Role of Echocardiography in the Diagnosis of Constrictive Pericarditis

STATE OF THE ART REVIEW
Role of Echocardiography in the Diagnosis of
Constrictive Pericarditis
Jacob P. Dal-Bianco, MD,* Partho P. Sengupta, MBBS, MD, DM,**
Farouk Mookadam, MD, Krishnaswamy Chandrasekaran, MD, A. Jamil Tajik, MD, and
Bijoy K. Khandheria, MBBS, FACC, FESC,Rochester, Minnesota; Scottsdale, Arizona
The clinical recognition of constrictive pericarditis (CP) is important but challenging. In addition to Doppler
echocardiography, newer echocardiographic techniques for deciphering myocardial deformation have facilitated
the noninvasive recognition of CP and its differentiation from restrictive cardiomyopathy. In a patient with heart
failure and a normal ejection fraction, echocardiographic demonstration of exaggerated interventricular interdependence, relatively preserved left ventricular longitudinal deformation, and attenuated circumferential deformation is diagnostic of CP. This review is a concise update on the pathophysiology and hemodynamic features of
CP, the transmural and torsional mechanics of CP, and the merits and pitfalls of the various echocardiographic
techniques used in the diagnosis of CP. (J Am Soc Echocardiogr 2009;22:24-33.)
Keywords: Constrictive pericarditis, Echocardiography, Doppler tissue imaging, Restrictive
cardiomyopathy, Left ventricular deformation
Constrictive pericarditis (CP) is characterized by impaired diastolic
cardiac filling and elevated ventricular filling pressures (Figure 1) due
to a rigid pericardium with fusion of the visceral and parietal layers.1,2
Patients present predominantly in heart failure with elevated jugular
venous pressure, dyspnea, peripheral edema, hepatomegaly, and
ascites.3 Pericarditis, cardiac surgery, and mediastinal irradiation are
the leading identifiable causes of CP in the developed world. However, pericardial fibrosis and calcification are often idiopathic in
origin.3 Pericardiectomy relieves pericardial restraint and, in the
absence of concomitant myocardial dysfunction, effectively restores
diastolic filling.4-6 CP due to cardiac surgery, especially coronary
artery bypass grafting7 and mediastinal irradiation,3,8,9 can involve
the pericardium and also the myocardium.3,10,11 Determining the
relative contribution of pericardial restraint versus myocardial dysfunction in such patients remains a diagnostic challenge.
PATHOPHYSIOLOGY OF CONSTRICTIVE PERICARDITIS
In CP, the visceral pericardium and the parietal pericardium are
fibrosed and fused together,2 although not necessarily always thickened (Figure 2A).12 Calcification can result in the formation of
pericardial calcium plaques (eggshell appearance), which may penetrate into the myocardium.2 The incidence and prevalence of peri-
From the Division of Cardiovascular Diseases, Mayo Clinic, Rochester, MN
(J.P.D.); and the Division of Cardiovascular Diseases, Mayo Clinic, Scottsdale, AZ
(P.P.S., F.M., K.C., A.J.T., B.K.K.).
This work was supported in part by a grant-in-aid to Dr Sengupta from the
American Society of Echocardiography (Morrisville, NC).
Reprint requests: Partho P. Sengupta, MD, DM, Mayo Clinic Arizona, Division of
Cardiovascular Diseases, 13400 East Shea Boulevard, Scottsdale, AZ 85259
(E-mail: [email protected]).
*
Drs Dal-Bianco and Sengupta contributed equally to this work.
0894-7317/$36.00
Copyright 2009 by the American Society of Echocardiography.
doi:10.1016/j.echo.2008.11.004
24
cardial calcification is dependent on the underlying cause of CP and
is less commonly encountered in developing nations as the incidence
of tuberculous pericarditis has declined. A recent series of 136
patients reported radiographic evidence of pericardial calcification in
approximately one third of patients.13 The myocardium is usually
normal in CP, but concomitant myocardial involvement may be seen,
for example, following mediastinal irradiation.14 Epicardial involvement may also result from subpericardial myocarditis.2
Characteristic hemodynamic changes in CP are attributed to
isolation of the cardiac chambers from intrathoracic respiratory pressure changes and a fixed end-diastolic ventricular volume (Figure 2B).
Although normally, the pressure gradient between the pulmonary
veins and the left atrium and left ventricle is relatively constant during
respiration, this pressure gradient decreases with inspiration in CP,
leading to reduced diastolic pulmonary vein flow and left atrial and
left ventricular (LV) filling (Figure 1). The rigid, noncompliant fibrous
pericardial sac couples both ventricles. Consequently, an increase in
filling on one side of the heart impedes contralateral filling through
the motion of the interventricular septum (IVS), thereby making both
ventricles markedly interdependent.15-17 The inspiratory reduction in
LV filling is therefore associated with a simultaneous increase in right
ventricular (RV) diastolic filling and an IVS shift toward the left
chamber.18 The opposite physiologic effects on filling gradients and
IVS shift are seen in expiration, with increased pulmonary venous
pressure and an attendant increase in LV diastolic inflow. This is
associated with simultaneous decrease in right-sided filling. Because
of elevated atrial pressures, ventricular filling is very rapid and
predominantly in early diastole, abruptly declining by middiastole.19
This hemodynamic pattern is reflected in the jugular venous and right
atrial pressure waveforms as elevated mean filling pressure with a
prominent “a” wave, a sharp “y” descent reflecting early diastolic
resistance-free rapid RV filling, and as well as a preserved “x” descent
due to accelerated atrial relaxation. The RV hemodynamic waveform
with a dip-and-plateau, or square root, pattern reflects rapid ventricular relaxation, with a subsequent sharp increase in filling pressure as
the expanding ventricle meets the pericardium (Figure 1A). At
end-diastole, the end-diastolic right atrial, RV, wedged pulmonary
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Volume 22 Number 1
Dal-Bianco et al
25
interactions between the right and left heart. Importantly, however,
although early ventricular filling is resistance free in CP, the effusion
in cardiac tamponade imposes pandiastolic resistance to ventricular
filling.
ECHOCARDIOGRAPHIC DIAGNOSIS OF CONSTRICTIVE
PERICARDITIS
The echocardiographic diagnosis of CP was originally based on
M-mode echocardiographic findings and subsequently on 2-dimensional echocardiography and Doppler hemodynamics in response to
the respiratory cycle. More recently, newer echocardiographic techniques, such as pulsed tissue Doppler, color Doppler tissue imaging
(DTI), and speckle-tracking imaging, have been used to assess the
unique changes in global and regional myocardial function seen in
CP. However, while interpreting the role of various echocardiographic techniques and comparing individual studies, several facts
must be kept in consideration: (1) CP is a heterogeneous disease, and
study populations vary significantly regarding the distribution of
underlying CP etiologies; (2) the confirmation of a firm diagnosis of
CP is based on different “gold standards” (eg, surgical confirmation vs
pericardial thickness on computed tomography); and (3) pericardial
constriction may be localized, affecting the right ventricle more than
the left ventricle or vice versa. Data on generalized versus localized
pericardial constriction are sparse.
Figure 1 Cardiac catheterization findings in patients with CP.
Respiratory changes in LV and RV systolic pressures (A) show a
reciprocal relationship throughout the respiratory cycle, suggesting increased interventricular interdependence. Similarly, the early
diastolic transmitral pressure gradient as estimated from the
difference of pulmonary capillary wedge pressure and LV minimum pressure (B) shows a significant respiratory change, suggesting dissociation of intrathoracic and intracardiac pressures.
Reproduced with permission from Circulation.17
arterial, and LV pressures are equally elevated. These physiologic
abnormalities that occur as a result of the ventricular interdependence and respiratory influenced changes are the fundamental processes that account for the clinical, hemodynamic, and echocardiographic findings of CP.
It is important to distinguish the hemodynamic features of CP from
those of cardiac tamponade, a condition characterized by excess fluid
in the pericardial sac that hinders diastolic filling. In cardiac tamponade, the intracardiac volumes are decreased on both sides of the
heart, with elevation and equalization of diastolic filling pressures
throughout the cardiac chambers, reflecting increased ventricular
Doppler Hemodynamics
Mitral inflow as assessed by Doppler echocardiography demonstrates
an increased early diastolic filling velocity followed by rapid deceleration, leading to a short filling period. Early mitral inflow deceleration
time is usually, but not always, ⬍160 ms.18,20
As first reported by Hatle et al18 and Oh et al21 in a larger cohort,
dynamic changes with respiration occur in patients with CP (Figure 2D),
but not in patients with restrictive cardiomyopathy (RCM; Figure 3B).
Factors responsible for these respiratory driven changes are the dissociation of intrathoracic pressure from intracardiac pressure and enhanced
ventricular interdependence. In CP, early diastolic mitral inflow is reduced with the onset of inspiration, and isovolumic relaxation time is
prolonged. With expiration, mitral inflow returns to normal, and isovolumic relaxation time shortens. Typically, patients with CP demonstrate an
increase in early diastolic mitral inflow velocity of ⱖ25% during expiration compared with inspiration.18,21 These changes can be seen with the
first beat of inspiration, when a decrease in transmitral flow velocity is
noted; the reverse occurs with expiration. Sensitivities and specificities of
Doppler assessment of respiratory changes have been reported to be as
high as 85% to 90%.22 Importantly, the percentage of respiratory
change in patients with “mixed” constrictive and restrictive disease (eg,
mediastinal radiation) is markedly lower.10 After complete pericardiectomy mitral inflow patterns return to normal, and little respiratory
variation is seen on Doppler echocardiography.18 Accordingly, Doppler
assessment of respiratory variation has been reported to be useful for
evaluating the outcome of pericardiectomy.21 Patients who had minimal
respiratory variation after pericardiectomy were asymptomatic compared with those who continued to show respiratory variation.23
Patients with RCM (Figure 3) and up to 20% of patients with CP
may lack the typical respiratory changes in the presence of mixed
constrictive-restrictive disease and/or markedly increased left atrial
pressure.21,24 Typical respiratory changes in the latter situation may
not be observed, because of mitral valve opening during the steep
portion of the LV pressure curve, when respiration has little effect on
26
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Figure 2 Findings in patients with CP. (A) Computed tomography in a patient with CP shows the extent of pericardial thickening
(red arrows). Apical 4-chamber view of the left ventricle (LV) (B) and dilated inferior vena cava (C) with increased respiratory
variations in transmitral early diastolic flow (D) and hepatic venous Doppler flow (E) are shown for the same patient with CP (red
arrows in D and E indicate respiratory-dependent change in Doppler flow). Exp, Expiration; Insp, inspiration.
Figure 3 Echocardiographic features in a patient with RCM due to cardiac amyloidosis. Apical 4-chamber view shows thick walled
left ventricle (LV) (A) with a restrictive transmitral early diastolic flow pattern (B). Note the lack of increase in early diastolic transmitral
velocity with the onset of expiration (Exp). Insp, Inspiration.
the transmitral pressure gradient. In these patients, maneuvers that
decrease preload (head-up tilt or sitting) can unmask the characteristic
respiratory variation in early mitral inflow velocity.24 Atrial fibrillation
complicates the interpretation of respiratory variation of Doppler
velocities, but respiratory variation can still be appreciated regardless
of cardiac cycle length. Usually, this requires longer recording periods
of Doppler tracings.
Phasic, respiratory changes in mitral inflow are not unique to CP,
however. Patients with chronic obstructive pulmonary disease or
severe RV dysfunction and large respiratory variations in intrathoracic
pressure may also show inspiratory decreases in early mitral inflow
velocities. Typically, these changes are more gradual and occur later
in the respiratory cycle rather than during the first inspiratory beat. In
such patients, a marked increase in inspiratory superior vena cava
systolic forward flow can be helpful to rule out CP.25 In chronic
obstructive pulmonary disease, there is a greater decrease in intrathoracic pressure in inspiration, which generates greater negative pressure changes in the thoracic cavity. This augments blood flow to the
right atrium from the superior vena cava during inspiration.
Inferior vena cava dilation (Figure 2C) and pulsed wave Doppler of
hepatic venous flow mirrors right atrial pressure. Pulsed Doppler
recordings of hepatic vein flow in CP show marked diastolic flow
reversal (Figure 2E), which increases in expiration compared with
inspiration.26 In patients with advanced constriction or with mixed
constrictive-restrictive physiology, it is not unusual to see significant
diastolic flow reversals during both inspiration and expiration. In
contrast, diastolic hepatic vein flow reversal is more prominent with
inspiration in RCM.
Doppler evaluation of the pulmonary veins shows marked respiratory
change in pulmonary venous flow in CP. The pulmonary venous systolic
wave and early diastolic wave velocities, especially the early diastolic
wave velocity, are increased during expiration and decreased during
inspiration.18 The changes in pulmonary venous flow velocities have
been reported to be more pronounced than changes in mitral inflow
velocities.27 Similar respiratory variation can also be observed in patients
with CP and atrial fibrillation.28 In contrast, patients with RCM show
blunting of the systolic wave velocity and a decrease in the ratio of
systolic to early diastolic wave velocity throughout the respiratory cycle,
with a large atrial reversal wave and without any significant respiratory
variation. (Table 1 summarizes sensitivities and specificities for hemodynamic CP Doppler findings.)
Color Doppler
Patients with CP typically demonstrate only mild mitral and tricuspid
regurgitation. A pronounced increase in tricuspid regurgitant jet
velocity from onset to peak inspiration can, however, help diagnose
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Table 1 Sensitivities and specificities of echocardiographic markers for CP
Marker
Doppler echocardiography
ⱖ25% respiratory variation of peak early diastolic MV inflow
velocity; augmented hepatic vein diastolic flow
reversals after the onset of expiration; ⱖ 25% of
forward diastolic velocity
ⱖ10% respiratory variation of peak early diastolic MV inflow
velocity
Color M-mode MV inflow propagation; first aliasing contour
ⱖ 100 cm/s
Respiratory variation in PV systolic/diastolic flow ratio ⱖ
65% in inspiration ⫹ % change of early mitral peak
diastolic flow ⱖ 40%
Respiratory variation in PV peak diastolic flow velocity ⱖ
18%
Dilated hepatic veins, “W” wave pattern (reverse flow in late
systole and diastasis)
LV septal/posterior wall radial motion
IVS bounce
M-mode
M-mode
2-dimensional
Biphasic early diastolic IVS motion by color DTI (ⱖ7 cm/s)
motion
Biphasic early diastolic IVS motion by pulsed tissue Doppler
LV posterior wall flattening
M-mode
M-mode
M-mode
Miscellaneous echocardiographic findings
Pericardial thickening
M-mode
M-mode
2-dimensional
Left atrial enlargement, M-mode
Premature PV opening, M-mode
Study
n
Secondary CP
Sensitivity
Specificity
Oh et al21
28
13
88%
67%
Rajagopalan et al30
19
6
84%
91%
Rajagopalan et al30
19
6
74%
91%
Klein et al27
14
10
86%
94%
Rajagopalan et al30
19
6
79%
91%
von Bibra et al26
13
8
68%
100%
Engel et al31
Candell-Riera et al32
Himelman et al33
Sengupta et al34
40
8
39
40
NA
NA
NA
30
40%
88%
62%
82%
80%
NA
93%
93%
Oki et al46
12
9
100%
100%
Voelkel et al38
Schnittger et al37
Engel et al31
12
14
40
2
6
NA
92%
64%
85%
100%
90%
82%
Engel et al31
Hatle et al18
Oh et al21
Engel et al31
Engel et al31
40
7
28
40
40
NA
4
19
NA
NA
53%
100%
36%
75%
14%
100%
50%
NA
100%
100%
MV, Mitral valve; NA, data not available; PV, pulmonary valve.
CP.29 Severe mitral and tricuspid regurgitation speak against isolated or
predominant constrictive physiology and are markers for underlying
myocardial disease. Color M-mode Doppler shows early-onset, elevated
mitral inflow velocities in patients with CP. Rajagopalan et al30 reported
sensitivity and specificity of 74% and 91%, respectively, to differentiate
CP from RCM (mitral inflow slope ⬎100 cm/s).
LV Mechanics
Radial motion of the IVS. Abrupt anterior or posterior motion of
the IVS in early diastole is common in patients with CP (Figure 4). The
direction of early diastolic IVS motion may depend on a number of
factors, including uneven distribution of fibrosis or calcification in the
pericardial sac, the timing of mitral and tricuspid opening, the relative
compliance of the right and left ventricles, and phase of respiration.31
In classic CP, the IVS shows a brisk, early diastolic motion toward the
left ventricle during inspiration, followed by a rebound in the opposite direction during expiration.32 This septal bounce reflects exaggerated interventricular dependence combined with forceful early
diastolic filling. Himelman et al33 reported in a large number of
patients that an IVS bounce is the most consistent 2-dimensional
echocardiographic sign for CP, with sensitivity of 62% and specificity
of 93%. Recently, Sengupta et al34 assessed IVS motion by color DTI
(Figure 4). In this study, the majority of patients with CP had
high-velocity early diastolic biphasic IVS motion (⬎7 cm/s), a finding
with 82.5% sensitivity and 92.7% specificity for CP (Table 1).
Radial motion of the LV posterior wall. In patients with CP, the
LV posterior wall rapidly expands posteriorly during early diastole,
followed by an abrupt cessation of such movement during middiastole and late diastole (Figure 4), which corresponds to the abrupt
termination of rapid ventricular filling.35,36 This lack of motion,
termed “flattening,” of the LV posterior wall during middiastole and
late diastole can be best observed with M-mode echocardiography.37,38 Palka et al39 assessed relative subendocardial versus subepicardial LV posterior wall velocities using myocardial velocity gradient
imaging. Patients with CP (n ⫽ 10) had markedly faster moving
subendocardium during rapid ventricular filling than normal subjects
and patients with RCM (Table 1).
Longitudinal LV mechanics. The quantitative assessment of longitudinal mitral annular motion by pulsed tissue Doppler and color
DTI estimates LV relaxation.40-42 In patients with CP, mechanoelastic properties of the myocardium are relatively preserved in the
longitudinal direction, and therefore longitudinal deformation of the
LV base (Figure 5) and longitudinal early diastolic velocities (Figure 6)
28
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Figure 4 Color-DTI for quantifying abnormal septal and posterior wall motion in CP. Characteristic findings on M-mode
echocardiography (A) are septal notching (arrow 1) and the abrupt flattening of the posterior wall (arrow 2). Color M-mode
echocardiography (B) shows high-velocity motion (arrow 3) in early diastole. Myocardial velocity tracings from the septum and
posterior wall (C) identify components of abnormal septal motion in constriction. The posterior wall shows rapid early diastolic radial
expansion (blue tracing), which corresponds to the abrupt outward motion seen on M-mode. The septum shows high-velocity early
diastolic fluttering (yellow tracing), which corresponds to the septal notching seen on M-mode. Am, Radial late diastolic velocity; Em,
radial early diastolic velocity; Exp, expiration; LV, left ventricle; RV, right ventricle; Sm, radial systolic velocity.
Figure 5 Longitudinal deformation of the left ventricle in CP and RCM. Longitudinal shortening strains were obtained by
2-dimensional speckle-tracking imaging. Speckle-tracking imaging is different from DTI in that strain data are angle independent.
Myocardial deformation is computed from continuous frame-by-frame tracking of a small image block of “natural acoustic
markers.” Tracking is based on searching the new location of the marker in the subsequent frame using a block-matching algorithm.
The basal septal and basal lateral wall segments show normal longitudinal shortening strains in CP (A, B) and reduced longitudinal
shortening strains in RCM (C, D). Apical longitudinal shortening strains are reduced in CP (B), whereas longitudinal strain at the LV
apical septum is preserved in RCM (D).
are either normal or exaggerated. Conversely, patients with RCM and
intrinsic myocardial abnormalities have reduced longitudinal deformation of the LV base and reduced early diastolic longitudinal
velocities. Pulsed tissue Doppler and color DTI have been shown to
be helpful to diagnose CP.30,43-48 A lateral or septal early diastolic
mitral annular velocity of ⬎8 cm/s on pulsed tissue Doppler is in
general the accepted cutoff value to distinguish patients with CP from
those with RCM. Mitral annular velocities are particularly useful in CP
when pronounced respiratory variations in peak early mitral inflow
velocities are not seen.49 The additional use of pulsed tissue Doppler
to hemodynamic Doppler and 2-dimensional and M-mode echocardiography increases predominantly the sensitivity of echocardiogra-
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Volume 22 Number 1
29
Figure 6 Longitudinal velocity of the left ventricle in CP and RCM. Longitudinal velocities were obtained by 2-dimensional
speckle-tracking imaging. The basal septal and basal lateral wall segments show normal mean longitudinal early diastolic velocity
in CP (⬎5 cm/s) (A, B) and reduced longitudinal early diastolic velocity in RCM (C, D). Em, Longitudinal early diastolic lengthening
velocity.
Table 2 Studies assessing longitudinal early diastolic tissue velocities in CP
Study
n
Mean ⴞ SD
age (y)
Secondary
CP
Mitral annular
sample site
Cutoff value
(cm/s)
Sensitivity
Specificity
NA
NA
NA
89%
100%
ⱖ8
⬎(16 ⫺ 0.14 ⫻ age)
⫹2
⬎8
95%
76%
96%
82%
89%
73%
70%
93%
100%
100%
93%
100%
92%
90%
Comparison group
Garcia et al44
8
62 ⫾ 13
6
Lateral*
Rajagopalan
et al30
19
56 ⫾ 13
6
Lateral*
Ha et al45
Sohn et al48
23
17
59 ⫾ 13
46 ⫾ 14
8
10
Septal*
NA
CA (n ⫽ 4), nonspecific fibrosis
(n ⫽ 1), DM/small-vessel
disease (n ⫽ 1), cardiac
transplantation (n ⫽ 1)
CA (n ⫽ 8), advanced HTN
(n ⫽ 1), DM/small-vessel
disease (n ⫽ 1), idiopathic
(n ⫽ 1)
CA (n ⫽ 38), RCM (n ⫽ 14)
Normal (n ⫽ 35)
Sengupta
et al47
Choi et al43
Sengupta
et al51
45
24 ⫾ 12
30
Septal and lateral*
RCM (n ⫽ 10), CA (n ⫽ 1)
17
16
55 ⫾ 12
62 ⫾ 13
12
9
RCM (n ⫽ 12), normal (n ⫽ 15)
CA (n ⫽ 15)
⬎8
⬎6.6
⬎5
Sengupta
et al57
26
56 ⫾ 13
13
Septal*
Septal*
Averaged from 4
corners of MV
annulus†
Septal and lateral
CA (n ⫽ 15), RCM (n ⫽ 4)
⬎5
ⱖ8
CA, Cardiac amyloid; DM, diabetes mellitus; HTN, hypertension; MV, mitral valve; NA, data not available.
*Pulsed tissue Doppler.
†Mean velocity by color DTI obtained in 11 of the 16 patients.
phy to diagnose CP.47 Table 2 summarizes studies that have explored
the diagnostic role of early diastolic mitral annular velocity in CP.
Attention needs to be paid to the differing patient age groups (mitral
annular velocities decrease with age50) and different percentages of
underlying CP etiologies, with varying patient numbers with presumed “mixed” constrictive and restrictive physiology. These factors
may explain the spread of early mitral annular velocities and reported
sensitivities and specificities.
When recording and interpreting early mitral annular velocities,
caution is warranted in the setting of extensive mitral annular
calcification, LV systolic dysfunction, or segmental nonuniform
myocardial velocities.47 Averaging additional mitral annular sites is
30
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January 2009
Figure 7 Twist mechanics of the left ventricle in CP and RCM. Short-axis cross-sectional views are divided into 6 segments, and
2-dimensional speckle-tracking imaging is used for computing LV rotation from 6 segments in each cross-sectional view. LV apical
and basal rotation is averaged from 6 segments in each view, and the difference of apical and basal rotation provides the net twist
angle of the left ventricle. LV apical rotation and net twist angles are reduced in CP (A). Similarly, apical early diastolic untwisting
velocities obtained from 6 segments and the averaged value (dotted line) are also reduced in CP (B). Apical and basal LV rotation
is normal in RCM with a normal net twist angle (C) and normal early diastolic apical untwisting velocities (D). Er, Early diastolic apical
untwisting velocity.
of incremental value to differentiate CP from RCM.51 Although
patients with CP and RCM had overlapping values of early
diastolic pulsed tissue Doppler velocities, clear separation of
groups was evident with averaged color DTI early diastolic annular
velocities (averaged from 4 walls). However, when using color
DTI, one must note that resulting tissue velocities represent mean
velocities, which are significantly lower compared with peak
velocities derived on pulsed tissue Doppler.52 This is important
because most CP studies and early diastolic mitral annulus cutoff
values are based on pulsed tissue Doppler velocities (Table 2).
Furthermore, in RCM, mitral annular velocities may not be representative if the disease process is heterogeneous, as reported
previously in patients with endomyocardial fibrosis.53 Thus, hypokinetic areas accompanied by hyperkinesis in neighboring areas
could result in relatively unaltered overall annular motion.47 This
limits the ability of pulsed tissue Doppler to differentiate RCM
because of endomyocardial fibrosis from CP.
Circumferential LV mechanics. Because of pericardial restraint44
and potential epicardial involvement,2,54-56 LV expansion in CP may
be limited in the circumferential rather than the longitudinal direction. Accordingly, patients with CP were found to have reduced
circumferential strain in combination with preserved longitudinal
early diastolic velocities.57 This concept is further supported by the
significant positive correlation of circumferential strain and radial
displacement.
Torsional LV mechanics. By speckle-tracking imaging, Sengupta
et al57 showed that patients with CP have significantly reduced net
LV twist and torsion compared with normal subjects and patients
with RCM. While the base and mid cardiac rotation was relatively
Table 3 Myocardial mechanics in CP and RCM
Deformation parameter
CP
Longitudinal strain
Normal*
Longitudinal early diastolic velocity Normal or increased
Circumferential strain
Decreased
Net twist angle
Decreased
Apical untwisting velocity
Decreased
RCM
Decreased
Decreased
Decreased
Normal
Normal†
*Except apical segments.
†Although normal in magnitude, early diastolic apical untwisting veloc-
ities may be delayed in timing.
preserved, apical rotation was markedly reduced, including the
peak systolic and diastolic rotation rates (Figure 7). Early diastolic
recoil, LV untwisting, and the magnitude of circumferential and
longitudinal expansion of the left ventricle are modulated by
pericardial tissue properties.58,59 A recent study showed that the
congenital absence of the left pericardium results in reduced LV
torsion.60 The reduction of apical rotation in CP could be due to
a lack of normal pericardium, enabling swift and friction-free
apical motion and potential scarring and inflammation extending
into the epicardial layer of the myocardial wall.2,54-56 Pericardial
tethering and/or diminished epicardial fiber function manifest as
reduced basal, mid, and apical circumferential strain and also
reduced longitudinal strain at the apex,57 which may be susceptible to additional endocardial involvement due to the close spatial
relationship of epicardial and endocardial fibers.61 Table 3 summarizes LV mechanics in CP versus RCM.
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ANCILLARY ECHOCARDIOGRAPHIC FINDINGS IN
CONSTRICTIVE PERICARDITIS
Pericardium
The detection of increased pericardial thickness may be of help in
making a diagnosis of CP,35 but M-mode38 and 2-dimensional echocardiography62 show poor sensitivity and correlation with pathologic specimen measurements. Transesophageal echocardiography, on the other
hand, because of superior resolution, showed high sensitivity (95%) and
specificity (86%) to detect a pericardium ⱖ3 mm thick.63 Nevertheless,
about 1 in 5 patients with CP have normal pericardium,12 and the
isolated finding of thickened pericardium does not imply constrictive
physiology.64
Recently, Lu et al65 evaluated the relative motion of pericardium
versus myocardium in patients with CP. By quantitative DTI, epicardial motion was found to be reduced, approaching that of the
pericardium, whereas the endocardium was moving more vigorously.
These findings are interesting because they seem to support the
observations on subepicardial involvement in CP.2
Cardiac Valves
A steep E-F slope on mitral valve M-mode tracing is suggestive of
rapid early diastolic filling and can therefore be seen in CP.66
Premature opening of the pulmonary valve due to increased middiastolic RV pressure can be observed in patients with CP, if RV
pressure exceeds pulmonary artery diastolic pressure.67
Aorta
Another M-mode sign of the rapid early ventricular filling in CP is
a sharp downward motion of the posterior aortic root in early
diastole.68
Posterior LV Wall and Posterior Left Atrium Angle
In CP, the angle between the posterior LV wall and posterior left
atrium can be blunted, because the thickened, constricting pericardium affects the posterior left ventricle more than the posterior left
atrium, which then expands at a more acute angle respective to the
LV wall.69
Interatrial Septum
Displacement of the interatrial septum toward the left atrium during
inspiration is another reported sign of CP.70 (Table 1 summarizes
sensitivities and specificities for the miscellaneous echocardiographic
findings of CP.)
CONCLUSION
Multiple echocardiographic findings are used to confirm a diagnosis
of CP, but demonstration of enhanced cardiac intracavitary blood
flow variations during respiration, abnormal interventricular septal
motion, and normal longitudinal mitral annular velocity provide the
greatest diagnostic yield. In the presence of a mixed physiology
(pericardial constraint combined with myocardial dysfunction),
the diagnosis and therefore referral for pericardiectomy remain
challenging. In such patients, the assessment of LV longitudinal, circumferential, and torsional mechanics by 2-dimensional speckle-tracking
imaging can potentially decipher the extent of endocardial, midmyocardial, or epicardial involvement. Further prospective studies are
warranted to address the potential clinical utility of such diagnostic
algorithms.
31
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