hemodyanmic features of constrictive pericarditis and restrictive cardiomyopathy, echocardiographic findings

1. By: Dr. Himanshu Rana
Chair: Prof. Dr. Prabha Nini Gupta

2. 
 Distinction of constrictive and restrictive hemodynamics remains one of
cardiovascular medicine’s most complex challenges.
 Both result in impaired ventricular filling with clinical manifestations
of predominantly right heart failure with preserved ejection fraction.
 Constrictive pericarditis (CP) is a potentially reversible cause of heart
failure, whereas restrictive cardiomyopathy (RCM) has very limited
therapeutic options.
Introduction

3.  CP - “pathological condition with encasement of the heart by a
thickened, fibrous, & sometimes calcified pericardium, with secondary
abnormalities in chamber filling”.
 Male predominance in most clinical series
 Though the prognosis is dependent upon the underlying etiology,
complete surgical removal of the pericardium can result in excellent
symptomatic improvement.
Introduction contd.

4. 
 RCM - “increased myocardial stiffness, which results in a rapid rise in
ventricular filling pressures reflected in both the systemic and
pulmonary circulations”.
 Despite marked abnormalities in diastolic function, left ventricular (LV)
ejection fraction is typically preserved
 Unfortunately, therapeutic approaches to RCM remain challenging.
Despite optimal heart failure care, definitive treatment is often limited to
cardiac transplantation
Introduction contd.

5. 
 Considerable overlap of CP and RCM may be present, particularly in the
setting of prior chest radiotherapy
 Mixed constrictive and restrictive hemodynamics pose a significant
management dilemma, because the clinical outcome of high-risk surgical
interventions may be uncertain
Introduction contd.

6. 
Normal cardiac
hemodynamics

7.  Although frequently separated during discussion, diastole & systole are
closely linked, with preload provided by diastolic filling necessary for
generating stroke volume via ‘Frank-Starling mechanism’
 Diastolic filling depends upon factors
 extrinsic to cardiac chamber
 the loading conditions imposed upon the heart,
 pericardial restraint,
 chest geometry
 intrinsic myocardial properties, such as
 viscoelastic forces,
 myocardial stiffness, &
 stress–strain relationships
Normal cardiac hemodynamics

8. 
 Complex sequence of interrelated events, & can be divided
into 3 components:
 ventricular relaxation,
 passive filling,
 and atrial contraction
Ventricular diastole

9.  Early rapid filling occurs due to a combination of
 ventricular relaxation,
 the driving pressure across the mitral valve from elevated LA pressure,
 pericardial restraint, and
 myocardial stiffness.
 Passive filling occurs as the result of
 continued ventricular relaxation and
 effective operating chamber compliance, and
 Atrial contraction serves to “prime” the ventricle by actively distending
the chamber via atrial mechanical emptying
Phases of ventricular diastole
which is sum total of passive filling
dependent upon pericardial
restraint, ventricular interaction
viscoelastic forces of myocardium.

10.  Pericardium encompasses both ventricles, RA and most of LA
 Most of the SVC & IVC are not intrathoracic, and thus are largely
unaffected by swings in intrathoracic pressure
 During inspiration, diaphragmatic descent results in a decrease in
intrathoracic pressure of 5 to 10 mm Hg, which is fully transmitted to the
cardiac chambers
 Given no change in systemic venous pressure, the drop in intrathoracic
pressure augments right heart filling
Flow hemodynamics

11.  The pulmonary veins are entirely intrathoracic; therefore, there is a
uniform decrease in pressure within the pulmonary veins and left-sided
cardiac chambers
 Thus, left-sided filling does not significantly alter during respiration
 During expiration, right-sided filling decreases relative to inspiration,
whereas left-heart filling remains relatively constant.
Flow hemodynamics

12. 
Hemodynamics of
constriction

13. 
 The primary hemodynamic consequence of constriction is –
 limitation of the total volume of blood that can be accommodated by the heart
during diastole across the respiratory cycle, &
 equalization of right- and left sided cardiac filling pressures.
Hemodynamics of constriction

14.  Accentuated early rapid ventricular filling occurs due to high atrial
driving pressures & unimpeded ventricular relaxation, followed by a
sudden rapid rise in pressure from pericardial restraint.
 This accounts for the rapid “y”descent on the atrial pressure waveform
and “square root” sign on ventricular pressures
 Although diastolic pressures are high, there is a paradoxically low stroke
volume from low preload.
 Preserved atrial relaxation, as well as an exaggerated ventricular
longitudinal contraction, result in an exaggerated “x” descent on atrial
pressure tracings
Hemodynamics of constriction

15. LV(blue) and RA(orange) hemodynamic pressure tracings in constrictive pericarditis
(CP). Prominent “x” and “y” descents are present with a square root sign (*).

16.  Rigid pericardium isolates cardiac chambers from intrathoracic pressure
swings.
 This causes under-transmission of reduced intrathoracic pressures to
cardiac chambers during inspiration.
 Inspiratory reduction in pulmonary capillary and venous pressures
reduces the flow between the pulmonary veins and left-sided cardiac
chambers.
 Rigid pericardium with a relatively fixed intrapericardial volume,
reduced LV filling allows increased RV filling.
 This is accompanied by inspiratory interventricular septal motion towards
the LV.
Hemodynamics of constriction

17.  Inspiratory increase in IVC flow, augmented by increased
trans-diaphragmatic pressure, competes with flow from the
SVC into the high-pressure RA.
 The resultant increase in JVP with inspiration is termed
‘Kussmaul’s sign’
Hemodynamics of constriction

18. 
 The converse is seen in expiration. With expiration, there is a rise in
intrathoracic (and therefore pulmonary venous) pressures.
 This augments flow into the left heart.
 Increased left heart filling within a fixed total intrapericardial volume
pushes the interventricular septum towards the right
 Thus, reducing RV filling, and creating expiratory diastolic flow
reversals transmitted back to the inferior vena cava and hepatic veins
Hemodynamics of constriction

19. Schematic representation of transvalvular and central venous flow velocities in CP.
During inspiration the decrease in LV filling results in a leftward septal shift,
allowing augmented flow into the RV. The opposite occurs during expiration.

20.  This respirophasic hemodynamic augmentation is an important and
specific feature of constrictive physiology.
 Increased ventricular interdependence directly translates to an alteration
in ventricular systolic pressures.
 Although these pressures rise and fall in parallel with respiration in
normal physiology, systolic pressures become discordant in CP, a marker
that is both sensitive and specific.
Hemodynamics of constriction

21. LV(blue) and RV(orange) hemodynamic pressure tracings in CP. End-diastolic
filling pressures are elevated & a “square root” sign is present on both pressure
tracings(*). Enhanced ventricular interdependence is present, demonstrated by
visualization of the systolic area index; RV(gray) and LV(dark gray) areas under the
curve are shown for both Insp. and Exp. During inspiration, there is an increase in
the area of RV pressure curve & decrease in the area of LV pressure curve.

22.  Conventional assessment of enhanced ventricular interdependence by
comparing peak ventricular pressures is not sensitive.
 A change in systolic area calculated by multiplying LVSP and systolic
ejection period is better determinant of beat to beat change
in stroke volumes.
 The systolic area index (SAI) is then calculated as the ratio of RV area
(mm Hg × s) to the LV area (mm Hg × s) in inspiration versus expiration.
 The index is significantly higher in patients with CCP compared with
RCMP. A ratio > 1.1 has a sensitivity of 97% & predicted accuracy of
100% for identification of CCP
Hemodynamics of Constriction

23. 
Hemodynamics of
Restriction

24.  Unlike the complex interplay of pulmonary and systemic pressures
associated with CP, RCM is the result of abnormalities intrinsic to the
myocardium, which are unchanged during respiration.
 As with CP, there is early rapid filling of the ventricles in early diastole,
due to high atrial pressures, followed by limitation in filling from the stiff
myocardium.
 This results in a prominent “y” descent on the atrial pressure curves, as
well as the “square root” sign on ventricular pressure curves.
 The stiff, noncompliant ventricles are unable to easily accept additional
increments in volume during atrial contraction, and thus the contribution
from atrial contraction is often minimal.
Hemodynamics of Restriction

25. LV and RA pressure hemodynamic pressure tracings in restrictive cardiomyopathy
(RCM). A prominent “y” descent is present, but the “x” descent is blunted

26.  Unlike CP, the “x” descent is frequently blunted, given poor atrial
relaxation and a limited descent of the annulus towards the apex.
 Increased venous flow with inspiration is unable to be accommodated by
a noncompliant RV; hence, there are diastolic flow reversals in the hepatic
vein with inspiration.
 Unlike CP, there is no discordance of intracavitary and intrathoracic
pressures.
Hemodynamics of Restriction

27. LV and RV pressure tracings in restrictive cardiomyopathy. Although end-diastolic
filling pressures are elevated and a square root sign (*) is present, there is no
evidence of enhanced ventricular interdependence, with parallel changes in LV and
RV pressure curve areas.

28. 
 High systemic venous pressure and reduced cardiac output induce
retention of sodium and water by the kidneys.
 Inhibition of natriuretic peptides may exacerbate increased filling
pressures
Hemodynamics

29. 
Clinical Features

30.  Equalization of intracardiac pressures in CP results in systemic venous
congestion, manifested as
 Edema,
 Hepatomegaly &
 Ascites (ascites out of proportion to the edema favours CP).
 Elevated JVP that increase with inspiration (Kussmaul’s sign).
 Prominent “x” and “y” descents.
 Robust early ventricular filling accompanying the “y” descent with
sudden deceleration results in an early diastolic, high-pitched pericardial
knock.
Clinical Features

31. 
 The most notable cardiac physical finding is the pericardial knock
 An early diastolic sound best heard at the left sternal border and/or the
cardiac apex.
 It occurs slightly earlier and has a higher frequency content than S3
 Corresponds to early, abrupt cessation of ventricular filling.
Clinical Features

32.  RCM results in predominant findings of systemic venous congestion.
 Compared with CP, concomitant pulmonary venous congestion is more
common in RCM, presenting as dyspnea.
 Kussmaul’s sign may be present in RCM and is therefore a nonspecific
finding.
 A prominent “y” descent is seen on the jugular venous contour,
accompanied by an S3, given rapid early filling of a stiffened ventricle.
 Unlike CP, a pronounced “x” descent is not seen
Clinical Features

33. 
Echocardiography

34.  Increased pericardial thickness can be recognized on TTE, although
interpretation is often challenging.
 Pericardial thickness >3 mm on TEE is both sensitive and specific
 Ventricular septal shifting on M-mode is usually the first
echocardiographic clue to the diagnosis of CP because it is present in
almost all patients with CP.
 Beyond the respirophasic motion, a septal “bounce,” also referred to as a
“shudder” or “diastolic checking,” may be present with each beat in
patients with CP, translating to a septal notch on M-mode imaging
Echocardiography

35. 

36. 

37. 
M-mode of the ventricular septum demonstrates respirophasic septal shift
(downward translation of the septum with inspiration, upward translation with
expiration) and septal shudder (circle, with enlarged view in upper right corner)
in a patient with constrictive pericarditis.
CCP

38.  Mitral (and tricuspid) Doppler inflow patterns in both CP and RCM are
early diastolic velocity (E-wave) predominant with a short deceleration
time, reflecting the predominance of early rapid ventricular filling.
 A critical difference is the presence of respiratory flow variation in CP,
which is absent in RCM
 Reportedly, mitral inflow in CP demonstrates a respiratory variation of
>25%, with increased velocities during expiration
 Mitral E-wave deceleration time is decreased & is usually < 160 ms.
 The lack of respiratory variation should not exclude the diagnosis of CP.
Doppler flow pattern

39. 
Pulsed-wave Doppler of the mitral inflow shows >25% expiratory increase in
velocities (arrows).
CCP

40. 
Pulsed-wave Doppler of the mitral inflow shows a restrictive pattern, with early
diastolic mitral inflow Doppler velocity (E) greater than late velocity (A) and short
deceleration time.
RCM

41. 
 Doppler respirophasic variation is similarly seen in the pulmonary veins,
with peak diastolic flow >18% variation suggestive of CP.
 Tricuspid inflow Doppler demonstrates the reverse finding, namely a
>40% increase in tricuspid velocity in the first beat after inspiration in CP.
 Hepatic vein Doppler interrogation in CP shows decreased expiratory
diastolic hepatic vein forward velocities with large expiratory diastolic
reversals.
Doppler flow pattern

42. 
Hepatic vein pulsed-wave Doppler shows decreased expiratory forward velocities
and large expiratory diastolic flow reversals (arrowhead).
CCP

43. 
Hepatic vein pulsed-wave Doppler shows increased inspiratory forward velocities
(arrow), inspiratory diastolic flow reversals (arrowhead), and minimal expiratory
diastolic flow reversals (rounded arrow).
RCM

44.  Of all echocardiographic parameters, perhaps the most useful to
distinguish CP and RCM is mitral annular tissue Doppler assessment.
 Early medial mitral annular tissue Doppler e’ velocities are normal or
paradoxically increased despite increased filling pressures, termed
“annulus paradoxus”
Tissue Doppler assessment

45. 
Medial mitral annulus tissue Doppler demonstrates elevated early diastolic
velocities (e’ ), despite increased filling pressures (annulus paradoxus).
CCP

46. 
 Tethering of the LV free wall can result in reversal of the relationship
between medial and lateral mitral annular tissue Doppler velocities,
 Lateral cardiac motion is limited; hence, ventricular filling depends upon
longitudinal cardiac motion.
 As such, the medial e’ is higher (typically >7 cm/s) than lateral e’, also
known as “Annulus reversus”.
Tissue Doppler assessment

47. 
CCP
Lateral mitral annulus e’ is decreased relative to the medial annulus (annulus reversus)
due to lateral tethering.

48. 
Lateral mitral annulus tissue Doppler demonstrates markedly reduced early diastolic
velocity (e’ ).
RCM

49. 
Apical 4-chamber 2-dimensional echocardiography reveals severe biatrial
enlargement (OWL’s eye appearance).
RCM

50. 
 Regional variations in deformation and strain include reduced LV
circumferential strain, torsion, and early diastolic untwisting with
preserved longitudinal strain and deformation in CP.
 In contrast, in RCM restriction, circumferential strain and untwisting are
preserved but these parameters are reduced in the longitudinal direction.
Tissue Doppler assessment

51. 
Cardiac Catheterization

52.  Gold standard for the diagnosis of CP and RCM
 In both diseases, catheterization demonstrates early rapid diastolic filling,
with elevation & equalization of end-diastolic pressures
 Although presence of pulmonary hypertension favours RCM, 1/3rd
patients with surgically proven CP have pulmonary hypertension
 After brisk early filling, ventricular pressure rises rapidly as pericardial
constraining volume is reached, resulting in “square root” or “dip and
plateau” sign.
 Although this can be seen in both CP and RCM, a LV rapid filling wave
with a height >7 mm Hg favors CP
Hemodynamic catheterization

53.  Kussmaul’s sign, quantified as <5 mm Hg decrease in inspiratory RA
pressure, is often present in both CP and RCM
 Disproportionate abnormalities of diastolic dysfunction result in a ratio of
RVEDP to RVSP >1:3 in CP
 Equalization of diastolic filling pressures in CP (≤5 mm Hg difference in
LV and RV end-diastolic pressure) results from fixed pericardial volume
and increased ventricular interdependence
Hemodynamic catheterization

54. 
 Dissociation of intrathoracic and intracardiac pressures can be analyzed
utilizing simultaneous LV and pulmonary artery wedge pressure
tracings.
 In CP, there is a decrease in the initial wedge-LV pressure gradient
during the first beat of inspiration, which is not present in RCM
Hemodynamic catheterization

55. CONSTRICTION RESTRICTION
Prominent y descent in venous
pressure
Present Variable
Paradoxic pulse ~1/3 cases Absent
Pericardial knock Present Absent
right- and left-sided filling
Pressures
Left at least 3-5 mm Hg
higher than right
Equal
Filling pressures > 25 mm Hg Rare Common
Pulmonary artery systolic
pressure > 60 mm Hg
No Common
pulmonary venous flow Normal systolic &
diastolic flow
systolic flow is blunted &
diastolic flow is increased
Hepatic veins demonstrate
(RICE)
enhanced expiratory flow
reversal with constriction
increased inspiratory flow
reversal
Square root sign Present Variable
Summary

56. CONSTRICTION RESTRICTION
Respiratory variation in left-
sided and right-sided
pressures/Flows
Exaggerated Normal
Enhanced respiratory variation
in mitral inflow velocity
Yes (>25%) No (≤ 10%)
Ventricular wall thickness Normal Usually increased
Pericardial thickness Increased Normal
Atrial size Possible LA enlargement Biatrial enlargement
Septal bounce Tissue Present Absent
Doppler E′ velocity Increased Reduced
Speckle tracking Normal longitudinal,
Decreased circumferential
Restoration
Decreased longitudinal,
normal circumferential
restoration
systolic area index Greater (>1.1) Lesser (<1.1)

57. 
Thank You!!!