Constrictive vs Restrictive Cardiomyopathy

25 Jan 2017Koh Choong Hou

Supervisor: A/P Ewe See Hooi

Restriction vs Constriction

Stiff Muscle Thick Skin

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

Constrictive Pericarditis

Thick Skin

CP Etiologies

Etiologies of CP were compared between the period from 1936 to 1982 and the period from 1985 to 1995. The majorchange 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.

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.

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

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.

Characteristic PW Doppler echo findings of CP are shown with mitral inflow velocity (top left), tricuspid inflow velocity (topright), 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 reversalvelocity, and systolic velocity, respectively, during expiration.

Knowledge Checkpoint

• Doppler findings of CP include the following:1. Mitral inflow: > 10% variation in mitral E during

respiration2. Tricuspid inflow: > 40% variation in tricuspid E

during respiration3. Hep Veins: Diastolic flow reversal more

prominent during inspiration4. Hep Veins: Increased diastolic forward velocities

with small reversals

Knowledge Checkpoint

• Doppler findings of CP include the following:1. Mitral inflow: > 10% variation in mitral E during

respiration2. Tricuspid inflow: > 40% variation in tricuspid E

during respiration3. Hep Veins: Diastolic flow reversal more

prominent during inspiration4. Hep Veins: Increased diastolic forward velocities

with small reversals

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 tamponade5. 2 and 3 are correct6. 1, 2 and 4 are correct

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 tamponade5. 2 and 3 are correct6. 1, 2 and 4 are correct

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

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

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.

Knowledge Checkpoint

• The following haemodynamic finding(s) is/are true of constrictive pericarditis:1. There is prominent Y descent in the right atrial

pressure tracing2. There is ventricular concordance in the simultaneous

or superimposed LV and RV pressure tracings3. There is diastolic equalization of pressures between

the RV and RA waveforms4. LVEDP and RVEDP pressures are within 15mmHg of

each other

Knowledge Checkpoint

• The following haemodynamic finding(s) is/are true of constrictive pericarditis:1. There is prominent Y descent in the right atrial

pressure tracing2. There is ventricular concordance in the simultaneous

or superimposed LV and RV pressure tracings3. There is diastolic equalization of pressures between

the RV and RA waveforms4. LVEDP and RVEDP pressures are within 15mmHg of

each other

CP Echo Criteria – ACC 2015 (Jae K Oh)

Optimising Echo for CP

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 distinguishinspiration and expiration

Optimising the Respirometer Waveform

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

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

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.

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

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

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.

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

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.

(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

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

Knowledge Checkpoint

• With regard to optimising echo in the evaluation of CP,1. Increase the sweep speed to assess respiratory variation2. SVC Doppler is not useful to differentiate between COPD

and CP3. Respiratory variation is calculated by: [Expiration –

Inspiration] ÷ Inspiration 4. Take the first beat of inspiration and the last beat of

expiration5. Respiratory variation of the Pulm Vein D wave can be

used

Knowledge Checkpoint

• With regard to optimising echo in the evaluation of CP,1. Increase the sweep speed to assess respiratory variation2. SVC Doppler is not useful to differentiate between COPD

and CP3. Respiratory variation is calculated by: [Expiration –

Inspiration] ÷ Inspiration 4. Take the first beat of inspiration and the last beat of

expiration5. Respiratory variation of the Pulm Vein D wave can be

used

Restrictive Cardiomyopathy

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

Stiff Muscle

Depressed EF Algorithm

CP vs RCMP

Hep Vein Doppler

The following correspond to the above HV doppler findings:1. A: RCMP, B: TR, C: Pulm Hypt, D: CP2. A: PH, B: RCMP, C: TR, D: CP3. A: RCMP, B: CP, C: PH, D: TR4. A: TR, B: CP, C: RCMP, D: PH

Hep Vein Doppler

The following correspond to the above HV doppler findings:1. A: RCMP, B: TR, C: Pulm Hypt, D: CP2. A: PH, B: RCMP, C: TR, D: CP3. A: RCMP, B: CP, C: PH, D: TR4. A: TR, B: CP, C: RCMP, D: PH

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

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

THE END