Echo Differentiation of Restrictive Cardiomyopathy and Constrictive Pericarditis

1. Constrictive vs Restrictive
Cardiomyopathy
25 Jan 2017
Koh Choong Hou
Supervisor: A/P Ewe See Hooi

2. Restriction vs Constriction
Stiff Muscle Thick Skin

3. RCMP vs CP
• Stiff Muscle vs Thick Skin
• Both can be present in the same patient
• Commonest etiology: IHD s/p CABG
– Others: infiltrative diseases (amyloidosis,
sarcoidosis), metastatic cancers, radiotherapy
– with PEff and tamponade physiology -> inflow
patterns mimic constriction but pericardium not
thickened/constrictive

5. Constrictive Pericarditis

6. Thick Skin

7. CP Etiologies
Etiologies of CP were compared between the period from 1936 to 1982 and the period from 1985 to 1995. The major
change is that cardiac surgery is now the most common known cause of constriction in developed countries. Ling LH, Oh JK et al.
Constrictive pericarditis in the modern era: evolving clinical spectrum and impact on outcome after pericardiectomy. Circulation 1999;
100:1380-6.

9. Ventricular Interdependence
On inspiration (left), there is a shift of the ventricular septum toward the left ventricle; and on expiration (right), there is a shift of the
ventricular septum toward the right ventricle.

10. 1) Annulus Reversus: TDI parameters of septal vs
annular E’
2) Annulus Paradoxus: E/E’ surrogate of filling
pressures (LAP) -> paradoxically improves with
worsening CP as septal E’ increases viz-a-viz increasing
lateral tethering
Echocardiographic Findings

11. M-mode echocardiogram of a typical CP patient with the ventricular septum moving with
respiration toward the left ventricle with inspiration (upward arrow) and toward the right
ventricle with expiration (downward arrow). A simultaneous respirometric recording is shown
at the bottom of the figure.

12. Characteristic PW Doppler echo findings of CP are shown with mitral inflow velocity (top left), tricuspid inflow velocity (top
right), hepatic vein (bottom left), and SVC (bottom right), with simultaneous respirometric recording. E, DRi, and Si represent E velocity,
diastolic reversal velocity, and systolic velocity, respectively, during inspiration. Ee, DRe, and Se represent E velocity, diastolic reversal
velocity, and systolic velocity, respectively, during expiration.

14. Knowledge Checkpoint
• Doppler findings of CP include the following:
1. Mitral inflow: > 10% variation in mitral E during
respiration
2. Tricuspid inflow: > 40% variation in tricuspid E
during respiration
3. Hep Veins: Diastolic flow reversal more
prominent during inspiration
4. Hep Veins: Increased diastolic forward velocities
with small reversals

15. Knowledge Checkpoint
• Doppler findings of CP include the following:
1. Mitral inflow: > 10% variation in mitral E during
respiration
2. Tricuspid inflow: > 40% variation in tricuspid E
during respiration
3. Hep Veins: Diastolic flow reversal more
prominent during inspiration
4. Hep Veins: Increased diastolic forward velocities
with small reversals

17. Knowledge Checkpoint
• Kussmaul’s sign is:
1. Deep and labored breathing pattern often
associated with severe metabolic acidosis
2. A paradoxical rise in JVP on inspiration, or a failure in
the appropriate fall of the JVP with inspiration
3. Can be seen in both constrictive and restrictive
cardiac disease
4. Is a feature of cardiac tamponade
5. 2 and 3 are correct
6. 1, 2 and 4 are correct

18. Knowledge Checkpoint
• Kussmaul’s sign is:
1. Deep and labored breathing pattern often
associated with severe metabolic acidosis
2. A paradoxical rise in JVP on inspiration, or a failure in
the appropriate fall of the JVP with inspiration
3. Can be seen in both constrictive and restrictive
cardiac disease
4. Is a feature of cardiac tamponade
5. 2 and 3 are correct
6. 1, 2 and 4 are correct

19. The Y descent is prominent, and rather than the normal fall in mean pressure
during inspiration, there is an increase noted. This is referred to as a positive
Kussmaul’s sign and is frequently seen in pericardial and right heart disease

20. Normally left ventricular (LV) diastolic and wedge pressures move in synchrony during
respiration, such that the diastolic gradient from wedge to LV remains constant. In constriction,
negative intrathoracic pressure “pulls” wedge pressure below LV diastolic, with the converse
occurring during expiration. This is termed “intrathoracic-intracardiac dissociation” and is a
sensitive and specific sign of constrictive pericarditis

21. As a result of enhanced ventricular interdependence, the systolic area under the
right ventricular (RV) pressure tracing increases during inspiration while the left
ventricular (LV) systolic area decreases (arrows). The opposite occurs during
expiration.

22. Knowledge Checkpoint
• The following haemodynamic finding(s) is/are
true of constrictive pericarditis:
1. There is prominent Y descent in the right atrial
pressure tracing
2. There is ventricular concordance in the
simultaneous or superimposed LV and RV pressure
tracings
3. There is diastolic equalization of pressures between
the RV and RA waveforms
4. LVEDP and RVEDP pressures are within 15mmHg of
each other

23. Knowledge Checkpoint
• The following haemodynamic finding(s) is/are
true of constrictive pericarditis:
1. There is prominent Y descent in the right atrial
pressure tracing
2. There is ventricular concordance in the
simultaneous or superimposed LV and RV pressure
tracings
3. There is diastolic equalization of pressures
between the RV and RA waveforms
4. LVEDP and RVEDP pressures are within 15mmHg of
each other

24. CP Echo Criteria – ACC 2015 (Jae K Oh)

25. Optimising Echo for CP

27. CP: apical four-chamber view with PW Doppler recording of the mitral inflow in a
patient with CP.
(Left) The respirometer waveform is indistinguishable because of poor gain.
After adjustment (right) the respiratory waves are now able to clearly distinguish
inspiration and expiration
Optimising the Respirometer Waveform

28. CP: 2D parasternal long-axis view demonstrating proper use of a respirometer in a patient with
CP. A high-quality tracing demonstrating both inspiration and expiration is essential.
The left panel and arrow point to upslope in inspiration only .
The right panel and arrows point to the peak of inspiration and expiration after an adjustment
of the respirometer was made
Optimising the Respirometer Waveform

29. CP: 2D parasternal long-axis view in a patient with CP. The arrow points to a focal region
of pericardial calcification extending from the atrioventricular groove to the posterior LV
wall. This finding, in conjunction with an interventricular septal bounce, is consistent with
CP.
Optimising the PLAX Depth

30. CP: M-mode of parasternal short-axis view. Several M-mode features of CP are
seen, including diastolic flattening of the posterior wall, pericardial calcification
(better seen in the right panel with a reduced 2D gain and fundamental imaging),
a septal diastolic shudder consistent with the septal bounce seen by 2D imaging,
and respiratory variation in LV cavity size.

33. CP: apical four-chamber view with PW Doppler of the mitral inflow in a patient with CP.
(Left) The sweep speed is set to 100 mm/sec, and the mitral inflow in inspiration and
expiration cannot be distinguished on the same image.
After adjustment to a sweep speed of 33 mm/sec (usually 25–50 mm/sec is suitable), the
mitral inflow can be clearly distinguished to show onset of in inspiration and expiration
Optimising Inflow Doppler

34. CP: apical four-chamber view with pulsed Doppler of mitral inflow in a patient with CP. The mitral E and
A waves are labeled. The first beat of inspiration is recorded, and peak expiration beat is recorded and
can be used to determine respiratory variation from expiration using the formula (expiration-
inspiration)/expiration. At least three beats should be averaged. Significant respiratory variation is
considered to be present when there is an inspiratory decrease of mitral inflow is >25%.
However, maneuvers (preload reduction as in sitting up or increase in volume loading) may be required
to demonstrate variation.
Calculating Inflow Variation

35. CP: apical 4-chamber view of the tricuspid inflow with PW Doppler in a patient with CP. Measurements of
peak E-wave velocity in inspiration and expiration are made. However, respiratory variation from
expiration is determined by the formula (expiration - inspiration)/expiration or, in this example, (25 -
41)/25 = -76%. A >40% respiratory variation of tricuspid inflow is considered consistent with CP.

36. CP apical four-chamber view with PW Doppler of the right upper pulmonary vein in a
patient with CP. The S (systolic), D (diastolic), and AR (atrial reversal waves) are noted.
Respiratory variation of the D wave should be determined similar to the method used for
the mitral E wave

37. CP: apical four-chamber view with PW Doppler of the mitral inflow in a patient with CP. The
study is performed with the patient in the upright position to decrease preload and
increase respiratory variation.
Marked respiratory variation of mitral inflow E wave is apparent. This maneuver should be
performed if CP is suspected but not readily evident in the basal state.

38. (Left) M-mode demonstrating a dilated and plethoric IVC;
(Right) HV flow with reduced diastolic (D) flow during expiration
and prominent AR reversals during expiration.
IVC and Hep Veins Doppler

39. CP: SVC flow as obtained from the right subclavicular fossa in a patient with CP.
(Left) A 2D image of the SVC.
(Right) S and D waves appearing similar to hepatic vein flow. Note the lack of significant
respiratory variation of systolic flow in CP, which is different from the finding of marked
respiratory variation of systolic flow noted in chronic obstructive pulmonary disease

40. Knowledge Checkpoint
• With regard to optimising echo in the evaluation
of CP,
1. Increase the sweep speed to assess respiratory
variation
2. SVC Doppler is not useful to differentiate between
COPD and CP
3. Respiratory variation is calculated by: [Expiration –
Inspiration] ÷ Inspiration
4. Take the first beat of inspiration and the last beat of
expiration
5. Respiratory variation of the Pulm Vein D wave can
be used

41. Knowledge Checkpoint
• With regard to optimising echo in the evaluation
of CP,
1. Increase the sweep speed to assess respiratory
variation
2. SVC Doppler is not useful to differentiate between
COPD and CP
3. Respiratory variation is calculated by: [Expiration –
Inspiration] ÷ Inspiration
4. Take the first beat of inspiration and the last beat of
expiration
5. Respiratory variation of the Pulm Vein D wave can
be used

43. Restrictive
Cardiomyopathy

44. RCMP
• Definition*:
– On the basis of anatomic, histological and
physiological criteria
• Abnormal LV diastolic filling (physio)
• Intracellular or interstitial infiltration / fibrosis (histo)
• Abnormal LV wall thickness + absence of LV dilatation
(anatomic) <although some RCMP disorders can present
with dilated LV>
– WHO: disease of the myocardium characterized by
restrictive filling and reduced diastolic volume of
either or both ventricles, with normal or near-normal
systolic function
– Essentially -> Diastolic Dysfunction, may be HFpEF
*Constrictive Pericarditis Versus Restrictive Cardiomyopathy? J Am Coll Cardiol
2016;67:2061-2076

45. Stiff Muscle

49. Depressed EF Algorithm

50. CP vs RCMP

51. Hep Vein Doppler
The following correspond to the above HV doppler findings:
1. A: RCMP, B: TR, C: Pulm Hypt, D: CP
2. A: PH, B: RCMP, C: TR, D: CP
3. A: RCMP, B: CP, C: PH, D: TR
4. A: TR, B: CP, C: RCMP, D: PH

52. Hep Vein Doppler
The following correspond to the above HV doppler findings:
1. A: RCMP, B: TR, C: Pulm Hypt, D: CP
2. A: PH, B: RCMP, C: TR, D: CP
3. A: RCMP, B: CP, C: PH, D: TR
4. A: TR, B: CP, C: RCMP, D: PH

54. Algorithm comparing constrictive pericarditis and restrictive cardiomyopathy. Note restriction is associated with elevated
E/A ratio, short DT and decreased mitral annular velocity (<6 cm/sec). The figure is based on data from Welch TD, Ling LH, Espinosa
RE, et al. Echocardiographic diagnosis of constrictive pericarditis: Mayo Clinic criteria. Circ Cardiovasc Imaging 2014;7:526–34.

55. References
• American Society of Echocardiography Clinical
Recommendations for Multimodality Cardiovascular
Imaging of Patients with Pericardial Disease J Am Soc
Echocardiogr 2013;26:965-1012
• Recommendations for the Evaluation of Left
Ventricular Diastolic Function by Echocardiography J
Am Soc Echocardiogr 2016;29:277-314
• ACC SAP 9 Chapters on Heart Failure / Pericardial
Disease
• Constrictive Pericarditis Versus Restrictive
Cardiomyopathy? J Am Coll Cardiol 2016;67:2061-2076

56. THE END