:
â—ŹAn altered myocardial wall with prominent trabeculae and deep
intertrabecular recesses, resulting in thickened myocardium with two
layers consisting of noncompacted myocardium and a thin compacted
layer of myocardium
â—ŹContinuity between the LV cavity and the deep intertrabecular
recesses, which are filled with blood from the ventricular cavity without
evidence of communication to the epicardial coronary artery system.
31.
32. LEFT VENTRICULAR NON COMPACTION
• For diagnosis of LVNC using echocardiography, we use the Jenni
criteria, which are the most widely accepted validated criteria.
Alternatively, some clinicians use the Chin or Stöllberger criteria,
which have also been validated.
33. JENNI CRITERIA
• The criteria are assessed in the parasternal short-axis view at base, mid, and
apical levels.
• The presence of all four of the following echocardiographic criteria are required
for diagnosis:
âť–A thickened LV wall consisting of two layers: a thin compacted epicardial layer
and a markedly thickened endocardial layer with numerous prominent
trabeculations and deep recesses with a maximum ratio of noncompacted to
compacted myocardium >2:1 at end-systole in the parasternal short-axis view
âť–Color Doppler evidence of flow within the deep intertrabecular recesses.
âť–Prominent trabecular meshwork in the LV apex or midventricular segments of
the inferior and lateral wall.
❖Compacted wall thickness ≤8.1 mm. The criterion of maximal systolic compacta
thickness of ≤8.1 mm was found to be very specific for myocardial thickening in
LVNC compared to normal controls or patients with aortic stenosis
34. STOLLBERGER CRITERIA FOR
HYPERTRABECULATION
• Stöllberger criteria emphasize hypertrabeculation
• •More than three trabeculations protruding from the LV wall, apically
to the papillary muscles, visible in a single image plane.
• •Intertrabecular spaces perfused from the ventricular cavity,
visualized on color Doppler imaging.
