JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY
21, 2016
Listen to this manuscript’s
audio summary by
JACC Editor-in-Chief
Dr. Valentin Fuster.
VOL. 68, NO.
ª 2016 BY THE AMERICAN COLLEGE OF CARDIOLOGY FOUNDATION ISSN 0735-1097/$36.00
PUBLISHED BY ELSEVIER http://dx.doi.org/10.1016/j.jacc.2016.08.050
FOCUS SEMINAR: PERICARDIAL AND MYOCARDIAL DISEASE
STATE-OF-THE-ART REVIEW
Differentiation of Constriction
and Restriction
Complex Cardiovascular Hemodynamics
Jeffrey B. Geske, MD, Nandan S. Anavekar, MD, Rick A. Nishimura, MD, Jae K. Oh, MD,
Bernard J. Gersh, MBCHB, DPHIL
ABSTRACT
Differentiation of constrictive pericarditis (CP) from restrictive cardiomyopathy (RCM) is a complex and often challenging
process. Because CP is a potentially curable cause of heart failure and therapeutic options for RCM are limited, distinction
of these 2 conditions is critical. Although different in regard to etiology, prognosis, and treatment, CP and RCM share
a common clinical presentation of predominantly right-sided heart failure, in the absence of significant left ventricular
systolic dysfunction or valve disease, due to impaired ventricular diastolic filling. Fundamental to the diagnosis of
either condition is a clear understanding of the underlying hemodynamic principles and pathophysiology. We
present a contemporary review of the pathophysiology, hemodynamics, diagnostic assessment, and therapeutic
approach to patients presenting with CP and RCM. (J Am Coll Cardiol 2016;68:2329–47) © 2016 by the American College
of Cardiology Foundation.
D istinction of constrictive and restrictive
hemodynamics remains one of cardiovas-
cular medicine’s most complex challenges
(1–10). Both result in impaired ventricular filling
CONSTRICTION AND RESTRICTION:
PATHOLOGY AND ETIOLOGY
PATHOLOGY AND ETIOLOGY OF CONSTRICTION. Iden-
with clinical manifestations of predominantly right tification of pericardial disease dates back to Biblical
heart failure with preserved ejection fraction. times, with observation of clinical signs attributable
Constrictive pericarditis (CP) is a potentially revers- to CP harkening to the renaissance period, and mod-
ible cause of heart failure, whereas restrictive ern understanding arising during the 19th century
cardiomyopathy (RCM) has very limited therapeutic (11). CP is a pathological condition with encasement
options. Therefore, the ability to differentiate of the heart by a thickened, fibrous, and sometimes
between these conditions remains paramount to calcified pericardium, with secondary abnormalities
therapy. Herein, we present a review of hemody- in chamber filling. The etiologic mechanisms of peri-
namic differentiation of CP and RCM, as well as cardial pathology have evolved over the past century,
discussion of the underlying pathophysiology, diag- with an increasingly significant iatrogenic contribu-
nostic assessment, and therapeutic approaches to tion from post-surgical inflammation and radiation
both entities. therapy (12). There are major geographic influences
From the Department of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota. Dr. Anavekar has reviewed literature for
Frestedt Incorporated, related to wound vacuum and TEE probe covers. Dr. Gersh has served on the data safety monitoring boards
of Mount Sinai St. Luke’s Hospital, Boston Scientific, Teva Pharmaceutical Industries, St. Jude Medical, Janssen Research &
Development, Baxter Healthcare, and the Cardiovascular Research Foundation; has been a consultant to Janssen Scientific Affairs
and Xenon Pharmaceuticals; and has been on the advisory board of Medtronic. All other authors have reported that they have no
relationships relevant to the contents of this paper to disclose.
Manuscript received May 2, 2016; revised manuscript received August 4, 2016, accepted August 9, 2016.
2330 Geske et al. JACC VOL. 68, NO. 21, 2016

Constriction vs. Restriction: Complex Cardiovascular Hemodynamics NOVEMBER 29, 2016:2329–47

ABBREVIATIONS on etiology, with tuberculosis the most associated sequelae. Unfortunately, therapeutic
AND ACRONYMS frequent cause of pericardial diseases in approaches to RCM remain challenging. Despite
developing countries and in the world, often optimal heart failure care, definitive treatment is
3D = 3-dimensional
coupled with human immunodeficiency virus often limited to cardiac transplantation.
BNP = B-type natriuretic
coinfection (13). Of noninfectious etiologies, MIXED CONSTRICTION AND RESTRICTION. Consid-
peptide
most are idiopathic, whereas the remainder erable overlap of CP and RCM may be present,
CMR = cardiac magnetic
resonance stem from iatrogenic causes, connective tis- particularly in the setting of prior chest radiotherapy.
CP = constrictive pericarditis
sue diseases, neoplasms, or uremia. Fibrous Radiation indiscriminately affects both pericardial
thickening of the pericardium forms a rigid, and myocardial tissues, in addition to the valves and
CT = computed tomography
0
noncompliant encasement of the heart, coronary vessels. Mixed constriction/restriction can
e = early diastolic velocity
resulting in progressive impairment in car- also be seen post-operatively, including post-cardiac
EMB = endomyocardial biopsy
diovascular filling and low cardiac output transplantation. Although post-operative mecha-
FDG = 18-fluorodeoxyglucose
(14). Symptoms often arise insidiously in CP, nisms remain suboptimally defined, mixed features
LA = left atrium/atrial
culminating in progressive signs and symp- may arise as a combination of pericardial scarring and
LV = left ventricle/ventricular toms of predominantly right heart failure. procedure-mediated myocardial dysfunction (23).
NYHA = New York Heart Complete surgical removal of the pericar- There is a paucity of data to describe this mixed pic-
Association
dium can result in excellent symptomatic ture, and therefore, the true prevalence remains un-
PET = positron emission
improvement. The prognosis is dependent known. Yamada et al. (23) reported that 12.7% of
tomography
upon the underlying etiology, with prior ra- patients referred for evaluation of CP or RCM were
RA = right atrium/atrial
diation therapy consistently associated with found to have overlapping features. In that same se-
RCM = restrictive
worse outcomes (12,15). For unclear reasons, ries, patients with mixed disease had significantly
cardiomyopathy
there is male predominance of CP in most worse survival compared with those with isolated CP.
RV = right ventricle/ventricular
clinical series. Mixed constrictive and restrictive hemodynamics
pose a significant management dilemma, because the
PATHOLOGY AND ETIOLOGY OF RESTRICTION. RCM is a
clinical outcome of high-risk surgical interventions
primary disease of the myocardium. RCM is charac-
may be uncertain.
terized by increased myocardial stiffness, which
results in a rapid rise in ventricular filling pressures HEMODYNAMIC CHARACTERISTICS OF
reflected in both the systemic and pulmonary cir- RESTRICTION AND CONSTRICTION
culations. Despite marked abnormalities in diastolic
function, left ventricular (LV) ejection fraction is NORMAL CARDIAC HEMODYNAMICS. Understanding
typically preserved. Primary RCM is often idiopathic, normal hemodynamic findings is integral for recog-
although familial inheritance has been described, nition of derangements present in CP and RCM.
with desmin and troponin I mutations implicated Although frequently separated during discussions of
(16,17). Idiopathic RCM has been associated with cardiac function, diastole and systole are intricately
distal skeletal myopathy (18). Amyloidosis is the linked, with diastolic filling providing the preload
most common secondary cause of RCM (18). Radia- necessary for generation of stroke volume via the
tion heart disease and post-operative etiologies Frank-Starling mechanism. This intimate relationship
represent an increasing proportion of RCM in the is exemplified at the molecular level by the depen-
developed countries. Non-eosinophilic endomyo- dence of both cardiac contraction and relaxation on
cardial disease (endomyocardial fibrosis) is an calcium cycling (24). Diastolic filling is dependent
important cause of RCM in tropical countries of upon factors extrinsic to the cardiac chamber (the
South America (Brazil and Venezuela) and Africa loading conditions imposed upon the heart, pericar-
(Mozambique, Uganda, and others) (19–21). dial restraint, chest geometry) and intrinsic myocar-
Numerous other secondary etiologies of RCM have dial properties, such as viscoelastic forces,
been identified: eosinophilic endomyocardial dis- myocardial stiffness, and stress–strain relationships.
ease, storage diseases (e.g., hemochromatosis, Although ventricular diastole is a complex
glycogen storage diseases, Fabry disease), sarcoid- sequence of interrelated events, it can be divided into
osis, scleroderma, carcinoid, medication-induced 3 components: ventricular relaxation, passive filling,
(serotonin, ergotamines), and chemotherapy- and atrial contraction. Sarcomere inactivation and
induced (anthracyclines) (14,22). Shared among calcium cycling, the load on the LV, and nonunifor-
these disorders are altered chamber wall mechanical mity of ventricular relaxation influence global ven-
properties with reduced compliance, resulting in a tricular relaxation (25). Early rapid filling occurs due
rise in mean circulatory filling pressures and to a combination of ventricular relaxation, the driving
JACC VOL. 68, NO. 21, 2016
NOVEMBER 29, 2016:2329–47
F I G U R E 1 RA Pressure Tracings in CP and RCM
Constrictive pericarditis
100 mm Hg
LV
50 mm Hg
RA
* x y y
0 mm Hg
(Left) Left ventricular (LV) (blue) and right atrial pressure (RA) (orange) hemodynamic pressure tracings in constrictive pericarditis (CP).
Prominent “x” and “y” descents are present with a square root sign (*). (Right) LV and RA pressure hemodynamic pressure tracings in restrictive
cardiomyopathy (RCM). A prominent “y” descent is present, but the “x” descent is blunted.
pressure across the mitral valve from elevated left
atrial (LA) pressure, pericardial restraint,
myocardial stiffness. Passive filling occurs as the
result of continued ventricular relaxation and effec-
tive operating chamber compliance, which is the sum
total of passive filling dependent upon pericardial
restraint, ventricular interaction, and viscoelastic
forces of the myocardium (25). Atrial contraction
serves to “prime” the ventricle by actively distending
the chamber via atrial mechanical emptying.
The pericardial sac encompasses both ventricles,
the right atrium (RA), and most of the LA, whereas the
pulmonary venous system and the majority of the su-
perior and inferior vena cavae are external to the
pericardium. Most of the superior and inferior vena
cavae are not intrathoracic, and thus are largely un-
affected by swings in intrathoracic pressure. During
inspiration, diaphragmatic descent results
decrease in intrathoracic pressure of 5 to 10 mm Hg,
which is fully transmitted to the cardiac chambers (26).
Given no change in systemic venous pressure, the drop
in intrathoracic pressure augments right heart filling.
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 signifi-
cantly alter during respiration. In the presence of a
normal pericardium and a compliant right ventricle
(RV), chamber distention caused by increased flow is
easily accommodated, and therefore does not result in
Geske et al. 2331
Constriction vs. Restriction: Complex Cardiovascular Hemodynamics
Restrictive cardiomyopathy
LV
RA
significant inspiratory rise in pressures. Although
and right- and left-sided diastolic pressures vary inde-
pendent of one another during respiration, systolic
pressures parallel one another, falling with inspiration
and rising with expiration, mirroring intrathoracic
pressures (27). During expiration, right-sided filling
decreases relative to inspiration, whereas left-heart
filling remains relatively constant.
HEMODYNAMICS OF CONSTRICTION. The primary
hemodynamic consequence of constriction is limita-
tion of the total volume of blood that can be accom-
modated by the heart during diastole across the
respiratory cycle, with equalization of right- and left-
sided cardiac filling pressures. Accentuated early
rapid ventricular filling occurs due to high atrial
driving pressures and unimpeded ventricular relaxa-
in a tion, 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. Reduced peri-
cardial compliance limits myocardial stretch during
diastole. Although diastolic pressures are high, there
is a paradoxically low stroke volume from low pre-
load. Preserved atrial relaxation, as well as an exag-
gerated ventricular longitudinal contraction, result in
an exaggerated “x” descent on atrial pressure tracings
(Figure 1).
Rigid cardiac encasement has the additional effect
of isolation of the cardiac chambers from swings in
2332 Geske et al. JACC VOL. 68, NO. 21, 2016

Constriction vs. Restriction: Complex Cardiovascular Hemodynamics NOVEMBER 29, 2016:2329–47

F I G U R E 2 Simultaneous RV and LV Hemodynamic Assessment in CP and RCM

Exp
100 mm Hg

Insp

Constrictive pericarditis LV

50 mm Hg

RV

100 mm Hg
Insp Exp

Restrictive cardiomyopathy LV

50 mm Hg

RV

(Top) Left ventricular (LV) (blue) and right ventricular (RV) (orange) hemodynamic pressure tracings in constrictive pericarditis. End-diastolic
filling pressures are elevated and 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 inspiration
(Insp) and expiration (Exp). During inspiration, there is an increase in the area of the RV pressure curve and decrease in the area of the LV
pressure curve. (Bottom) 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.

intrathoracic pressure. With inspiration, there is a
reduction in intrathoracic pressures not fully trans-
mitted to the cardiac chambers. Because the pulmo-
nary veins are intrathoracic, inspiratory reduction in
pulmonary capillary and venous pressures reduces
the flow between the pulmonary veins and left-sided
cardiac chambers. In the setting of a noncompliant
pericardium with a relatively fixed intrapericardial
volume, reduced LV filling allows increased RV
filling. This is accompanied by inspiratory interven-
tricular septal motion towards the LV. Increased
inferior vena cava flow during inspiration,
augmented by increased transdiaphragmatic pres-
sure, competes with flow from the superior vena cava
into the high-pressure RA. The resultant increase in
jugular venous pressure with inspiration is termed
Kussmaul’s sign (28). The converse is seen in expi-
ration. With expiration, there is a rise in intrathoracic
JACC VOL. 68, NO. 21, 2016
NOVEMBER 29, 2016:2329–47
(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,
reducing RV filling, and creating expiratory diastolic
flow reversals transmitted back to the inferior vena
cava and hepatic veins. This respirophasic hemody-
namic augmentation is an important and specific
feature of constrictive physiology. Increased ven-
tricular interdependence directly translates to an
alteration in ventricular systolic pressures. Although
these pressures rise and fall in parallel with respira-
tion in normal physiology, systolic pressures become
discordant in CP, a marker that is both sensitive and
specific (Figure 2) (29). Loss of pericardial compliance
amplifies the effect of adjacent cardiac chambers,
with resultant equalization of intracardiac diastolic
pressures.
HEMODYNAMICS OF RESTRICTION. Unlike the com-
plex 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
(30,31). 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. Unlike CP,
the “x” descent is frequently blunted, given poor
atrial relaxation and a limited descent of the annulus
towards the apex (Figure 1). Increased venous flow
with inspiration is unable to be accommodated by a
noncompliant RV; hence, there are diastolic flow re-
versals in the hepatic vein with inspiration. Unlike
CP, there is no discordance of intracavitary and
intrathoracic pressures (Figure 2).
DIAGNOSTIC EVALUATION
PHYSICAL EXAMINATION. Equalization of intracar-
diac pressures in CP results in predominantly sys-
temic venous congestion, manifested as edema,
hepatomegaly, and ascites. As previously noted,
elevated jugular venous pressures that increase with
inspiration (Kussmaul’s sign) are usually present.
Recognition of the characteristic jugular venous
contour with prominent “x” and “y” descents is a
critical portion of the physical examination (Figure 1).
It should be noted that the “x” descent is lost in pa-
tients with atrial fibrillation, even in the presence
Geske et al. 2333
Constriction vs. Restriction: Complex Cardiovascular Hemodynamics
of CP. Robust early ventricular filling accompanying
the “y” descent with sudden deceleration results in
an early diastolic, high-pitched pericardial knock,
although this is an insensitive sign.
As with CP, 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 (32,33). 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 (Figure 1).
ELECTROCARDIOGRAM AND LABORATORY TESTING.
Electrocardiographic findings are overall nonspecific
for differentiating CP and RCM, although differences
in P-wave morphology, QRS voltage, and presence of
conduction abnormalities may be present, depen-
dent upon the underlying substrate. In CP, pericar-
dial inflammation and fibrosis can extend to the
atrial wall, resulting in an intra-atrial conduction
delay and a wide, notched, low-amplitude P-wave
(34). Conversely, RCM is characterized by severe
biatrial enlargement, which translates to electrocar-
diographic findings of wide, increased amplitude P
waves (34). Atrial fibrillation is a complication of
both CP and RCM, and likely represents electro-
physiological sequelae of atrial enlargement and
perhaps inflammation. Discordance between QRS
voltage and wall thickness should trigger clinical
concern for cardiac amyloidosis. Ventricular con-
duction abnormalities (bundle branch block) are
present in 20% to 30% of RCM, likely reflecting the
influence of infiltrative pathologies, but are uncom-
mon in CP (34).
In the setting of effusive CP, cytokine assessment
can be additive in determining the underlying cause,
with serum interleukin-10 levels significantly higher
in patients with underlying tuberculosis (35). Tar-
geted laboratory testing to assess for various sec-
ondary causes of RCM should be performed on an
individualized basis. It is reasonable to assess for
monoclonal paraproteinemia and paraproteinuria to
evaluate for amyloidosis, although this test alone is
not sufficient to confirm amyloidosis (36). Additional
testing, including evaluation for connective tissue
diseases or infiltrative diseases, should be guided by
clinical history and imaging assessment.
B-type natriuretic peptide (BNP) is a polypeptide
neurohormone released by the myocardium in
response to stretch. Although both CP and RCM result
in elevated filling pressures, patients with RCM
2334 Geske et al.
Constriction vs. Restriction: Complex Cardiovascular Hemodynamics
F I G U R E 3 M-Mode of the Interventricular Septum in CP
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 (CP).
develop massive biatrial enlargement. In keeping
with this, BNP tends to be higher in patients with
RCM (>600 pg/ml) compared with CP (>200 pg/ml)
(37,38). However, significant overlap is present,
limiting the clinical utility of BNP in differentiating
the 2 conditions (39). The difference in BNP between
patients with RCM and CP is lessened in the setting of
renal insufficiency (37,40).
ECHOCARDIOGRAPHY. 2-dimensional echocardiography.
Echocardiography is the initial imaging test of choice
in patients with signs and symptoms of constriction
or restriction. Increased pericardial thickness can
be recognized on transthoracic echocardiography,
although interpretation is often challenging (41).
Pericardial thickness >3 mm on transesophageal
echocardiography is both sensitive and specific for
the detection of a thickened pericardium (42).
Adherence of the visceral and parietal pericardium
can result in tethering, a finding often appreciated
on the RV free wall from the subcostal or apical
4-chamber views (43). Identification of a calcified
pericardium with ventricular contour distortion is
infrequent, but strongly suggests CP (43). Pericardial
calcification is much better appreciated via chest
x-ray or computed tomography (CT). Presence of a
pericardial effusion may signify the presence of
effusive–constrictive physiology.
Assessment of ventricular septal motion on both
M-mode and 2-dimensional echocardiography can
provide insight into ventricular interdependence
with inspiratory leftward motion of the septum and
expiratory rightward shift (Figure 3). Respirophasic
JACC VOL. 68, NO. 21, 2016
NOVEMBER 29, 2016:2329–47
ventricular septal shifting is usually the first echo-
cardiographic 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, trans-
lating to a septal notch on M-mode imaging (Figure 3).
The mechanism of this “bounce” is a combination
of differential timing of filling and pericardial
constraint, leading to rapid cessation of filling (44).
Systemic venous congestion is present in both RCM
and CP. A plethoric inferior vena cava and engorged
hepatic veins are expected in both conditions.
Absence of a dilated inferior vena cava in a patient
without recent diuresis should call into question the
diagnosis of hemodynamically significant CP or RCM.
Cavity size and ventricular wall thickness tend to
be normal in primary (idiopathic) RCM (18). Severe
atrial enlargement is often present (Figure 4).
Although hemodynamics are similar in primary and
secondary (infiltrative) forms of RCM, ventricular
wall thickness is commonly increased in infiltrative
diseases (22). Unfortunately, many infiltrative car-
diomyopathies can have a similar appearance on
2-dimensional echocardiography (45). Further
assessment via serologic evaluation, strain, tissue
characterization at cardiac magnetic resonance (CMR)
imaging, or endomyocardial biopsy (EMB) may be
needed to clarify further.
D o p p l e r e c h o c a r d i o g r a p h y . Detailed Doppler he-
modynamic evaluation is central to the diagnosis of
both CP and RCM (46), and may be sufficient to
JACC VOL. 68, NO. 21, 2016 Geske et al. 2335
NOVEMBER 29, 2016:2329–47 Constriction vs. Restriction: Complex Cardiovascular Hemodynamics

F I G U R E 4 Echocardiographic Findings in RCM

(Upper left) 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. (Upper right) Hepatic vein pulsed-wave Doppler shows increased inspiratory forward velocities (arrow), inspiratory diastolic flow reversals
(arrowhead), and minimal expiratory diastolic flow reversals (rounded arrow). (Lower left) Lateral mitral annulus tissue Doppler demonstrates markedly reduced early
diastolic velocity (e0 ). (Lower right) Apical 4-chamber 2-dimensional echocardiography reveals severe biatrial enlargement. RCM ¼ restrictive cardiomyopathy.

confirm CP without hemodynamic catheterization in
in many patients. Mitral (and tricuspid) Doppler
inflow patterns in both CP and RCM are early diastolic
velocity (E-wave) predominant with a short deceler-
ation time, reflecting the predominance of early rapid
ventricular filling. A critical difference is the presence
of respiratory flow variation in CP (Figure 5), which is
absent in RCM (Figure 4) (3,4,6,47,48). Reportedly,
mitral inflow in CP demonstrates a respiratory varia-
tion of $25%, with increased velocities during expi-
ration (49). The initial observation of mitral inflow
respiratory variation in CP was on the basis of 7 pa-
tients (49), and subsequent larger studies have shown
the absence of respiratory variation in one-third of
patients with CP (50,51). Therefore, the lack of respi-
ratory variation should not exclude the diagnosis of
CP. Doppler respirophasic variation is similarly seen
in the pulmonary veins, with peak diastolic flow
>18% variation suggestive of CP (6). 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 interro-
gation in CP shows decreased expiratory diastolic
hepatic vein forward velocities with large expiratory
diastolic reversals. The presence of atrial fibrillation
results in variable cardiac cycle length and accom-
panying alteration of mitral inflow velocities.
Although this makes evaluation for CP via mitral
inflow Doppler challenging, hepatic vein flow re-
versals and annular tissue Doppler assessment
remain reliable metrics for assessment of CP (43).
Mitral and tricuspid inflow patterns in patients
with chronic obstructive pulmonary disease can
mimic CP because of increased changes in intratho-
racic pressure. Superior vena cava Doppler assess-
ment is warranted in all patients with significant
respiratory disease undergoing evaluation for CP.
Chronic obstructive pulmonary disease results in a
marked increase in superior vena cava systolic
forward flow velocity with inspiration, whereas pa-
tients with CP do not demonstrate such respiratory
variation (52).
Of all echocardiographic parameters, perhaps the
most useful to distinguish CP and RCM is mitral
annular tissue Doppler assessment. The early dia-
stolic velocity of the mitral annulus (e 0 ) sampled via
2336 Geske et al. JACC VOL. 68, NO. 21, 2016

Constriction vs. Restriction: Complex Cardiovascular Hemodynamics NOVEMBER 29, 2016:2329–47

F I G U R E 5 Doppler Findings in CP

(Upper left) Pulsed-wave Doppler of the mitral inflow shows >25% expiratory increase in velocities (arrows). (Upper right) Hepatic vein pulsed-wave Doppler shows
decreased expiratory forward velocities and large expiratory diastolic flow reversals (arrowhead). (Lower left) Medial mitral annulus tissue Doppler demonstrates
elevated early diastolic velocities (e0 ), despite increased filling pressures (annulus paradoxus). (Lower right) Lateral mitral annulus e0 is decreased relative to the medial
annulus (annulus reversus) due to lateral tethering. CP ¼ constrictive pericarditis.

tissue Doppler reflects the status of LV myocardial mitral inflow E-wave and the mitral annular e 0 ve-
relaxation, with good inverse correlation with the locity is routinely used in patients with myocardial
time constant of relaxation, tau (53). Under normal diseases for diastolic function assessment, because it
conditions, the medial e 0 velocity is lower than the provides better estimates of LV filling pressures than
lateral e 0 velocity, given the relative anatomic teth- other methods, such as pulmonary vein assessment
ering of the ventricular septum, which is influenced (55,56).
by relaxation of both ventricles (54). The ratio of the As myocardial stiffening occurs and relaxation be-
comes delayed, e 0 velocities become reduced, a hall-
mark of RCM (57) (Figure 4). In CP, the mechanism of
T A B L E 1 Echocardiographic Characteristics of Surgically Confirmed CP
increased filling pressures is not at the level of the
Criterion Sensitivity Specificity PPV NPV myocardium or due to reduced myocardial relaxation.
1. Ventricular septal shift 93 69 92 74 Lateral cardiac motion is limited, due to pericardial
2. Change in mitral E velocity >14.6% 84 73 92 55
constriction; hence, ventricular filling depends upon
3. Medial e0 velocity >9 cm/s 83 81 94 57
longitudinal cardiac motion. Consequently, mitral
4. Medial e0 /lateral e0 $0.91 75 85 95 50
5. Hepatic vein diastolic reversal velocity/ 76 88 96 49
annular tissue Doppler e 0 velocities are normal or
forward velocity in expiration $0.79 paradoxically increased despite increased filling
1 and 3 80 92 97 56 pressures, termed “annulus paradoxus” (58). In CP,
1 with 3 or 5 87 91 97 65 tethering of the LV free wall can result in reversal of
1 with 3 and 5 64 97 99 42
the relationship between medial and lateral mitral
Values are %. Reprinted with permission from Welch et al. (43).
annular tissue Doppler velocities, such that the
CP ¼ constrictive pericarditis; E ¼ early diastolic mitral inflow Doppler velocity; e0 ¼ early medial e 0 is higher (typically >7 cm/s) than lateral e 0 , a
diastolic mitral annular tissue Doppler velocity; NPV ¼ negative predictive value; PPV ¼ positive
predictive value.
phenomenon referred to as “annulus reversus”
(Figure 5) (59).
JACC VOL. 68, NO. 21, 2016
NOVEMBER 29, 2016:2329–47
We favor a multifaceted approach to echocardio-
graphic evaluation of CP, utilizing the Mayo Clinic
criteria such as interventricular septal motion, mitral
inflow velocity pattern, mitral annulus e 0 velocity,
and hepatic vein diastolic flow reversals with expi-
ration (43) (Table 1).
Newer echocardiographic t e c h n i q u e s . Novel
echocardiographic techniques of speckle-tracking
strain and 3-dimensional (3D) echocardiography
have supplemented the collective understanding of
both CP and RCM (60). Three-dimensional echocar-
diographic imaging can provide additive information,
specifically in regard to pericardial visualization (61),
pericardial effusion assessment (62), and pericardial
tethering (62,63). In RCM, such as amyloidosis, 3D
echocardiography allows for determination of tem-
poral and regional characterization of LV dyssyn-
chrony (64,65). Myocardial deformation (LV strain)
may be useful in the future, because preliminary
data have shown that patients with CP have mark-
edly abnormal circumferential deformation, torsion,
and untwisting velocity, but relative sparing of
longitudinal mechanics, whereas RCM is associated
with abnormal longitudinal mechanics, most pro-
nounced at the base, with relative sparing of LV
rotation (66). Characteristic strain “fingerprints”
have been identified for various infiltrative car-
diomyopathies, such as relative apical longitudinal
strain preservation in patients with cardiac amyloid-
osis (67,68). Because the use of 3D strain continues to
expand, further patterns and mechanistic insights
may emerge (69). At this juncture, these techniques,
although promising, remain investigational, and
require further validation in larger studies before be-
ing incorporated into a routine diagnostic imaging
strategy.
CHEST X-RAY AND CARDIAC CT. Chest radiography
may demonstrate calcification of the pericardium,
best assessed on the lateral view, but this finding is
only seen in approximately one-quarter of patients
with CP (Figure 6) (70,71). Because calcification is a
result of chronic inflammation, it is not typically
present in patients with subacute pericarditis, which
may include effusive CP (34). Findings of pulmonary
venous congestion, pleural effusions, or massive
biatrial enlargement are more suggestive of RCM (18).
Chest CT has largely replaced chest x-ray as the
radiographic modality of choice for characterizing
pericardial anatomy, given its excellent spatial reso-
lution and 3D assessment of anatomy (Figure 7) (72).
Notably, pericardial calcification not recognized on
chest x-ray is frequently identified on CT (71). Normal
pericardial thickness by CT is <2 mm (73). Although a
Geske et al. 2337
Constriction vs. Restriction: Complex Cardiovascular Hemodynamics
pathologically thickened pericardium (>4 mm) is
highly suggestive of CP, lack of this finding should
not be used in isolation to exclude the diagnosis,
because pericardial thickness is normal in 20% of
patients with CP (74). In addition to defining peri-
cardial anatomy, gated cardiac CT provides excellent
assessment of pericardial effusion size, cardiac
chamber size, deformation of the cardiac contour,
and pertinent noncardiac findings, such as hepatic
congestion, dilation of the inferior vena cava, and
pulmonary vascular congestion. Reduced lateral
mitral annular early diastolic tissue Doppler veloc-
ities correlate well with pericardial thickness on CT in
patients with CP (75). Beyond anatomic definition,
respirophasic septal motion in CP is well seen on the
gated acquisition (76).
CARDIAC MAGNETIC RESONANCE. CMR provides
excellent assessment of the pericardium and cardiac
chambers independent of chest geometry, and
has emerged as a powerful diagnostic study in the
evaluation of pericardial and myocardial diseases
(77,78). Both CP and RCM present with significant
biatrial enlargement. The relative atrial ratio (ratio
of LA volume to RA volume) is greater in patients
with CP than RCM when assessed via CMR, with a
ratio of 1.32 providing the highest specificity and
sensitivity (79). Beyond cardiac anatomy, CP has
been associated with increased liver stiffness, as
evaluated via magnetic resonance elastography,
likely secondary to chronic hepatic congestion or
fibrosis (80).
CMR has excellent accuracy (93%) for detection of
pericardial thickening >4 mm (77). Fast spin-echo T2-
weighted sequences, especially with fat saturation,
and short-tau inversion-recovery sequences are use-
ful to visualize pericardial edema and inflammation
(81). CMR affords insight into the presence and extent
of pericardial inflammation via delayed gadolinium
enhancement, as well as defining the size and loca-
tion of pericardial effusion in effusive CP (Figure 8)
(82). Interpretation of post-contrast pericardial
enhancement abnormalities can be challenging in
isolation, as delayed enhancement may represent a
spectrum of pathologies from inflammation to
fibrosis. Combination of post-contrast data with
edema-sensitive sequences provides further imaging
insight into the presence of active inflammation. The
presence of abnormal pericardial delayed enhance-
ment in the setting of pericardial constriction may
identify those patients who may benefit from a
trial of aggressive anti-inflammatory therapy (83).
Improvement in late gadolinium enhancement par-
allels inflammatory markers in reversible CP treated
2338 Geske et al. JACC VOL. 68, NO. 21, 2016

Constriction vs. Restriction: Complex Cardiovascular Hemodynamics NOVEMBER 29, 2016:2329–47

F I G U R E 6 Chest Radiograph in CP

(Left) Anterior-posterior chest radiograph demonstrates pericardial calcification (arrowheads) along the right atrial border and inferior margin
of the heart. (Right) Lateral radiograph demonstrates extensive anterior pericardial calcification. CP ¼ constrictive pericarditis.

with anti-inflammatory agents (83).
follow-up recommendations to assess improvement
of these imaging findings with medical therapy are
based upon anecdotal experience.
Myocardial delayed enhancement, indicative of
myocardial inflammation or fibrosis, is classically
absent in CP. In myopericarditis, there is extension of
pericardial delayed enhancement to the myocardium,
which has been associated with worse outcomes
compared with patients without myocardial involve-
ment (84). Myocardial delayed enhancement is seen
in nearly one-third of cases of RCM (79). Patterns
of myocardial delayed enhancement and other
Currently, cine-imaging characteristics may help to identify
infiltrative cardiomyopathies resulting in RCM (85).
For example, in cardiac amyloidosis, global or sub-
endocardial delayed myocardial enhancement is
frequently present, with a characteristic inability to
adequately null the myocardium (86). Furthermore,
delayed gadolinium enhancement provides incre-
mental prognostic information over serum bio-
markers in immunoglobulin light-chain cardiac
amyloidosis (87).
CMR avoids the radiation exposure associated with
cardiac CT. This allows real-time, free-breathing im-
age acquisition with CMR, facilitating respirophasic

F I G U R E 7 Pericardial Assessment on CT

(Left) Anatomic location and extent of pericardial calcification (arrowheads) demonstrated on axial noncontrast computed tomography (CT) in
a patient with constrictive pericarditis. (Right) Thickened pericardium (>4 mm) in a patient with constrictive pericarditis, with deformation of
the cardiac contour in the absence of pericardial calcification.
JACC VOL. 68, NO. 21, 2016
NOVEMBER 29, 2016:2329–47
F I G U R E 8 CMR Imaging Delayed Gadolinium Enhancement of the Pericardium
(Left) Axial delayed enhancement images demonstrate pericardial enhancement (arrowheads) in the setting of fibrotic constrictive pericarditis. (Right) Delayed
enhancement of the visceral (arrowheads) and parietal pericardium (arrows) in a patient with effusive constrictive pericarditis and circumferential pericardial effusion.
CMR ¼ cardiac magnetic resonance.
assessment of ventricular septal motion and ventric-
ular coupling (Online Video 1). Ventricular septal
excursion, defined as the maximal difference in
expiratory and inspiratory position of the ventricular
septum, normalized to biventricular diameter, has
been found to discriminate between CP and RCM,
with a cutoff value of 11.8% (88). Similarly, in patients
with CP, the ratio of biventricular end-diastolic area
at end-inspiration to biventricular end-diastolic area
at end-expiration remains close to 1 (1.03  0.03),
whereas the ratio was greater in patients without
pericardial constraint and ventricular interdepen-
dence (1.28  0.10) (89).
Tagged cine CMR is a noncontrast technique for
myocardial labeling and regional displacement
assessment, analogous to echocardiographic mea-
sures of strain. Cine CMR has been used to assess CP,
as it may allow better recognition of tissue tethering,
or “lack of slippage” (90,91). Adherence of the
visceral and parietal pericardium can result in
persistent concordance of tagged signals between the
tissue planes throughout both systole and diastole.
Tagging and cine CMR have been used to further
understand myocardial mechanics in cardiomyopa-
thies, such as Duchenne muscular dystrophy (92,93),
and such techniques may also benefit RCM
Geske et al. 2339
Constriction vs. Restriction: Complex Cardiovascular Hemodynamics
evaluation. CMR and echocardiographic tissue
tracking-derived left ventricular mechanics provide
comparable diagnostic information for differentiating
CP from RCM (94).
NUCLEAR IMAGING. Positron emission tomography
(PET) utilizing 18-fluorodeoxyglucose (FDG) provides
unique insight into in vivo metabolism, with
increased uptake useful for identification of inflam-
mation or neoplasm. Recognition of normal, physio-
logical patterns of myocardial and pericardial FDG
uptake is an important first step in isolating patho-
physiology (95). PET imaging provides novel meta-
bolic information not obtained via anatomic imaging,
such as CMR or CT; however, these data are not
exclusive to the modality, and can be coupled with
anatomic imaging for further cardiac and pericardial
tissue characterization (81,96). In combination with
cardiac CT, FDG-PET has been utilized to assess
pericardial inflammation in various infectious and
inflammatory causes of pericarditis (96–100), and
may be helpful in differentiating between different
etiologies (101).
99m
Technetium (Tc)-pyrophosphate scintigraphy
has emerged as a clinical tool for differentiating both
familial and wild-type transthyretin-type cardiac
2340 Geske et al.
Constriction vs. Restriction: Complex Cardiovascular Hemodynamics
amyloidosis from other forms (102–104). Patients
with familial transthyretin-type cardiac amyloidosis
have significantly higher quantitative and semi-
quantitative cardiac visual scores than patients with
light-chain cardiac amyloidosis (103). The role of
99m
Tc-pyrophosphate scintigraphy or FDG-PET in
other forms of RCM remains largely unknown.
HEMODYNAMIC CATHETERIZATION. Hemodynamic
catheterization has served as the gold standard for
the diagnosis of CP and RCM. In both diseases, cath-
eterization demonstrates early rapid diastolic filling,
with elevation and equalization of end-diastolic
pressures. In some patients who have undergone
aggressive diuresis, filling pressures can be low to
normal, but these patients will have a low cardiac
output due to the abnormal diastolic pressure volume
relationships. Thus, for either CP or RCM to be pre-
sent, there needs to be elevation of diastolic pres-
sures, a low cardiac output, or both. In patients who
have low-to-normal filling pressures, a fluid challenge
is required.
Differentiation of CP from RCM is a diagnostic
challenge in patients who have early rapid ventric-
ular filling with elevation and near equalization
of diastolic pressures. In the past, several crite-
ria have helped to differentiate between CP and
RCM (1,105).
 Elevations of left-sided filling pressures are better
tolerated in CP than right-sided elevations, ac-
counting for the predominance of right-sided fail-
ure symptoms and less pulmonary hypertension in
CP (pulmonary arterial systolic pressure <55 mm Hg)
(29). Although the presence of pulmonary hyper-
tension favors restrictive RCM, one-third of patients
with surgically proven CP have pulmonary hyper-
tension (106).
 After brisk early filling, ventricular pressure rises
rapidly as the pericardial constraining volume is
reached, resulting in a “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 (29).
 Kussmaul’s sign, quantified as <5 mm Hg decrease
in inspiratory RA pressure, is often present in both
CP and RCM (29).
 Disproportionate abnormalities of
dysfunction result in a ratio of RV end-diastolic
pressure to systolic pressure >1:3 in CP (107).
 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 (47).
JACC VOL. 68, NO. 21, 2016
NOVEMBER 29, 2016:2329–47
However, these criteria have been shown to have
poor sensitivity and specificity in differentiating be-
tween CP and RCM (Figure 9). In addition, any disease
in which there is an overload of the RV can result in
similar pressure abnormalities, such as severe
tricuspid regurgitation or RV infarction.
Respirophasic variation in pressures is the most
helpful hemodynamic catheterization finding in
differentiating between CP and RCM. Careful hemo-
dynamic assessment at catheterization allows for
exquisitely detailed assessment of dissociation of
intrathoracic and intracavitary pressures, as well as
determination of enhanced ventricular interdepen-
dence. 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 (48). Initial investigations of
respirophasic ventricular interdependence in CP
focused on comparison of RV and LV peak systolic
pressures as a surrogate of stroke volume (47). Sub-
sequent investigations have found that area under
the systolic pressure curves is a better determinant of
beat-to-beat stroke volume (29). Use of the systolic
area index (defined as the ratio of the RV area to LV
area in inspiration vs. expiration) yields a much more
specific and sensitive measure than use of systolic
pressures (cutoff >1.1, 97% sensitive, 100% specific)
(Figure 2) (29). In CP, respirophasic swings in stroke
volume occur because of the fixed pericardial volume,
with enhanced ventricular interdependence. Recog-
nition of this finding and dissociation of intracardiac
and intrathoracic pressures remains paramount to
identification of CP.
ENDOMYOCARDIAL BIOPSY. The role of EMB in
differentiating CP from RCM is poorly defined. EMB
has the potential to diagnose forms of RCM with tar-
geted therapies available (cardiac sarcoidosis,
amyloidosis, and Fabry disease). In some cases, EMB
can aid in prognostication, such as differentiation of
subtypes of amyloidosis (108). EMB may be reason-
able in patients with suspected RCM of uncertain
etiology. A recent series by Bennett et al. (109) noted
that EMB was diagnostic in 29% of 281 patients with
heart failure associated with unexplained RCM. A
diastolic consensus document from the Association for Euro-
pean Cardiovascular Pathology and the Society for
Cardiovascular Pathology provides further diagnostic
guidance, dependent upon suspected underlying pa-
thology (110). Although not proven, a completely
negative biopsy may favor constriction, particularly
in radiation heart disease.
JACC VOL. 68, NO. 21, 2016 Geske et al. 2341
NOVEMBER 29, 2016:2329–47 Constriction vs. Restriction: Complex Cardiovascular Hemodynamics

F I G U R E 9 Invasive Hemodynamic Assessments to Differentiate CP and RCM

30 1.0
LVEDP – RVEDP (mm Hg)

20 0.8

RVEDP/RVESP
0.6
10
0.4
0
0.2

-10 0.0
CP RCM CP RCM
100 mm Hg

LV

50 mm Hg

RVEDP / RVESP
= 0.38 RV
LVEDP - RVEDP
= 4 mm Hg

0 mm Hg

There have been a number of criteria proposed comparing left ventricular (LV) and right ventricular (RV) pressures at the time of cardiac
catheterization in order to differentiate constrictive pericarditis (CP) from restrictive cardiomyopathy (RCM). These include: 1) the difference of
left ventricular end-diastolic pressure (LVEDP) and right ventricular end-diastolic pressure (RVEDP); and 2) the ratio of RVEDP to right ven-
tricular end-systolic pressure (RVESP). Although there is a statistical difference when comparing the means of these criteria in a number of
patients, the degree of overlap makes application to an individual case difficult. Adapted with permission from Talreja et al. (29).

SUMMATIVE APPROACH TO DIAGNOSIS
Diagnosis of CP and RCM requires a multifaceted
approach, as outlined herein in detail. Understanding
the multimodality imaging features of both entities is
a crucial step (Figure 10). An organized approach to
recognition of historical, examination, imaging, and
hemodynamic findings of both CP and RCM should
guide evaluation (Central Illustration).
CLINICAL IMPACT OF
DIAGNOSIS/TREATMENT
Although clinical and diagnostic features of CP and
RCM often overlap, management differs significantly.
CP represents a reversible cause of heart failure, with
pericardiectomy potentially
frequently, RCM has few treatment options. Recog-
nition of the primary underlying disease process is
critical, given these therapeutic differences.
Diuresis is the mainstay of pharmacotherapy in CP,
and may be sufficient to control mild symptoms.
A subset of the patients with relatively recent onset
(<3 months) of CP can be managed with nonsteroidal
anti-inflammatory agents or steroids if there is
ongoing marked pericardial inflammation, as evi-
denced by elevated inflammatory biomarkers and
intense delayed enhancement on CMR (84). In sub-
acute CP, such as effusive CP, spontaneous resolution
or resolution with anti-inflammatory agents has been
demonstrated (83,111,112). In one series, effusive CP
was present in 17% of cases, and it may be reasonable
to observe or treat with anti-inflammatory agents for
a period of 3 months, dependent upon the clinical
scenario (112). Anti-inflammatory agents may be
nonspecific or specifically targeted, such as antitu-
bercular therapies. Treatment of tubercular pericar-
ditis remains a challenge, with neither prednisolone
curative, whereas nor Mycobacterium indicus pranii immunotherapy
demonstrating a significant effect on a composite
endpoint of death, cardiac tamponade requiring
pericardiocentesis, or progression to CP (113).
Further consideration of strategies such as
angiotensin-converting enzyme inhibitor therapy and
2342 Geske et al. JACC VOL. 68, NO. 21, 2016

Constriction vs. Restriction: Complex Cardiovascular Hemodynamics NOVEMBER 29, 2016:2329–47

F I G U R E 1 0 Multimodality Approach to Differentiating CP and RCM

Advanced cardiovascular imaging

Echocardiography

Medial e’ Medial e’ to Transmitral Septal Hepatic

velocity lateral e’ ratio inflow shift veins

Decreased Increased
Increased forward velocity + forward velocity +
Increased Decreased Increased Decreased respiratory Normal increased increased
variation expiratory diastolic inspiratory diastolic
reversals reversals

Favors Favors Favors Favors Favors Favors Favors Favors

Constriction Restriction Constriction Restriction Constriction Restriction Constriction Restriction

Cardiac CT Cardiac MRI

Abnormal Abnormal
Increase Cardiac Increased Pericardial Increased Severe Abnormal
Severe biatrial Septal myocardial myocardial
pericardial contour pericardial inflammation/ respirophasic biatrial wall
enlargement bounce delayed nulling
thickness deformation thickness fibrosis septal shift enlargement thickness
enhancement amyloidosis

Favors Favors Favors Favors Favors Favors Favors Favors Favors Favors Favors
Constriction Constriction Restriction Constriction Constriction Constriction Constriction Restriction Restriction Restriction Restriction

Imaging features of echocardiography, cardiac CT, and CMR to distinguish constriction and restriction (Online Video 1). Abbreviations as in Figures 1, 7, and 8.

intrapericardial fibrinolytic agents for preventing
tuberculous CP has been proposed (114).
The gold standard for treatment of CP remains
complete pericardiectomy (12,15,115).
cardiectomy results in significant improvement in
New York Heart Association (NYHA) functional class
and filling pressures (12,115,116). Timing of surgery is
tailored to the patient; however, earlier surgical
intervention may prevent ventricular dysfunction,
thereby avoiding myocardial atrophy and post-
operative ventricular dilation (117). Numerous oper-
ative approaches have been described (118), with
some favoring median sternotomy for exposure,
whereas others prefer left anterolateral thoracotomy
to avoid sternal infection (115,119). Data from large
series would suggest no clear benefit associated with
specific surgical approaches (15). Despite this, there is
significant evidence for the benefit of complete peri-
cardiectomy over partial pericardiectomy or pericar-
dial window, namely lower perioperative mortality,
less post-operative low-output syndrome, shorter
hospitalization stays, and improved long-term sur-
vival (15).
Complete pericardiectomy refers to wide excision
of the pericardium anteriorly between the 2 phrenic
nerves, and from the great arteries superiorly to the
Peri- diaphragm inferiorly, posterior to the left phrenic
nerve (which remains on a pedicle) to the left pul-
monary veins, including the pericardium on the dia-
phragmatic and posterior surfaces of the ventricles,
with constricting layers of epicardium also removed
(12,120). During surgery, the previously strangled
heart can be seen to “jump” out of the constraining
pericardium. Complete surgical excision of the peri-
cardium may be technically challenging, because
inflammation can result in dense adherence of tissue
layers; therefore, it is not uncommon for areas of the
pericardium to remain, especially in the vicinity of
the epicardial coronary vessels. This may result in
persistence of abnormal hemodynamics, albeit with
milder clinical manifestations and improved patient
tolerance. Further data are warranted comparing
concomitant surgical coronary artery bypass grafting
during a long, technically challenging cardiac surgery
versus percutaneous revascularization. The method
of revascularization is a decision that necessitates
JACC VOL. 68, NO. 21, 2016 Geske et al. 2343
NOVEMBER 29, 2016:2329–47 Constriction vs. Restriction: Complex Cardiovascular Hemodynamics

C ENTR AL I LL U STRA T I O N CP and RCM: Approach to Diagnosis

Constrictive Restrictive
pericarditis cardiomyopathy

Cardiac Genetic
procedures disease

Radiation
Radiation
Patient history Connective tissue
disease
Connective tissue
disease Infection
(eosinophilic disease)
Infection
(TB) Amyloidosis

y x y x
Jugular venous
pressure (JVP)
Kussmaul’s sign Kussmaul’s sign

Lab/ECG/X-ray

Pericardial
thickness Wall thickness

Pericardial Biatrial
Echocardiogram
enlargement
Advanced cardiac Cardiac MRI Pericardial Systolic and diastolic
imaging dysfunction
Imaging correlates of Pulmonary
Cardiac CT
ventricular interdependence hypertension
Pericardial Tissue characterization
abnormalities

Discordant respirophasic Concordant respirophasic

Invasive
ventricular pressure ventricular pressure
hemodynamics
changes changes

Geske, J.B. et al. J Am Coll Cardiol. 2016;68(21):2329–47.

Differentiation of constriction and restriction requires a multifaceted approach inclusive of history, physical examination, laboratory testing, and multimodality imaging.
Even with this toolset, hemodynamic catheterization remains necessary to provide definitive hemodynamic assessment for a subset of patients. CP ¼ constrictive
pericarditis; CT ¼ computed tomography; ECG ¼ electrocardiogram; JVP ¼ jugular venous pressure; MRI ¼ magnetic resonance imaging; RCM ¼ restrictive
cardiomyopathy; TB ¼ tuberculosis.

cardiovascular surgery expertise and individualized with advanced NYHA symptom class, underlying ra-
patient decision-making. Although pericardiectomy diation heart disease (with the best outcome seen
is largely referred to as “curative” (even herein), when the etiology is idiopathic), advanced age,
outcomes in patients post-pericardiectomy are worse female sex, impaired renal function, pericardial
2344 Geske et al. JACC VOL. 68, NO. 21, 2016

Constriction vs. Restriction: Complex Cardiovascular Hemodynamics NOVEMBER 29, 2016:2329–47

calcification, LV systolic dysfunction, and pulmonary FUTURE DIRECTIONS

hypertension (12,15,121–123). Perioperative mortality
is generally <5%, excluding patients with prior radi-
ation therapy, whose perioperative mortality may
exceed 20% (15,118,123).
Unfortunately, treatment options remain limited in
RCM. Diuretic agents may alleviate symptoms in
those with milder clinical phenotype. Infiltrative eti-
ologies may have disease-specific therapies targeted
at the underlying disease, although in many cases,
cardiac dysfunction persists, despite therapies. Pedi-
atric studies of primary RCM demonstrate relentless
progression to death or transplantation (124,125).
Ammash et al. (126) found a 5-year survival of 64%
(expected 85%) in a predominantly adult population
(mean age 64 years). Men >70 years of age with
higher NYHA functional class and LA dimension
>60 mm demonstrated the worst prognosis. Prior
series have suggested that patients with primary RCM
present earlier, but survive longer after presentation
than those with amyloidosis (127); however, interval
advances in therapy for cardiac amyloidosis may alter
these results. In symptomatic patients who are
transplant candidates, transplant evaluation is war-
ranted. Post-cardiac transplantation outcomes are
comparable to those of non-RCM patients, although
subgroup analysis suggests increased mortality for
RCM secondary to radiation or amyloidosis (128). In
patients who are not transplant candidates, palliative
measures should be pursued.
Management of mixed constriction/restriction re-
mains a challenging undertaking. Should hemody-
namic evaluation reveal a substantial component of
constrictive physiology in a patient unresponsive to
diuretic therapy, pericardiectomy may be considered,
with tempered expectations. Consistent
demonstrating worse outcomes in patients with
radiation-induced CP may indeed reflect subclinical
overlap in CP and RCM.
Further exploration of underlying inflammatory pro-
cesses and myopericardial tissue mechanics may
provide insight as to why certain patient cohorts have
a predilection for developing CP following an in-
flammatory insult, whereas others spontaneously
resolve. Although the study of CP has been extensive,
there is a distinct paucity of data on the natural his-
tory and pathophysiology of primary RCM. Given the
markedly poor prognosis and lack of treatment op-
tions apart from transplantation, there is potential for
development of a host of novel therapeutics in RCM,
spanning pharmacological therapies, biologic agents,
and mechanical interventions.
Despite the multifaceted, evolving approach to
differentiating between CP and RCM, diagnosis often
remains a clinical dilemma, with recognition an
exigent need, given the life-altering impact of timely
surgery in CP. Our understanding of the role of cardiac
CT and CMR in CP continues to evolve (46); however,
validated refinement in diagnostic schemata, akin to
the Mayo Clinic echocardiographic criteria (43), are
warranted on a multimodality level. In cases of mixed
constrictive and restrictive hemodynamics, tools to
assess the relative contribution of each component are
lacking, and even complex invasive hemodynamic
catheterization in expert hands can yield un-
certainties. The ability to discern the relative contri-
bution of each hemodynamic insult has potential to
guide therapeutic decision-making and temper oper-
ative expectations. Although decades of study have
provided a glimpse into the underpinnings of CP and
RCM, there remains much to discover.
data REPRINT REQUESTS AND CORRESPONDENCE: Dr.
Jeffrey B. Geske, Department of Cardiovascular Dis-
eases, Mayo Clinic, 200 First Street SW, Rochester,
Minnesota 55905. E-mail: geske.jeffrey@mayo.edu.

REFERENCES

1. Connolly DC, Wood EH. Cardiac catheterization
in heart failure and cardiac constriction. Trans Am
Coll Cardiol 1957;7:191–201.
2. Nishimura RA, Connolly DC, Parkin TW, et al.
Constrictive pericarditis: assessment of current
diagnostic procedures. Mayo Clin Proc 1985;60:
397–401.
from restrictive cardiomyopathy by Doppler
transesophageal echocardiographic measure-
ments of respiratory variations in pulmonary
venous flow. J Am Coll Cardiol 1993;22:
1935–43.
5. Guntheroth WG. Constrictive pericarditis versus
restrictive cardiomyopathy. Circulation 1997;95:
7. Goldstein JA. Cardiac tamponade, constrictive
pericarditis, and restrictive cardiomyopathy. Curr
Probl Cardiol 2004;29:503–67.
8. Morshedi-Meibodi A, Menuet R, McFadden M,
et al. Is it constrictive pericarditis or restrictive
cardiomyopathy? A systematic approach. Congest
Heart Fail 2004;10:309–12.

3. Klein AL, Cohen GI. Doppler echocardiographic
assessment of constrictive pericarditis, cardiac
amyloidosis, and cardiac tamponade. Cleve Clin J
Med 1992;59:278–90.
4. Klein AL, Cohen GI, Pietrolungo JF, et al.
Differentiation of constrictive pericarditis
542–3.
6. Rajagopalan N, Garcia MJ, Rodriguez L, et al.
Comparison of new Doppler echocardiographic
methods to differentiate constrictive pericardial
heart disease and restrictive cardiomyopathy. Am
J Cardiol 2001;87:86–94.
9. Yazdani K, Maraj S, Amanullah AM. Differenti-
ating constrictive pericarditis from restrictive car-
diomyopathy. Rev Cardiovasc Med 2005;6:61–71.
10. Sorajja P. Invasive hemodynamics of constric-
tive pericarditis, restrictive cardiomyopathy, and
cardiac tamponade. Cardiol Clin 2011;29:191–9.
JACC VOL. 68, NO. 21, 2016
NOVEMBER 29, 2016:2329–47
11. Spodick DH. Medical history of the pericar-
dium. The hairy hearts of hoary heroes. Am J
Cardiol 1970;26:447–54.
12. Ling LH, Oh JK, Schaff HV, et al. Constrictive
pericarditis in the modern era: evolving clinical
spectrum and impact on outcome after peri-
cardiectomy. Circulation 1999;100:1380–6.
13. Adler Y, Charron P, Imazio M, et al. 2015 ESC
guidelines for the diagnosis and management of
pericardial diseases. Eur Heart J 2015;36:2921–64.
14. Mookadam F, Jiamsripong P, Raslan SF, et al.
Constrictive pericarditis and restrictive cardiomy-
opathy in the modern era. Future Cardiol 2011;7:
471–83.
15. Bertog SC, Thambidorai SK, Parakh K, et al.
Constrictive pericarditis: etiology and cause-
specific survival after pericardiectomy. J Am Coll
Cardiol 2004;43:1445–52.
16. Zhang J, Kumar A, Stalker HJ, et al. Clinical and
molecular studies of a large family with desmin-
associated restrictive cardiomyopathy. Clin Genet
2001;59:248–56.
17. Mogensen J, Kubo T, Duque M, et al. Idiopathic
restrictive cardiomyopathy is part of the clinical
expression of cardiac troponin I mutations. J Clin
Invest 2003;111:209–16.
18. Kushwaha SS, Fallon JT, Fuster V. Restrictive
cardiomyopathy. N Engl J Med 1997;336:267–76.
19. Mocumbi AO, Ferreira MB, Sidi D, et al.
A population study of endomyocardial fibrosis in a
rural area of Mozambique. N Engl J Med 2008;
359:43–9.
20. Salemi VM, Rochitte CE, Shiozaki AA, et al.
Late gadolinium enhancement magnetic reso-
nance imaging in the diagnosis and prognosis of
endomyocardial fibrosis patients. Circ Cardiovasc
Imaging 2011;4:304–11.
21. Acquatella H, Schiller NB, Puigbo JJ, et al.
Value of two-dimensional echocardiography in
endomyocardial disease with and without eosino-
philia. A clinical and pathologic study. Circulation
1983;67:1219–26.
22. Garcia MJ. Constrictive pericarditis versus
restrictive cardiomyopathy? J Am Coll Cardiol
2016;67:2061–76.
23. Yamada H, Tabata T, Jaffer SJ, et al. Clinical
features of mixed physiology of constriction and
restriction: echocardiographic characteristics and
clinical outcome. Eur J Echocardiogr 2007;8:
185–94.
24. Braunwald E. The war against heart failure:
the Lancet lecture. Lancet 2015;385:812–24.
25. Nishimura RA, Tajik AJ. Evaluation of diastolic
filling of left ventricle in health and disease:
Doppler echocardiography is the clinician’s
Rosetta Stone. J Am Coll Cardiol 1997;30:8–18.
26. Morgan BC, Guntheroth WG, Dillard DH.
Relationship of pericardial to pleural pressure
during quiet respiration and cardiac tamponade.
Circ Res 1965;16:493–8.
27. Ragosta M. Pericardial disease and restrictive
cardiomyopathy. In: Ragosta M, editor. Textbook
of Clinical Hemodynamics. Philadelphia, PA:
Saunders, 2008:123–47.
Constriction vs. Restriction: Complex Cardiovascular Hemodynamics
28. Kussmaul A. Über schwielige Mediastino-
Perikarditis und den paradoxen Puls. Berliner
klinische Wochenschrift 1873;10:433–5. 445–9,
461–4.
29. Talreja DR, Nishimura RA, Oh JK, et al.
Constrictive pericarditis in the modern era: novel
criteria for diagnosis in the cardiac catheterization
laboratory. J Am Coll Cardiol 2008;51:315–9.
30. Hirota Y, Kohriyama T, Hayashi T, et al. Idio-
pathic restrictive cardiomyopathy: differences of
left ventricular relaxation and diastolic wave forms
from constrictive pericarditis. Am J Cardiol 1983;
52:421–3.
31. Hirota Y, Shimizu G, Kita Y, et al. Spectrum of
restrictive cardiomyopathy: report of the national
survey in Japan. Am Heart J 1990;120:188–94.
32. Mehta A, Mehta M, Jain AC. Constrictive peri-
carditis. Clin Cardiol 1999;22:334–44.
33. Park JY, Eleid MF, Michelena HI. Abnormal
neck veins. JAMA Cardiol 2016;1:487–8.
34. Hancock EW. Differential diagnosis of restric-
tive cardiomyopathy and constrictive pericarditis.
Heart 2001;86:343–9.
35. Ntsekhe M, Matthews K, Syed FF, et al. Prev-
alence, hemodynamics, and cytokine profile of
effusive-constrictive pericarditis in patients with
tuberculous pericardial effusion. PLoS One 2013;8:
e77532.
36. Lachmann HJ, Booth DR, Booth SE, et al.
Misdiagnosis of hereditary amyloidosis as AL (pri-
mary) amyloidosis. N Engl J Med 2002;346:
1786–91.
37. Leya FS, Arab D, Joyal D, et al. The efficacy of
brain natriuretic peptide levels in differentiating
constrictive pericarditis from restrictive cardio-
myopathy. J Am Coll Cardiol 2005;45:1900–2.
38. Babuin L, Alegria JR, Oh JK, et al. Brain
natriuretic peptide levels in constrictive pericar-
ditis and restrictive cardiomyopathy. J Am Coll
Cardiol 2006;47:1489–91.
39. Sengupta PP, Krishnamoorthy VK,
Abhayaratna WP, et al. Comparison of usefulness
of tissue Doppler imaging versus brain natriuretic
peptide for differentiation of constrictive pericar-
dial disease from restrictive cardiomyopathy. Am J
Cardiol 2008;102:357–62.
40. Reddy PR, Dieter RS, Das P, et al. Utility of
BNP in differentiating constrictive pericarditis
from restrictive cardiomyopathy in patients with
renal insufficiency. J Card Fail 2007;13:668–71.
41. Voelkel AG, Pietro DA, Folland ED, et al.
Echocardiographic features of constrictive peri-
carditis. Circulation 1978;58:871–5.
42. Ling LH, Oh JK, Tei C, et al. Pericardial thick-
ness measured with transesophageal echocardi-
ography: feasibility and potential clinical
usefulness. J Am Coll Cardiol 1997;29:1317–23.
43. Welch TD, Ling LH, Espinosa RE, et al. Echo-
cardiographic diagnosis of constrictive pericarditis:
Mayo Clinic criteria. Circ Cardiovasc Imaging 2014;
7:526–34.
44. Coylewright M, Welch TD, Nishimura RA.
Mechanism of septal bounce in constrictive peri-
carditis: a simultaneous cardiac catheterisation
and echocardiographic study. Heart 2013;99:1376.
Geske et al. 2345
45. Seward JB, Casaclang-Verzosa G. Infiltrative
cardiovascular diseases: cardiomyopathies that
look alike. J Am Coll Cardiol 2010;55:1769–79.
46. Klein AL, Abbara S, Agler DA, et al. American
Society of Echocardiography clinical recommen-
dations for multimodality cardiovascular imaging
of patients with pericardial disease: endorsed by
the Society for Cardiovascular Magnetic Reso-
nance and Society of Cardiovascular Computed
Tomography. J Am Soc Echocardiogr 2013;26:
965–1012.e15.
47. Hurrell DG, Nishimura RA, Higano ST, et al.
Value of dynamic respiratory changes in left and
right ventricular pressures for the diagnosis of
constrictive pericarditis. Circulation 1996;93:
2007–13.
48. Nishimura RA. Constrictive pericarditis in the
modern era: a diagnostic dilemma. Heart 2001;86:
619–23.
49. Hatle LK, Appleton CP, Popp RL. Differentia-
tion of constrictive pericarditis and restrictive
cardiomyopathy by Doppler echocardiography.
Circulation 1989;79:357–70.
50. Oh JK, Hatle LK, Seward JB, et al. Diagnostic
role of Doppler echocardiography in constrictive
pericarditis. J Am Coll Cardiol 1994;23:154–62.
51. Ha JW, Oh JK, Ommen SR, et al. Diagnostic
value of mitral annular velocity for constrictive
pericarditis in the absence of respiratory variation
in mitral inflow velocity. J Am Soc Echocardiogr
2002;15:1468–71.
52. Boonyaratavej S, Oh JK, Tajik AJ, et al. Com-
parison of mitral inflow and superior vena cava
Doppler velocities in chronic obstructive pulmo-
nary disease and constrictive pericarditis. J Am
Coll Cardiol 1998;32:2043–8.
53. Nagueh SF, Appleton CP, Gillebert TC, et al.
Recommendations for the evaluation of left ven-
tricular diastolic function by echocardiography.
J Am Soc Echocardiogr 2009;22:107–33.
54. Chahal NS, Lim TK, Jain P, et al. Normative
reference values for the tissue Doppler imaging
parameters of left ventricular function: a
population-based study. Eur J Echocardiogr 2010;
11:51–6.
55. Ommen SR, Nishimura RA, Appleton CP, et al.
Clinical utility of Doppler echocardiography and
tissue Doppler imaging in the estimation of left
ventricular filling pressures: a comparative simul-
taneous Doppler-catheterization study. Circulation
2000;102:1788–94.
56. Nagueh SF, Middleton KJ, Kopelen HA, et al.
Doppler tissue imaging: a noninvasive technique
for evaluation of left ventricular relaxation and
estimation of filling pressures. J Am Coll Cardiol
1997;30:1527–33.
57. Ha JW, Ommen SR, Tajik AJ, et al. Differenti-
ation of constrictive pericarditis from restrictive
cardiomyopathy using mitral annular velocity by
tissue Doppler echocardiography. Am J Cardiol
2004;94:316–9.
58. Ha JW, Oh JK, Ling LH, et al. Annulus para-
doxus: transmitral flow velocity to mitral annular
velocity ratio is inversely proportional to pulmo-
nary capillary wedge pressure in patients with
2346 Geske et al.
Constriction vs. Restriction: Complex Cardiovascular Hemodynamics
constrictive pericarditis. Circulation 2001;104:
976–8.
59. Reuss CS, Wilansky SM, Lester SJ, et al. Using
mitral ’annulus reversus’ to diagnose constrictive
pericarditis. Eur J Echocardiogr 2009;10:372–5.
60. Veress G, Feng D, Oh JK. Echocardiography in
pericardial diseases: new developments. Heart Fail
Rev 2013;18:267–75.
61. Hernandez CM, Singh P, Hage FG, et al. Live/
real time three-dimensional transthoracic echo-
cardiographic assessment of pericardial disease.
Echocardiography 2009;26:1250–63.
62. D’Cruz IA, Minderman D, Dockery BK. Three-
dimensional imaging of intrapericardial adhesions
within a large pericardial effusion. Can J Cardiol
2009;25:366.
63. Scohy TV, Maat AP, McGhie J, et al. Three-
dimensional transesophageal echocardiography:
diagnosing the extent of pericarditis constrictiva
and intraoperative surgical support. J Card Surg
2009;24:305–8.
64. Nucci EM, Lisi M, Cameli M, et al. The role of
3D and speckle tracking echocardiography in car-
diac amyloidosis: a case report. Eur Rev Med
Pharmacol Sci 2014;18:74–7.
65. Migrino RQ, Harmann L, Woods T, et al.
Intraventricular dyssynchrony in light chain
amyloidosis: a new mechanism of systolic
dysfunction assessed by 3-dimensional echocar-
diography. Cardiovasc Ultrasound 2008;6:40.
66. Sengupta PP, Krishnamoorthy VK,
Abhayaratna WP, et al. Disparate patterns of left
ventricular mechanics differentiate constrictive
pericarditis from restrictive cardiomyopathy. J Am
Coll Cardiol Img 2008;1:29–38.
67. Phelan D, Collier P, Thavendiranathan P, et al.
Relative apical sparing of longitudinal strain using
two-dimensional speckle-tracking echocardiogra-
phy is both sensitive and specific for the diagnosis
of cardiac amyloidosis. Heart 2012;98:1442–8.
68. Baccouche H, Maunz M, Beck T, et al. Differ-
entiating cardiac amyloidosis and hypertrophic
cardiomyopathy by use of three-dimensional
speckle tracking echocardiography. Echocardiog-
raphy 2012;29:668–77.
69. Nemes A, Földeák D, Domsik P, et al. Different
patterns of left ventricular rotational mechanics in
cardiac amyloidosis-results from the three-
dimensional speckle-tracking echocardiographic
MAGYAR-Path Study. Quant Imaging Med Surg
2015;5:853–7.
70. Austin JH. The lateral chest radiograph in the
assessment of nonpulmonary health and disease.
Radiol Clin North Am 1984;22:687–98.
71. Ling LH, Oh JK, Breen JF, et al. Calcific
constrictive pericarditis: is it still with us? Ann
Intern Med 2000;132:444–50.
72. Robles P, Rubio A, Olmedilla P. Value of multi-
detector cardiac CT in calcified constrictive pericar-
ditis for pericardial resection. Heart 2006;92:1112.
73. Bull RK, Edwards PD, Dixon AK. CT dimensions
of the normal pericardium. Br J Radiol 1998;71:
923–5.
74. Talreja DR, Edwards WD, Danielson GK, et al.
Constrictive pericarditis in 26 patients with
histologically normal pericardial thickness. Circu-
lation 2003;108:1852–7.
75. Choi JH, Choi JO, Ryu DR, et al. Mitral and
tricuspid annular velocities in constrictive peri-
carditis and restrictive cardiomyopathy: correla-
tion with pericardial thickness on computed
tomography. J Am Coll Cardiol Img 2011;4:567–75.
76. Ghersin E, Lessick J, Litmanovich D, et al.
Septal bounce in constrictive pericarditis. Diag-
nosis and dynamic evaluation with multidetector
CT. J Comput Assist Tomogr 2004;28:676–8.
77. Masui T, Finck S, Higgins CB. Constrictive
pericarditis and restrictive cardiomyopathy: eval-
uation with MR imaging. Radiology 1992;182:
369–73.
78. Soulen RL, Stark DD, Higgins CB. Magnetic
resonance imaging of constrictive pericardial dis-
ease. Am J Cardiol 1985;55:480–4.
79. Cheng H, Zhao S, Jiang S, et al. The relative
atrial volume ratio and late gadolinium enhance-
ment provide additive information to differentiate
constrictive pericarditis from restrictive cardio-
myopathy. J Cardiovasc Magn Reson 2011;13:15.
80. Fenstad ER, Dzyubak B, Oh JK, et al. Evalua-
tion of liver stiffness with magnetic resonance
elastography in patients with constrictive peri-
carditis: preliminary findings. J Magn Reson Im-
aging 2016;44:81–8.
81. Alter P, Figiel JH, Rupp TP, et al. MR, CT, and
PET imaging in pericardial disease. Heart Fail Rev
2013;18:289–306.
82. Gahide G, Granier M, Frapier JM. Effusive
constrictive pericarditis: functional and anatomical
magnetic resonance findings. Eur Heart J 2009;
30:1371.
83. Feng D, Glockner J, Kim K, et al. Cardiac
magnetic resonance imaging pericardial late gad-
olinium enhancement and elevated inflammatory
markers can predict the reversibility of constrictive
pericarditis after antiinflammatory medical ther-
apy: a pilot study. Circulation 2011;124:1830–7.
84. Aquaro GD, Barison A, Cagnolo A, et al. Role
of tissue characterization by cardiac magnetic
resonance in the diagnosis of constrictive peri-
carditis. Int J Cardiovasc Imaging 2015;31:1021–31.
85. Muehlberg F, Toepper A, Fritschi S, et al.
Magnetic resonance imaging applications on infil-
trative cardiomyopathies. J Thorac Imaging 2016;
31:336–47.
86. Syed IS, Glockner JF, Feng D, et al. Role of
cardiac magnetic resonance imaging in the
detection of cardiac amyloidosis. J Am Coll Cardiol
Img 2010;3:155–64.
87. Boynton SJ, Geske JB, Dispenzieri A, et al. LGE
provides incremental prognostic information over
serum biomarkers in AL cardiac amyloidosis. J Am
Coll Cardiol Img 2016;9:680–6.
88. Francone M, Dymarkowski S, Kalantzi M, et al.
Assessment of ventricular coupling with real-time
cine MRI and its value to differentiate constrictive
pericarditis from restrictive cardiomyopathy. Eur
Radiol 2006;16:944–51.
89. Anavekar NS, Wong BF, Foley TA, et al. Index
of biventricular interdependence calculated using
cardiac MRI: a proof of concept study in patients
JACC VOL. 68, NO. 21, 2016
NOVEMBER 29, 2016:2329–47
with and without constrictive pericarditis. Int J
Cardiovasc Imaging 2013;29:363–9.
90. Kojima S, Yamada N, Goto Y. Diagnosis of
constrictive pericarditis by tagged cine magnetic
resonance imaging. N Engl J Med 1999;341:373–4.
91. Bogabathina H, Biederman RW. Lack of slip-
page by cardiovascular magnetic resonance im-
aging is sine qua non for constrictive pericarditis.
Circulation 2011;123:e418–9.
92. Hagenbuch SC, Gottliebson WM, Wansapura J,
et al. Detection of progressive cardiac dysfunction
by serial evaluation of circumferential strain in
patients with Duchenne muscular dystrophy. Am J
Cardiol 2010;105:1451–5.
93. Hor KN, Wansapura J, Markham LW, et al.
Circumferential strain analysis identifies strata of
cardiomyopathy in Duchenne muscular dystrophy:
a cardiac magnetic resonance tagging study. J Am
Coll Cardiol 2009;53:1204–10.
94. Amaki M, Savino J, Ain DL, et al. Diagnostic
concordance of echocardiography and cardiac
magnetic resonance-based tissue tracking for
differentiating constrictive pericarditis from
restrictive cardiomyopathy. Circ Cardiovasc Imag-
ing 2014;7:819–27.
95. Lobert P, Brown RK, Dvorak RA, et al. Spec-
trum of physiological and pathological cardiac and
pericardial uptake of FDG in oncology PET-CT. Clin
Radiol 2013;68:e59–71.
96. James OG, Christensen JD, Wong TZ, et al.
Utility of FDG PET/CT in inflammatory cardiovas-
cular disease. Radiographics 2011;31:1271–86.
97. Ha JW, Lee JD, Ko YG, et al. Images in car-
diovascular medicine. Assessment of pericardial
inflammation in a patient with tuberculous effu-
sive constrictive pericarditis with 18F-2-deoxy-
glucose positron emission tomography. Circulation
2006;113:e4–5.
98. Losik SB, Studentsova Y, Margouleff D.
Chemotherapy-induced pericarditis on F-18 FDG
positron emission tomography scan. Clin Nucl Med
2003;28:913–5.
99. Blockmans D, Stroobants S,
Vanderschueren S, et al. FDG-PET scan in the
diagnosis of postmeningococcal pericarditis. Clin
Nucl Med 2002;27:59.
100. Strobel K, Schuler R, Genoni M. Visualization
of pericarditis with fluoro-deoxy-glucose-positron
emission tomography/computed tomography. Eur
Heart J 2008;29:1212.
101. Dong A, Dong H, Wang Y, et al. 18F-FDG PET/
CT in differentiating acute tuberculous from idio-
pathic pericarditis: preliminary study. Clin Nucl
Med 2013;38:e160–5.
102. Falk RH, Quarta CC, Dorbala S. How to image
cardiac amyloidosis. Circ Cardiovasc Imaging 2014;
7:552–62.
103. Bokhari S, Castaño A, Pozniakoff T, et al.
99mTc-pyrophosphate scintigraphy for differen-
tiating light-chain cardiac amyloidosis from the
transthyretin-related familial and senile cardiac
amyloidoses. Circ Cardiovasc Imaging 2013;6:
195–201.
104. Glaudemans AW, Slart RH, Zeebregts CJ,
et al. Nuclear imaging in cardiac amyloidosis. Eur J
Nucl Med Mol Imaging 2009;36:702–14.
JACC VOL. 68, NO. 21, 2016
NOVEMBER 29, 2016:2329–47
105. Nishimura RA, Carabello BA. Hemodynamics
in the cardiac catheterization laboratory of the
21st century. Circulation 2012;125:2138–50.
106. Soo WM, Ling LH, Schaff HV, et al. P5217:
Elevated pulmonary artery systolic pressure pre-
dicts poorer survival in constrictive pericarditis
(abstr). Eur Heart J 2014;35 Suppl 1:920–1.
107. Little WC, Freeman GL. Pericardial disease.
Circulation 2006;113:1622–32.
108. From AM, Maleszewski JJ, Rihal CS. Current
status of endomyocardial biopsy. Mayo Clin Proc
2011;86:1095–102.
109. Bennett MK, Gilotra NA, Harrington C, et al.
Evaluation of the role of endomyocardial biopsy in
851 patients with unexplained heart failure from
2000-2009. Circ Heart Fail 2013;6:676–84.
110. Leone O, Veinot JP, Angelini A, et al. 2011
consensus statement on endomyocardial biopsy
from the Association for European Cardiovascular
Pathology and the Society for Cardiovascular Pa-
thology. Cardiovasc Pathol 2012;21:245–74.
111. Haley JH, Tajik AJ, Danielson GK, et al. Tran-
sient constrictive pericarditis: causes and natural
history. J Am Coll Cardiol 2004;43:271–5.
112. Sagristà-Sauleda J, Permanyer-Miralda G,
Candell-Riera J, et al. Transient cardiac constric-
tion: an unrecognized pattern of evolution in
effusive acute idiopathic pericarditis. Am J Cardiol
1987;59:961–6.
113. Mayosi BM, Ntsekhe M, Bosch J, et al., IMPI
Trial Investigators. Prednisolone and Mycobacte-
rium indicus pranii in tuberculous pericarditis.
N Engl J Med 2014;371:1121–30.
114. Mutyaba AK, Mayosi BM. Emerging Countries
and Tuberculosis Constriction. ACC.org Latest in
Constriction vs. Restriction: Complex Cardiovascular Hemodynamics
Cardiology. April 13, 2016. Available at: http://
www.acc.org/latest-in-cardiology/articles/2016/
04/12/13/28/emerging-countries-and-tuberculosis
-constriction. Accessed September 1, 2016.
115. Chowdhury UK, Subramaniam GK, Kumar AS,
et al. Pericardiectomy for constrictive pericarditis:
a clinical, echocardiographic, and hemodynamic
evaluation of two surgical techniques. Ann Thorac
Surg 2006;81:522–9.
116. Szabó G, Schmack B, Bulut C, et al.
Constrictive pericarditis: risks, aetiologies and
outcomes after total pericardiectomy: 24 years of
experience. Eur J Cardiothorac Surg 2013;44:
1023–8; discussion 1028.
117. Dines DE, Edwards JE, Burchell HB. Myocar-
dial atrophy in constrictive pericarditis. Proc Staff
Meet Mayo Clin 1958;33:93–9.
118. Schwefer M, Aschenbach R, Heidemann J, et al.
Constrictive pericarditis, still a diagnostic chal-
lenge: comprehensive review of clinical manage-
ment. Eur J Cardiothorac Surg 2009;36:502–10.
119. Clare GC, Troughton RW. Management of
constrictive pericarditis in the 21st century. Curr
Treat Options Cardiovasc Med 2007;9:436–42.
120. McCaughan BC, Schaff HV, Piehler JM, et al.
Early and late results of pericardiectomy for
constrictive pericarditis. J Thorac Cardiovasc Surg
1985;89:340–50.
121. Tuna IC, Danielson GK. Surgical management
of pericardial diseases. Cardiol Clin 1990;8:
683–96.
122. Cameron J, Oesterle SN, Baldwin JC, et al.
The etiologic spectrum of constrictive pericarditis.
Am Heart J 1987;113:354–60.
Geske et al. 2347
123. Syed FF, Schaff HV, Oh JK. Constrictive peri-
carditis–a curable diastolic heart failure. Nat Rev
Cardiol 2014;11:530–44.
124. Webber SA, Lipshultz SE, Sleeper LA,
et al., Pediatric Cardiomyopathy Registry In-
vestigators. Outcomes of restrictive cardiomy-
opathy in childhood and the influence of
phenotype: a report from the Pediatric Cardio-
myopathy Registry. Circulation 2012;126:
1237–44.
125. Weller RJ, Weintraub R, Addonizio LJ,
et al. Outcome of idiopathic restrictive cardio-
myopathy in children. Am J Cardiol 2002;90:
501–6.
126. Ammash NM, Seward JB, Bailey KR, et al.
Clinical profile and outcome of idiopathic restric-
tive cardiomyopathy. Circulation 2000;101:
2490–6.
127. Katritsis D, Wilmshurst PT, Wendon JA, et al.
Primary restrictive cardiomyopathy: clinical and
pathologic characteristics. J Am Coll Cardiol 1991;
18:1230–5.
128. DePasquale EC, Nasir K, Jacoby DL. Outcomes
of adults with restrictive cardiomyopathy after
heart transplantation. J Heart Lung Transplant
2012;31:1269–75.
KEY WORDS constrictive pericarditis, heart
failure, hemodynamics, restrictive
cardiomyopathy
A PPE NDI X For a supplemental video and
its legend, please see the online version of this
article.