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www.uptodate.com 2017 UpToDate

Constrictive pericarditis

Author: Brian D Hoit, MD


Section Editors: Martin M LeWinter, MD, Gabriel S Aldea, MD, Edward Verrier, MD
Deputy Editor: Brian C Downey, MD, FACC

All topics are updated as new evidence becomes available and our peer review process is complete.
Literature review current through: Aug 2017. | This topic last updated: Jun 16, 2017.

INTRODUCTION The normal pericardium is a fibroelastic sac surrounding the heart that contains a thin
layer of fluid. When larger amounts of fluid accumulate (pericardial effusion) or when the pericardium
becomes scarred and inelastic, one of three pericardial compressive syndromes may occur:

Cardiac tamponade Cardiac tamponade, which may be acute or subacute, is characterized by the
accumulation of pericardial fluid under pressure. Variants include low pressure (occult) and regional
tamponade.

Constrictive pericarditis Constrictive pericarditis is the result of scarring and consequent loss of the
normal elasticity of the pericardial sac. Pericardial constriction is typically chronic, but variants include
subacute, transient, and occult constriction.

Effusive-constrictive pericarditis Effusive-constrictive pericarditis is characterized by underlying


constrictive physiology with a coexisting pericardial effusion, often with cardiac tamponade [1]. This
usually results in a mixed hemodynamic picture with features of both constriction and tamponade.
Such patients may be mistakenly thought to have only cardiac tamponade; however, elevation of the
right atrial and pulmonary wedge pressures after drainage of the pericardial fluid points to the
underlying constrictive process.

In both typical constrictive pericarditis and effusive-constrictive pericarditis, cardiac filling is impeded by an
external force. The normal pericardium can stretch to accommodate physiologic changes in cardiac
volume. However, after its reserve volume is exceeded the pericardium markedly stiffens. In severe
pericardial compressive syndromes, the pericardium becomes virtually inelastic, resulting in minimal ability
to adapt to volume changes.

As a result, an important pathophysiologic feature of constrictive pericarditis is greatly enhanced


ventricular interdependence, in which the hemodynamics of the left and right heart chambers are directly
influenced by each other to a much greater degree than normal. (See 'Hemodynamic evaluation' below.)

The physiology, clinical presentation, diagnosis, and treatment of constrictive pericarditis and effusive-
constrictive pericarditis will be reviewed here. Issues related to cardiac tamponade, and the evaluation
and management of pericardial diseases that do not compromise hemodynamics, are discussed
separately. (See "Cardiac tamponade" and "Diagnosis and treatment of pericardial effusion" and "Acute
pericarditis: Clinical presentation and diagnostic evaluation".)

PHYSIOLOGY In patients with a normal pericardium, intrathoracic pressure decreases during


inspiration, leading to an increase in venous return to the right heart and transient increase in right
ventricular chamber size. Because the normal pericardium accommodates the increased venous return by
expanding, this increase in venous return does not impair left ventricular filling.

In constrictive pericarditis the upper limit of cardiac volume is constrained by the inelastic pericardium.

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The thickened, rigid pericardium prevents the normal inspiratory decrease in intrathoracic pressure from
being transmitted to the heart chambers. The abnormal pericardium also does not expand to
accommodate increased venous return to the right heart during inspiration. Thus, pulmonary venous
pressure, but not left ventricular pressure, declines during inspiration, leading to a reduction in left
ventricular volume because of a decrease in the transpulmonary gradient. Because of ventricular
interaction, the right heart volume expands via shifting of the interventricular septum. This interaction
between the left and right ventricles is termed ventricular interdependence.

With constrictive pericarditis, early diastolic filling is even more rapid than normal. Compression does not
occur until the cardiac volume approximates that of the pericardium, which begins in mid-diastole. As a
result, virtually all ventricular filling occurs in early diastole with little or no filling subsequently. As
constrictive pericarditis becomes more severe, ventricular volumes and stroke volumes are reduced.

The hemodynamic findings and distinction between constrictive pericarditis and restrictive cardiomyopathy
are discussed in more detail elsewhere. (See "Differentiating constrictive pericarditis and restrictive
cardiomyopathy".)

ETIOLOGY Constrictive pericarditis can occur after virtually any pericardial disease process [2,3]. The
following causes of constrictive pericarditis were identified in case series from tertiary care centers [4-9]
(see "Recurrent pericarditis"):

Idiopathic or viral 42 to 61 percent


Post-cardiac surgery 11 to 37 percent
Post-radiation therapy 2 to 31 percent, primarily after Hodgkin disease or breast cancer
Connective tissue disorder 3 to 7 percent
Postinfectious (tuberculous or purulent pericarditis) 3 to 15 percent
Miscellaneous causes (malignancy, trauma, drug-induced, asbestosis, sarcoidosis, uremic
pericarditis) 1 to 10 percent

The risk of constrictive pericarditis following a first episode of acute pericarditis was assessed in 500
patients prospectively studied over a mean follow-up of six years (24 to 120 months) [10]. Constrictive
pericarditis developed in 1.8 percent (2 of 416 patients with idiopathic/viral pericarditis and 7 of 84 patients
with a nonviral/nonidiopathic etiology). The incidence rate of constrictive pericarditis was:

Idiopathic/viral 0.76 cases per 1000 person-years


Connective tissue/pericardial injury syndrome 4.40 cases per 1000 person-years
Neoplastic pericarditis 6.33 cases per 1000 person-years
Tuberculous pericarditis 31.65 cases per 1000 person-years
Purulent pericarditis 52.75 cases per 1000 person-years

Constrictive pericarditis following recurrent pericarditis is rare. Cases of constrictive pericarditis following
orthotopic heart transplant have also been reported [11]. Tuberculosis accounted for 49 percent of cases
of constrictive pericarditis in a series reported in 1962 [12]. While tuberculosis is now only a rare cause of
constrictive pericarditis in developed countries, it should be considered as a possible etiology among
patients at high risk of developing active tuberculosis, including those from endemic areas and patients
with HIV infection. (See "Tuberculous pericarditis".)

Patients with a newly recognized systemic multi-organ fibroinflammatory disease associated with elevated
serum levels of IgG4 may present with constrictive pericarditis; histopathologically, the pericardium is
fibrotic with IgG4-positive lymphoplasmacytic infiltration [13]. Another unusual cause of constrictive
pericarditis is Whipple's disease [14].

CLINICAL PRESENTATION

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History Patients with constrictive pericarditis typically present with one or both of the following
constellations of symptoms [3]:

Symptoms related to fluid overload, ranging from peripheral edema to anasarca


Symptoms related to diminished cardiac output in response to exertion, such as fatigability and
dyspnea on exertion

In one series of 135 patients, 67 percent of patients presented with symptoms of heart failure, 8 percent
with chest pain, 6 percent with abdominal symptoms, 4 percent with atrial arrhythmia, and only 5 percent
with symptoms of cardiac tamponade (eg, dyspnea, fatigue, reduced exercise capacity) [5]. (See
"Evaluation of the patient with suspected heart failure", section on 'Clinical presentation' and "Cardiac
tamponade".)

The history in a patient with suspected constrictive pericarditis should also include focused questioning
directed at the potential underlying causes (eg, known history of prior malignancy with thoracic radiation
treatment, prior cardiothoracic surgery, chest trauma, etc).

Physical examination The vast majority of patients with constrictive pericarditis display elevated
jugular venous pressure (JVP) on physical examination. Other important but less common features on the
physical examination include pulsus paradoxus, Kussmaul's sign, a pericardial knock, edema, ascites,
and/or cachexia.

Elevated JVP has been reported in as many as 93 percent of patients with surgically confirmed
constrictive pericarditis [5]. The characteristics of the jugular venous waveforms in patients with
constrictive pericarditis are different from those in normal patients and patients with other types of
cardiac disease (figure 1). The x and y troughs are more prominent than the a and v peaks and the
inspiratory decline in venous pressure is confined to the depth of the y descent. (See 'Hemodynamic
evaluation' below.)

Pericardial constriction should be considered in any patient with an unexplained elevation in JVP,
particularly if there is a history of a predisposing condition such as malignancy, prior cardiac surgery,
or prior radiation therapy.

JVP may be normal in patients with early or mild constrictive pericarditis. In addition, occult
constrictive pericarditis has been described in patients who are volume depleted; in this setting, the
increase in venous pressure may not be apparent prior to volume expansion [15]. (See 'Occult
constrictive pericarditis' below.)

Pulsus paradoxus (an exaggerated drop in systemic blood pressure greater than 10 mmHg during
inspiration) occurs in less than 20 percent of patients with constrictive pericarditis (waveform 1) [5,16].
It may be more common in patients with concomitant pericardial effusion with cardiac tamponade or
coexisting pulmonary disease. (See "Pulsus paradoxus in pericardial disease".)

Kussmaul's sign (the lack of an inspiratory decline in JVP) is present in patients with constrictive
pericarditis, but does not distinguish constrictive pericarditis from severe tricuspid valve disease or
right-sided heart failure. While case series have noted rates of 13 to 21 percent, Kussmaul's sign is
more common in our experience [4,5]. (See "Examination of the jugular venous pulse".)

A pericardial knock, an accentuated heart sound occurring slightly earlier than an S3 which may be
audible and rarely is palpable, has been reported in 47 percent of patients with constrictive
pericarditis in one series [5]. Sixteen percent of patients in the same series had a pericardial friction
rub.

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Profound cachexia, peripheral edema, ascites, pulsatile hepatomegaly (part of the syndrome of
congestive hepatopathy), and pleural effusion are common findings with more severe constrictive
pericarditis.

EVALUATION Patients with suspected constrictive pericarditis, based on history and physical
examination, should undergo initial evaluation with electrocardiography, chest radiography, and
echocardiography [3]. While the diagnosis of constrictive pericarditis is often made by echocardiography,
patients commonly undergo cardiac catheterization prior to surgical intervention. Invasive hemodynamic
evaluation during cardiac catheterization can confirm the diagnosis and concurrent coronary angiography
defines the patients coronary anatomy prior to possible surgical intervention. In patients being evaluated
for pericardiectomy, particularly those with prior radiation exposure, computed tomography or cardiac
magnetic resonance imaging can provide additional detailed anatomic information about adjacent vascular
structures and the extent of pericardial thickening, calcification, and scarring.

Electrocardiography There are no pathognomonic electrocardiographic findings in constrictive


pericarditis. Nonspecific ST and T wave changes and tachycardia are common, and low voltage may
sometimes be present. In advanced cases, atrial fibrillation is common due to increased atrial pressures.
In a series of 143 patients with surgically confirmed constrictive pericarditis, 22 percent had atrial
fibrillation and 27 percent had low voltage [17].

Chest radiograph The presence of pericardial calcification by chest radiograph, especially in


conjunction with the appropriate clinical presentation, is highly consistent with constrictive pericarditis.
However, the majority of patients with constrictive pericarditis will not have pericardial calcification, so its
absence does not exclude constrictive pericarditis.

Chest radiographs that demonstrate a ring of calcification around the heart, best seen on lateral or
anterior oblique projections, strongly suggest constrictive pericarditis in patients with symptoms of right-
sided heart failure (image 1) [3]. Pericardial calcification can occur in the absence of constriction, but is
usually less dense and has a patchy distribution. A retrospective review of 135 patients with constrictive
pericarditis confirmed surgically or at autopsy reported that 36 patients (27 percent) had pericardial
calcification [18]. Compared to those without calcification, patients with calcification had the following:

A greater likelihood of having idiopathic pericardial disease (67 versus 21 percent)


A longer duration of symptoms
A greater likelihood of having a pericardial knock, larger atria, and/or atrial arrhythmias
A higher perioperative mortality, but the same long-term survival

Echocardiography Transthoracic echocardiography (TTE) is an essential diagnostic test in patients


being evaluated for constrictive pericarditis. The American College of Cardiology/American Heart
Association/American Society of Echocardiography guidelines and the European Society of Cardiology
guidelines recommend the use echocardiography for the evaluation of all patients with suspected
pericardial disease [3,19]. Two-dimensional and M-mode echocardiography allow structural visualization
while Doppler echocardiography provides hemodynamic information.

Two-dimensional and M-mode One or more of the following findings may be seen by two-dimensional
or M-mode echocardiography in patients with constrictive pericarditis [2,17,20,21] (see
"Echocardiographic evaluation of the pericardium"):

Increased pericardial thickness TTE demonstrates increased pericardial thickness with or without
calcification in approximately 40 percent of patients. Mildly increased pericardial thickening is often
missed, and false positive results suggesting calcification can be obtained if the gain setting is too
high. In comparison, measurement of pericardial thickness by transesophageal echocardiography
(TEE) correlates strongly with that obtained by computed tomography [22].

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M mode No sign or combination of signs on M-mode is diagnostic of constrictive pericarditis.


However, a normal M-mode study with none of the following findings virtually rules out the diagnosis
of constrictive pericarditis [23]:

Abrupt posterior motion of the ventricular septum in early diastole with inspiration (septal shudder
and bounce) caused by underfilling of the left ventricle (due to the decreased pulmonary vein-left
atrial gradient with inspiration).

Systemic venous return is not increased with inspiration due to volume constraints imposed by
the abnormal pericardium [24].

Notching of the ventricular septal tracing in early diastole or with atrial systole due to a transient
reversal of ventricular septal transmural pressure at these times in the cardiac cycle [25].

Rapid posterior motion of the left ventricular posterior wall in early diastole followed by a
flattening of the wall in mid diastole (also a feature of restrictive cardiomyopathy).

Premature opening of the pulmonic valve as right ventricular diastolic pressure rises above
pulmonary arterial pressure.

Two-dimensional echocardiography Two-dimensional echocardiography may reveal [26,27]:

Dilatation of the inferior vena cava and hepatic veins (plethora) with absent or diminished
inspiratory collapse.

Moderate biatrial enlargement (severe enlargement is more compatible with restrictive


cardiomyopathy).

A sharp halt in ventricular diastolic filling (corresponding to the end of early rapid diastolic filling
as noted on Doppler).

Septal bounce with abrupt transient rightward movement of the interventricular septum.

Hypermobile atrioventricular valves.

An abnormal contour between the posterior left ventricular and left atrial walls.

In a reported series of 143 patients with surgically confirmed constriction, 138 underwent TTE [17].
Increased pericardial thickness was seen in 37 percent, abnormal septal motion in 49 percent, and atrial
enlargement in 61 percent.

Doppler echocardiography Doppler echocardiography is critical for the diagnosis of constrictive


pericarditis. The following findings on Doppler echocardiography are suggestive of constrictive pericarditis:

Abnormal passive filling of the ventricles during early diastole High E velocity of right and left
ventricle (LV) inflow is seen due to the abnormally rapid early diastolic filling associated with the
combination of a small ventricular volume and rapid recoil. The early diastolic Doppler tissue velocity
at the mitral annulus (E') is prominent (unless slowed by mitral annular calcification). The usually
positive linear relation between E/E and left atrial pressure, which is useful for assessing left atrial
pressure in cardiomyopathy, is reversed (annular paradox) in constrictive pericarditis [28]. (See
"Differentiating constrictive pericarditis and restrictive cardiomyopathy".)

Annular early diastolic (E') velocities are lower in constrictive pericarditis secondary to surgery or
radiation than idiopathic or post-pericarditis etiologies. The mitral lateral/medial E' ratio is reversed in
the majority of patients with constrictive pericarditis. After pericardiectomy, annular velocities are
reduced, and the lateral/medial E' ratio normalizes [29].

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The propagation velocity of early diastolic transmitral flow on color M-mode is normal or increased.

Pronounced respiratory variation in ventricular filling Mitral inflow velocity falls as much as 25 to 40
percent and tricuspid velocity greatly increases (>40 to 60 percent) in the first cardiac cycle following
inspiration. The respiratory variation in pulmonary venous flow is even more pronounced [30]. These
phenomena, which are manifestations of ventricular interdependence, are not present in either
normal subjects or patients with restrictive cardiomyopathy [31]. Increased respiratory variation of
mitral inflow may be missing in patients with markedly elevated left atrial pressure, but can sometimes
be elicited in such patients by preload reduction with semi-recumbent (rather than supine) positioning
or diuretic administration [32]. In an American Society of Echocardiography consensus statement,
calculation of percentage respiratory variation for mitral and tricuspid inflow was standardized as:

[(expiration-inspiration)/expiration] X 100

Changes of 25 and 40 percent respectively are considered significant [33].

Hepatic venous flow reversal increases with expiration, reflecting the ventricular interdependence and
the dissociation of intracardiac and intrathoracic pressures [2].

In a study of one hundred thirty patients with surgically-confirmed constrictive pericarditis who were
compared with 36 patients with restrictive myocardial disease or severe tricuspid regurgitation, ventricular
septal shift, medial annular E', and hepatic vein expiratory diastolic reversals were independently
associated with constrictive pericarditis [34]. The finding of ventricular septal shift in combination with
either a medial E' 9 cm/sec or an expiratory diastolic reversal ratio 0.79 was 87 and 91 percent
sensitive and specific for constriction, respectively [34].

CT scan Computed tomographic (CT) scanning of the heart, obtained by rapid scanning gated to the
cardiac cycle, is useful in the diagnosis of constrictive pericarditis and can provide additional data to guide
perioperative management decisions.

Findings on CT include increased pericardial thickness and calcification [3]. In a review of 143 patients
with surgically confirmed constrictive pericarditis, 97 underwent preoperative CT imaging [17]. A
pathologically thickened pericardium (>4 mm) was seen in 72 percent and pericardial calcification in 25
percent. A normal appearance, or nonvisualization, of the pericardium does not rule out constrictive
pericarditis. Other findings on CT scanning which suggest constrictive pericarditis include dilatation of the
inferior vena cava, deformed ventricular contours, and angulation of the ventricular septum.
Nonvisualization of the posterolateral left ventricular wall on dynamic CT may indicate myocardial fibrosis
or atrophy and is associated with a poor surgical outcome [35].

CT imaging may also be used to examine the effect of cardiac motion transmitted to the surrounding
pulmonary parenchyma. Failure of the immediately adjacent pulmonary structures to pulsate during the
cardiac cycle, in the presence of a regionally or globally thickening pericardium, is virtually diagnostic of
constrictive pericarditis.

CT may assist in the preoperative planning of pericardiectomy in patients with a history of previous
cardiothoracic surgery given its ability to identify critical vascular structures [36]. Additionally, in selected
patients, CT offers the ability to:

Assess the extent of lung injury in patients with previous radiation exposure
Evaluate the location and extent of pericardial calcification
Avoid the need for invasive coronary angiography in those with normal CT coronary angiography

Magnetic resonance imaging Gated cardiac magnetic resonance imaging (CMR) provides direct
visualization of the normal pericardium, which is composed of fibrous tissue and has a low MRI signal

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intensity [37]. CMR is advocated by some as the diagnostic procedure of choice for the detection of
certain pericardial diseases, including constrictive pericarditis [38-40]. Characteristic CMR features in
patients with constrictive pericarditis include increased pericardial thickening, ventricular interdependence,
and dilatation of the inferior vena cava, an indirect sign of impaired right ventricular diastolic filling [3].
(See "Clinical utility of cardiovascular magnetic resonance imaging".)

While computed tomography (CT) is superior to CMR in detecting calcification, CMR better differentiates
small effusions from pericardial thickening. CMR also has the potential to resolve hemodynamic events
such as septal bounce and to better identify pericardial inflammation and pericardial-myocardial
adherence [36]. CMR with late gadolinium enhancement (LGE) of the pericardium is fairly common but not
universal in patients with constrictive pericarditis [41,42]. Persons with constrictive pericarditis and
pericardial LGE had greater fibroblast proliferation, chronic inflammation, and pericardial thickening
compared to those without LGE [42]. Pericardial LGE might also be a predictor of reversibility of
constrictive pericarditis following treatment with antiinflammatory agents [41]. Real-time, free-breathing
phase contrast flow (RT-PC CMR) can detect the characteristic respirophasic changes in mitral and
tricuspid flow; in a study of 16 patients, the sensitivity and specificity of respiratory variation of transmitral
flow >25 percent and transtricuspid flow >45 percent were 100 and 100 percent and 90 and 88 percent,
respectively [43].

We suggest using CMR imaging in patients being considered for pericardiectomy to provide additional
detailed anatomic information that might alter the decision to proceed with surgery based on the likelihood
of resolution with medical therapy alone. (See 'Transient constrictive pericarditis' below.)

Hemodynamic evaluation Invasive hemodynamic evaluation is occasionally needed to confirm the


diagnosis of constrictive pericarditis, particularly in patients with suboptimal or nondiagnostic
echocardiographic findings [2]. Moreover, patients with diagnostic-quality echocardiographic findings may
require coronary angiography for evaluation of coronary anatomy prior to pericardiectomy. In this setting,
an invasive hemodynamic evaluation may also be performed. (See "Differentiating constrictive pericarditis
and restrictive cardiomyopathy".)

The major hemodynamic findings in patients with constrictive pericarditis include:

Increased right atrial pressure.

Prominent x and y descents of venous and atrial pressure tracings (figure 1). In contrast, the y
descent of diastolic ventricular filling is absent in tamponade. (See "Cardiac tamponade", section on
'Physical findings'.)

Kussmaul's sign (the lack of an inspiratory decline or an inspiratory increase in central venous
pressure).

Increased RV end-diastolic pressure, usually to a level one-third or more of RV systolic pressure.

"Square root" signs in the RV and LV diastolic pressure tracings (an early diastolic dip followed by a
plateau of diastasis; the last stage of diastole just before contraction), often with an absent a wave [3].
This finding, also called dip and plateau, reflects rapid early diastolic filling of the ventricles, followed
by lack of additional filling due to compression in mid and late diastole (figure 2). The plateau
configuration (of the dip and plateau pattern) may be diminished or absent in patients who are
tachycardic due to the shortening of diastole.

A greater inspiratory fall in pulmonary capillary wedge pressure compared to left ventricular diastolic
pressure.

Equalization of LV and RV diastolic plateau pressure tracings, with little separation with exercise,

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since filling, and therefore diastolic pressure, in both ventricles is constrained by the inelastic
pericardium [3]. In some patients, this finding is seen only during inspiration (waveform 2).

Mirror-image discordance between RV and peak LV systolic pressures during inspiration, another
sign of increased ventricular interdependence. During peak inspiration, an increase in RV pressure
occurs when LV pressure is lowest [44]. These changes can be detected by both invasive
hemodynamic monitoring and Doppler echocardiography.

In a series of 143 patients with surgically confirmed constrictive pericarditis, 78 underwent cardiac
catheterization [17]. The mean right atrial pressure was 21 mmHg. A dip and plateau pattern was seen in
77 percent, diastolic equalization of pressures in 81 percent, and respiratory variation in the RV-LV
pressure relationship in 44 percent.

Plasma BNP Plasma concentrations of brain natriuretic peptide (BNP) are increased in patients with
left ventricular dysfunction; as a result, plasma BNP is used in the evaluation of dyspnea and to assess
the efficacy of therapy and estimate prognosis in patients with heart failure (HF). (See "Natriuretic peptide
measurement in heart failure".)

Among patients with cardiomyopathy, BNP is released in response to left ventricular dysfunction and wall
stretch. However, wall stretch is limited in constrictive pericarditis by the thickened stiff pericardium. These
physiologic differences suggest that the elevation in plasma BNP in constrictive pericarditis should be
much less than in other types of cardiomyopathy (eg, restrictive cardiomyopathy), which is consistent with
findings of small studies showing BNP values to be lower in patients with constrictive pericarditis. (See
"Differentiating constrictive pericarditis and restrictive cardiomyopathy", section on 'Plasma BNP'.)

DIFFERENTIAL DIAGNOSIS The symptoms and physical findings in patients with constrictive
pericarditis are similar to those of several other disorders. Most importantly, the clinician must distinguish
between constrictive pericarditis, which is treated by pericardiectomy; cardiac tamponade, which is treated
by drainage of the pericardial effusion; and disorders such as restrictive cardiomyopathy and cirrhosis,
which require markedly different treatment [2].

Comparison with restrictive cardiomyopathy Patients with both constrictive pericarditis and
restrictive cardiomyopathy have elevated left and right sided filling pressures, often of equal magnitude,
and usually have normal systolic ventricular function. While the history, physical examination, and
radiographic findings may suggest a particular diagnosis (algorithm 1), constrictive pericarditis and
restrictive cardiomyopathy are usually distinguished by hemodynamic findings from Doppler
echocardiography and/or cardiac catheterization [3]. This is discussed in more detail separately. (See
"Differentiating constrictive pericarditis and restrictive cardiomyopathy".)

The history and physical examination may provide helpful clues to the diagnosis. Constrictive pericarditis
is suggested in a patient with prior pericarditis or a systemic disease predisposing to constrictive
pericarditis (eg, tuberculosis). Restrictive cardiomyopathy is more likely in a patient with a predisposing
systemic disease such as diabetes mellitus or amyloidosis. A pericardial knock favors constrictive
pericarditis, but is difficult to distinguish from the third heart sound of heart failure. (See "Differentiating
constrictive pericarditis and restrictive cardiomyopathy", section on 'History and physical examination'.)

Radiographic findings may also provide clues to the diagnosis. Increased thickness or calcification of the
pericardium favors the diagnosis of constrictive pericarditis, while myocardial thickening and abnormal
myocardial texture suggest restrictive cardiomyopathy. Although increased pericardial thickness is a highly
specific finding for the diagnosis of constrictive pericarditis, it is not present in all patients. In one series of
patients with surgically confirmed constrictive pericarditis, pericardial thickening was seen in only 37
percent by transthoracic echocardiogram and 72 percent by computed tomography [17]. On
histopathologic examination of pericardiectomy specimens, 26 patients (18 percent) had normal

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pericardial thickness.

Hemodynamic findings that distinguish the two conditions include:

Respiratory variation in ventricular inflow velocities Doppler echocardiography reveals an abnormal


increase in respiratory variation of the ventricular inflow velocities in patients with constrictive
pericarditis compared to a normal pattern in patients with restrictive cardiomyopathy. (See
'Echocardiography' above.)

Hepatic venous flow reversal Hepatic venous flow usually reverses during expiration in constrictive
pericarditis but reverses during inspiration in restrictive cardiomyopathy.

Doppler tissue velocity The early diastolic Doppler tissue velocity at the mitral annulus (E') is
decreased (<8 cm/sec) in restrictive cardiomyopathy, due to an intrinsic decrease in myocardial
contraction and relaxation. In contrast, the transmitral E' is frequently increased (>12 cm/sec) in
constrictive pericarditis, since the longitudinal movement of the myocardium is enhanced because of
constricted radial motion [28,45]. (See 'Echocardiography' above.)

While E' values <8 cm/sec or >12 cm/sec are highly specific for constrictive pericarditis or restrictive
cardiomyopathy, many patients will have an E' velocity between 8 cm/sec and 12 cm/sec, which is
nondiagnostic. Despite excellent specificity of E' for differentiating restrictive cardiomyopathy from
constrictive pericarditis, its sensitivity is more modest.

Ventricular end-diastolic pressures Right and left ventricular end-diastolic pressures (RVEDP and
LVEDP) are equal or nearly equal in constrictive pericarditis, while LVEDP is usually higher than
RVEDP in restrictive cardiomyopathy. However, in many cases of restrictive cardiomyopathy the
plateau of diastolic pressure is equally elevated in both ventricles as typically occurs in constrictive
pericarditis. (See "Differentiating constrictive pericarditis and restrictive cardiomyopathy", section on
'Cardiac catheterization'.)

In the rare patient in whom the hemodynamic and imaging studies fail to establish the diagnosis,
endomyocardial or pericardial biopsy may be helpful [46,47].

Comparison with cardiac tamponade Several similarities exist in the clinical presentation of
constrictive pericarditis and cardiac tamponade. However, these entities differ in their effects on diastolic
ventricular filling, which can lead to different findings on physical examination, echocardiography, and
invasive hemodynamic assessment. A detailed discussion of these similarities and differences is
presented in the UpToDate topic on cardiac tamponade. (See "Cardiac tamponade", section on
'Comparison with constrictive pericarditis'.)

Comparison with chronic liver disease Patients with constrictive pericarditis presenting with ascites
as the main manifestation are often thought to have cirrhosis, sometimes considered cryptogenic because
the history does not reveal an obvious cause. Common physical examination findings in both constrictive
pericarditis and chronic liver disease include peripheral edema, ascites, pulsatile hepatomegaly (part of
the syndrome of congestive hepatopathy), and pleural effusion. These findings may lead to the
misdiagnosis of chronic liver disease.

The evaluation of jugular venous pressure (JVP) can help distinguish constrictive pericarditis from chronic
liver disease. In constrictive pericarditis, there is marked elevation in JVP with characteristic changes in
the morphology of the jugular venous pulsations (figure 1). In patients with cirrhosis from chronic liver
disease, unless there is tense ascites, JVP is normal or only slightly elevated. JVP in patients with tense
ascites should rapidly normalize following removal of some of the ascitic fluid [48]. (See "Clinical
manifestations and diagnosis of edema in adults", section on 'Distribution of edema and central venous
pressure' and "Congestive hepatopathy".)

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TREATMENT AND OUTCOME The constrictive symptoms of constrictive pericarditis are permanent
and often progressive in the majority of patients unless the constrictive pericarditis is surgically treated
with pericardiectomy. Constriction is transient or reversible in a minority of patients with constrictive
pericarditis. Thus, in the absence of evidence that the condition is chronic (eg, cachexia, atrial fibrillation,
hepatic dysfunction, or pericardial calcification), patients with newly diagnosed constrictive pericarditis
who are hemodynamically stable may be given a trial of conservative management for two to three
months before pericardiectomy is recommended.

Transient constrictive pericarditis A subset of patients with constrictive pericarditis undergo


spontaneous resolution of constrictive pericarditis or respond to medical therapy [3,49,50]. In a review of
212 patients with echocardiographic findings of constrictive pericarditis, 17 percent had follow-up studies
showing resolution at an interval ranging from two months to two years [50]. The most common cause of
transient constrictive pericarditis was pericardial inflammation after pericardiotomy (25 percent of cases);
infection (viral, bacterial, or tuberculous), idiopathic, collagen vascular disease, trauma, and malignancy
accounted for the remaining cases. Treatment had included nonsteroidal antiinflammatory drugs, steroids,
antibiotics, chemotherapy, and angiotensin converting enzyme inhibitors plus diuretics. Five patients had
resolution of constriction without any specific therapy.

Cardiac magnetic resonance (CMR) imaging, specifically the degree of late gadolinium enhancement
(LGE) of the pericardium, appears promising as a way to predict which patients with constrictive
pericarditis will have reversal or resolution of the process. In a retrospective cohort study of 29 patients
with constrictive pericarditis who underwent CMR prior to receiving antiinflammatory medications, 14 of
the 29 patients ultimately had resolution of constrictive pericarditis [41]. There was significantly greater
baseline LGE pericardial thickness (4 mm versus 2 mm) and greater LGE qualitative intensity in those
with transient constrictive pericarditis than in the group of patients who ultimately had persistent
constrictive pericarditis. LGE pericardial thickness 3 mm predicted reversibility of constriction with 86
percent sensitivity and 80 percent specificity.

The clinical course in these patients implies the presence of acute inflammatory pericarditis with
constriction due to inflammation that resolved after standard treatment with antiinflammatory agents for
acute pericarditis. For patients with newly diagnosed constrictive pericarditis who are hemodynamically
stable and without evidence of chronic constriction, we suggest a trial of conservative management rather
than pericardiectomy. Conservative management with anti-inflammatory agents can be continued for two
to three months before proceeding with pericardiectomy, if indicated. Patients with markers of chronic
constriction (eg, cachexia, atrial fibrillation, hepatic dysfunction, or pericardial calcification) or signs of
progressive systemic congestion (eg, dyspnea, unexplained weight gain, and/or a new or increased
pleural effusion or ascites) should undergo earlier surgical intervention. (See "Acute pericarditis: Clinical
presentation and diagnostic evaluation" and "Etiology of pericardial disease", section on 'Acute
pericarditis'.)

Chronic constrictive pericarditis Pericardiectomy is the only definitive treatment option for patients
with chronic constrictive pericarditis. Medical therapy (ie, diuretics) may be used as a temporizing
measure and for patients who are not candidates for surgery [3]. While the majority of patients have a
significant improvement in symptoms following pericardiectomy, there is a significant perioperative
morbidity and mortality. Outcomes are best at high volume surgical centers with greater experience
performing pericardiectomy.

Removal of the thickened and inflamed pericardium is technically challenging. Efforts should be made to
remove as much of the pericardium as is technically feasible [3,6,17,51]. Most patients have relief of
symptoms after pericardiectomy, with some evidence that earlier surgical intervention is associated with
better outcomes [9]. In one series, NYHA functional class improved markedly among long-term survivors
(mean follow-up four years), with 69 percent free of clinical symptoms [5]. Surgical removal of the

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pericardium has a significant operative mortality. In one series of patients who underwent surgery between
1970 and 1985, the operative mortality was 12 percent [4]. A lower mortality rate of between 4 and 8
percent has been noted in patients who underwent pericardiectomy between 1977 and 2012 [5-9,51-53].

Pericardiectomy is the accepted standard of treatment in patients with chronic constrictive pericarditis who
have persistent and prominent symptoms. Preoperative diuretics should be used sparingly with the goal of
reducing elevated venous pressure, ascites, and edema. This approach can help to optimize the patients
hemodynamics prior to surgery and may improve their functional status. Diuretics can also be considered
for the palliative control of symptoms in patients who are not candidates for surgery.

Due to the complex nature of the surgery and the associated operative mortality, surgery should be
considered cautiously in patients with either mild or very advanced disease and in those with radiation-
induced constriction, myocardial dysfunction, significant renal dysfunction, or mixed constrictive-restrictive
disease. Such patients may not benefit from pericardiectomy:

In patients with constrictive pericarditis, mild symptoms, and a mild to moderate increase in central
venous pressure with little or no edema, the surgical risk of pericardiectomy likely outweighs any
potential benefit. A trial of medical management can be considered in such patients, with
reassessment for pericardiectomy should symptoms progress [50].

In our experience, patients with "end-stage" constrictive pericarditis derive little or no benefit from
pericardiectomy and the operative risk is markedly elevated. Manifestations of end-stage disease can
include cachexia, reduced resting cardiac output (cardiac index 1.2 L/m2 per min), hypoalbuminemia
due to protein losing enteropathy, and/or liver dysfunction due to chronic congestion or cardiogenic
cirrhosis.

Symptoms may persist after successful pericardiectomy in patients with mixed constrictive-restrictive
disease (eg, radiation-induced disease) because of abnormalities in intrinsic myocardial compliance.
In these patients it is important to assess the extent of myocardial damage with tissue Doppler and/or
endomyocardial biopsy.

Long-term survival after pericardiectomy is inferior to that of an age- and sex-matched population,
although in many patients this is likely related to comorbid conditions [5,6,9]. In one series, the 5- and 10-
year survival rates were 78 and 57 percent, respectively [5]. Independent adverse predictors of long-term
outcome included older age and worse NYHA class. In other series, independent adverse predictors have
included older age, renal dysfunction, pulmonary hypertension, right ventricular dysfunction, left
ventricular dysfunction, concomitant coronary heart disease, chronic obstructive pulmonary disease, and
hyponatremia [6,54].

The etiology of the pericardial disease is also an important determinant of survival [5-8]. Prior ionizing
radiation, because it may induce myocardial injury as well as pericardial disease, is associated with poorer
long-term outcomes following surgery. In one series, the seven-year survival rates after surgery for
patients with idiopathic, postsurgical, and radiation-induced constrictive pericarditis were 88, 66, and 27
percent [6]. In two subsequent case series, the five-year survival rates after surgery for patients with
idiopathic, postsurgical, and post-radiation constrictive pericarditis were 80, 56, and 11 percent, and 81, 50
and 0 percent, respectively [7,8].

Preoperative indices of contraction and relaxation also predict postoperative prognosis. An observational
study of 40 patients undergoing preoperative cardiac catheterization reported that patients with both an
abnormal rate of left ventricular pressure decline (- LV dP/dt <1200 mmHg/s) and an abnormal time
constant of LV isovolumic relaxation (tau >50 ms) required more frequent postoperative inotropic support,
had higher immediate postoperative mortality, and significantly lower long-term survival (median follow-up
2.4 years) than patients with either two normal values or one abnormality [55]. Preoperative right

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ventricular systolic dysfunction also appears associated with postoperative morbidity and mortality [56].

OCCULT CONSTRICTIVE PERICARDITIS A report in 1977 described 19 patients with a syndrome


called occult constrictive pericardial disease [15]. The symptom complex of this proposed syndrome was
comprised of chest pain, dyspnea, and fatigue. Risk factors and examination findings consistent with
constrictive pericarditis in this patient population included a history of acute pericarditis in 12 patients
(which was recurrent in five), pericardial calcification in 2 patients, and nonspecific repolarization changes
in 16 patients. A plausible cause for pericardial disease was present in 10. The authors proposed that very
mild constrictive pericarditis can cause these symptoms in the absence of abnormal physical or
hemodynamic findings when the patient is evaluated in the basal state.

To test this hypothesis, they measured hemodynamics invasively before and after infusing a liter or warm
saline over a period of six to eight minutes to determine if occult constriction would then become overt. Six
patients known not to have heart disease and 12 patients with myocardial disease served as controls.

The results can be summarized as follows:

Saline infusion caused an elevation and equalization of ventricular filling pressures, and development
of pressure waveforms in diastole characteristic of constrictive pericarditis (waveform 3) in the
patients presumed to have occult constriction.

Ventricular filling pressures and diastolic waveform were unaltered in the subjects free from heart
disease.

The patients with myocardial disease developed elevated ventricular filling pressures, but unequally
on the two sides.

Eleven of the symptomatic patients underwent pericardiectomy with dramatic improvement. All 11 cases
had mild gross or histologic evidence of pericardial disease. The fluid challenge was repeated
postoperatively in five of the patients, with normal hemodynamic findings.

Despite these promising findings, little has subsequently been published on occult constrictive pericardial
disease, and no additional case series or randomized studies have replicated these results. Even though
the above study was conducted using high fidelity pressure tracings in a laboratory well known for high-
quality hemodynamic studies, it is unclear why or how such mild constriction could cause disabling
symptoms, or why chest pain was a feature. Furthermore, the dramatic relief by pericardiectomy is
unexplained in these patients with normal venous pressures that were modestly elevated by a rapid fluid
challenge, and in whom the cardiac output was normal at rest and was not changed by the infusion.

A saline infusion test looking for occult constrictive pericarditis is seldom indicated and, if performed, the
results should not be the sole evidence leading to pericardiectomy. Patients suspected of having occult
pericardial constrictive disease should undergo cardiac catheterization, including measurement of oxygen
consumption during progressive bicycle exercise. A study of this nature would document exertional
dyspnea and fatigue, and may help clarify the responsible mechanism. Rather than infusing fluid, diuretic
therapy can be suspended for several days prior to cardiac catheterization.

EFFUSIVE CONSTRICTIVE PERICARDITIS The pericardial cavity is typically obliterated in patients


with constrictive pericarditis. Thus, even the normal amount of pericardial fluid is absent. However,
pericardial effusion may be present in some cases. In this setting, the scarred pericardium not only
constricts the cardiac volume but can also put pericardial fluid under increased pressure, leading to signs
suggestive of cardiac tamponade. Pericardial pathology consistent with constrictive pericarditis with a
concomitant effusion is called effusive constrictive pericarditis [1,4,57-59]. (See "Cardiac tamponade".)

Incidence and etiology Effusive constrictive pericarditis appears to be relatively uncommon, although

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there are only limited published data. In one series of 95 patients undergoing surgery for constrictive
pericarditis, 24 percent were diagnosed with effusive constrictive pericarditis [4]. In another series of 190
patients with cardiac tamponade who underwent pericardiocentesis and cardiac catheterization, effusive
constrictive pericarditis was diagnosed in 15 patients (8 percent) [58].

Most cases of effusive constrictive pericarditis are idiopathic, reflecting the frequency of idiopathic
pericardial disease in general [4,58]. Other reported causes include radiation, malignancy, chemotherapy,
infection, and postsurgical pericardial disease. Tuberculosis is a frequent cause of effusive constrictive
pericarditis in regions where tuberculosis is common [60]. In a series of 68 patients with tuberculous
pericardial effusion, effusive-constrictive disease was present in just over half; compared with patients with
pericardial effusion without constrictive physiology, pre-pericardiocentesis right atrial pressure and
pericardial and serum IL-10 levels were higher among patients with effusive constrictive pericarditis [61].
(See "Tuberculous pericarditis".)

Clinical features Patients with effusive constrictive pericarditis usually present with clinical features of
pericardial effusion or constrictive pericarditis or both. The following clinical observations were made in
one series of 15 patients [58]:

Symptoms were usually present for less than three months (range 4 days to 26 months)
All patients had jugular venous distention and hepatomegaly
Eight patients had pericardial chest pain, fever, and a pericardial rub
Pulsus paradoxus was seen in 10 patients
All patients were in normal sinus rhythm
No patients had pericardial calcification

A number of clinical clues suggest that a patient with suspected constrictive pericarditis may actually have
effusive constrictive pericarditis:

Pulsus paradoxus is often present; this finding is uncommon in classical constrictive pericarditis
because the inspiratory decline in intrathoracic pressure is not transmitted to the right heart
chambers. (See 'Echocardiography' above.)
A pericardial knock is absent.
The Y descent is less marked than expected. (See 'Hemodynamic evaluation' above.)
Kussmaul's sign is frequently absent.

Diagnosis The diagnosis of effusive constrictive pericarditis often becomes apparent during
pericardiocentesis in patients initially considered to have uncomplicated cardiac tamponade. In such
cases, the right atrial pressure remains abnormal after removal of the pericardial effusion due to
constriction by the visceral pericardium. Right heart pressures and systemic arterial blood pressure should
be monitored simultaneously during elective pericardiocentesis in order to allow for the detection of
effusive constrictive pericarditis [62]. (See "Diagnosis and treatment of pericardial effusion", section on
'Treatment'.)

Prior to pericardiocentesis, effusive constrictive pericarditis is suggested by the unexpected persistence of


the v wave of right atrial pressure. The diagnosis is further supported by the following findings on the right
atrial pressure tracing after pericardiocentesis [58,59]:

Persistence of elevated right atrial pressure despite lowering of the pericardial pressure to near zero
with pericardiocentesis
The development of a marked, rapid y descent
The lack of an inspiratory decline in right atrial pressure

By definition, in patients with effusive constrictive pericarditis, pericardiocentesis fails to decrease the right

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atrial pressure by 50 percent or to a level below 10 mmHg [58]. A persistently elevated right atrial
pressure after pericardiocentesis may also be due to right heart failure or tricuspid regurgitation. Thus,
appropriate studies should be performed to exclude these disorders before making the diagnosis of
effusive constrictive pericarditis.

Noninvasive imaging is not useful to confirm the diagnosis of effusive constrictive pericarditis [59]. The
visceral layer of pericardium, which is responsible for the constrictive component of this process, is not
typically thickened to a degree that is detectable on imaging studies.

Treatment and course Pericardiocentesis alone may produce at least temporary relief of symptoms in
patients with effusive constrictive pericarditis, although it does not fully reverse the underlying condition. In
a series of 15 patients, marked improvement after pericardiocentesis was noted in five, mild improvement
in eight, and no benefit in two [58].

In effusive constrictive pericarditis, it is mainly the visceral layer of pericardium, not the parietal layer,
which constricts the heart. Thus, if surgery is required, a visceral pericardiectomy must be performed [59].
However, the visceral component of the pericardiectomy is often difficult, requiring sharp dissection of
many small fragments until an improvement in ventricular motion is observed [58]. Thus, pericardiectomy
for effusive constrictive pericarditis should be performed only at centers with experience in
pericardiectomy for constrictive pericarditis.

INFORMATION FOR PATIENTS UpToDate offers two types of patient education materials, "The
Basics" and "Beyond the Basics." The Basics patient education pieces are written in plain language, at the
5th to 6th grade reading level, and they answer the four or five key questions a patient might have about a
given condition. These articles are best for patients who want a general overview and who prefer short,
easy-to-read materials. Beyond the Basics patient education pieces are longer, more sophisticated, and
more detailed. These articles are written at the 10th to 12th grade reading level and are best for patients
who want in-depth information and are comfortable with some medical jargon.

Here are the patient education articles that are relevant to this topic. We encourage you to print or e-mail
these topics to your patients. (You can also locate patient education articles on a variety of subjects by
searching on "patient info" and the keyword(s) of interest.)

Beyond the Basics topic (see "Patient education: Pericarditis (Beyond the Basics)")

SUMMARY AND RECOMMENDATIONS

The critical pathophysiologic features of constrictive pericarditis, which are responsible for the
physical exam, hemodynamic, and imaging findings, are (see 'Physiology' above):

Greatly enhanced ventricular interaction or interdependence

The dissociation of intracardiac and intrathoracic pressures

The vast majority of patients with constrictive pericarditis display elevated jugular venous pressure
(JVP) on physical examination. Other important but less common features include pulsus paradoxus,
Kussmauls sign, a pericardial knock, edema, ascites, and/or cachexia. (See 'Clinical presentation'
above.)

Patients with suspected constrictive pericarditis should undergo initial evaluation with
electrocardiography, chest radiography, and echocardiography. (See 'Evaluation' above.)

Additional testing is often required if the diagnosis of constrictive pericarditis remains uncertain or if
surgical intervention is planned. We suggest using CMR imaging in patients being considered for
pericardiectomy to provide additional detailed anatomic information that might alter the decision to

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proceed with surgery based on the likelihood of resolution with medical therapy alone. Computed
tomography (CT) can provide additional detailed anatomic information about the extent of pericardial
thickening and scarring. Invasive hemodynamic evaluation during cardiac catheterization can confirm
the diagnosis and concurrent coronary angiography defines the patients coronary anatomy prior to
possible surgical intervention. (See 'Evaluation' above.)

Common findings on imaging studies include pericardial thickening with or without calcification,
dilatation of the inferior vena cava and hepatic veins (plethora) with absent or diminished inspiratory
collapse, abnormal passive filling of the ventricles during early diastole, and pronounced respiratory
variation in ventricular filling. (See 'Evaluation' above.)

The symptoms and physical findings in patients with constrictive pericarditis are similar to those of
several other disorders. Most importantly, the clinician must distinguish between constrictive
pericarditis, which is treated by pericardiectomy; cardiac tamponade, which is treated by drainage of
the pericardial effusion; and disorders such as restrictive cardiomyopathy and cirrhosis, which require
markedly different treatment. (See 'Differential diagnosis' above.)

For patients with newly diagnosed constrictive pericarditis who are hemodynamically stable and
without evidence of chronic constriction (ie, no evidence of cachexia, weight loss, reduced cardiac
output at rest, or hypoalbuminemia due to protein losing enteropathy and/or impaired hepatic function
due to chronic congestion or cardiogenic cirrhosis), we suggest a trial of conservative management
rather than pericardiectomy (Grade 2C). (See 'Transient constrictive pericarditis' above.)

Pericardiectomy is the only definitive treatment option for patients with chronic symptomatic
constrictive pericarditis. Medical therapy (ie, diuretics) may be used as a temporizing measure and for
patients who are not candidates for surgery. While the majority of patients have a significant
improvement in symptoms following pericardiectomy, there is a significant perioperative morbidity and
mortality. Outcomes are best at high-volume surgical centers with greater experience performing
pericardiectomy. (See 'Chronic constrictive pericarditis' above.)

Pericardial pathology consistent with constrictive pericarditis with a concomitant effusion is called
effusive constrictive pericarditis. The distinction from isolated cardiac tamponade becomes apparent
by the unexpected persistence of an elevated right atrial pressure following pericardiocentesis. (See
'Effusive constrictive pericarditis' above.)

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unrecognized pattern of evolution in effusive acute idiopathic pericarditis. Am J Cardiol 1987; 59:961.
50. Haley JH, Tajik AJ, Danielson GK, et al. Transient constrictive pericarditis: causes and natural
history. J Am Coll Cardiol 2004; 43:271.
51. 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.
52. DeValeria PA, Baumgartner WA, Casale AS, et al. Current indications, risks, and outcome after
pericardiectomy. Ann Thorac Surg 1991; 52:219.
53. Ghavidel AA, Gholampour M, Kyavar M, et al. Constrictive pericarditis treated by surgery. Tex Heart
Inst J 2012; 39:199.
54. Busch C, Penov K, Amorim PA, et al. Risk factors for mortality after pericardiectomy for chronic
constrictive pericarditis in a large single-centre cohort. Eur J Cardiothorac Surg 2015; 48:e110.
55. Ha JW, Oh JK, Schaff HV, et al. Impact of left ventricular function on immediate and long-term
outcomes after pericardiectomy in constrictive pericarditis. J Thorac Cardiovasc Surg 2008;
136:1136.
56. Choudhry MW, Homsi M, Mastouri R, et al. Prevalence and Prognostic Value of Right Ventricular
Systolic Dysfunction in Patients With Constrictive Pericarditis Who Underwent Pericardiectomy. Am J
Cardiol 2015; 116:469.
57. Hancock EW. On the elastic and rigid forms of constrictive pericarditis. Am Heart J 1980; 100:917.
58. Sagrist-Sauleda J, Angel J, Snchez A, et al. Effusive-constrictive pericarditis. N Engl J Med 2004;
350:469.
59. Hancock EW. A clearer view of effusive-constrictive pericarditis. N Engl J Med 2004; 350:435.
60. Hugo-Hamman CT, Scher H, De Moor MM. Tuberculous pericarditis in children: a review of 44
cases. Pediatr Infect Dis J 1994; 13:13.
61. Ntsekhe M, Matthews K, Syed FF, et al. Prevalence, hemodynamics, and cytokine profile of effusive-
constrictive pericarditis in patients with tuberculous pericardial effusion. PLoS One 2013; 8:e77532.
62. Shabetai R. The Pericardium, Kluwer Academic Publishers, Norwell, MA 2003. p.227.

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GRAPHICS

Jugular venous pulse in constrictive pericarditis

The normal jugular venous pulse waveform is represented in blue. In


comparison, the jugular venous pulse waveform in a patient with constrictive
pericarditis (CP) is shown in red. Note the sharp, prominent y descent in CP,
which represents rapid passive filling during early diastole that abruptly stops
when the ventricular volume reaches the maximal capacity allowed by the stiff,
thickened pericardium.

Graphic 80995 Version 2.0

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Pulsus alternans and pulsus paradoxus arterial waveforms

In pulsus alternans, which is associated with severe left ventricular systolic dysfunction, there is
variation in the amplitude of the systolic arterial pressure with every other beat. In contrast,
pulsus paradoxus varies with the respiratory cycle, such that the duration of arterial pressure
reduction is sustained during the inspiratory portion of the respiratory cycle.

Courtesy of Barry A Borlaug, MD.

Graphic 70731 Version 5.0

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Lateral chest radiograph (CXR) showing pericardial


calcium

This lateral chest radiograph demonstrates coarse calcification involving the


entire pericardium (arrows) in this patient who has a history of prior
hemopericardium.

Photo courtesy of Jonathan Kruskal, MD.

Graphic 64005 Version 3.0

Normal lateral chest radiograph

Courtesy of Steven Weinberger, MD.

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Dip and plateau in constrictive pericarditis

Hemodynamic record of a patient with surgically proven constrictive pericarditis.


(A) Slow-paper-speed recording of high-gain left ventricular (LV) pressure and simultaneous right heart
pullback from pulmonary capillary wedge (PCW) to pulmonary artery (PA), right ventricle (RV), and
right atrium (RA).
(B) Fast-paper-speed recording of LV and simultaneous RV and RA pressure tracings. Note the
increased and equal atrial and diastolic pressures, the prominent X and Y descents on the RA tracing,
and the dip and plateau on the RV and LV tracings during longer diastoles.

From: Hoit BD. Pericardial disease and pericardial heart disease. In: O'Rourke RA, ed. Stein's Internal
Medicine, 5th Edition. St. Louis: Mosby-Year Book, 1998. Illustration used with the permission of Elsevier Inc.
All rights reserved.

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Equalization of pressures in severe constrictive


pericarditis

Tracing from a patient with severe constrictive pericarditis shows that the wedge
and right atrial pressures are equal during inspiration, but that the wedge
pressure is higher during expiration.

Courtesy of Ralph Shabetai, MD.

Graphic 80355 Version 2.0

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Algorithmic approach to the differentiation of constrictive pericarditis


versus restrictive cardiomyopathy.

BNP: brain natriuretic peptide; CMR: cardiovascular magnetic resonance; CP: constrictive pericarditis; CT:
computed tomography; ECG: electrocardiogram; RCM: restrictive cardiomyopathy.

Graphic 99929 Version 1.0

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Saline loading unmasks occult constrictive pericarditis

Right atrial pressure tracings before (top panel) and after (bottom panel) saline
loading in a patient with occult constrictive pericarditis. The previously normal
right atrial pressure developed the characteristics of constrictive pericarditis
(with equalization of pressures after fluid challenge.

Redrawn from: Bush CA, Stang JM, Wooley CF, Kilman JW. Circulation 1977; 56:924.

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Contributor Disclosures
Brian D Hoit, MD Speaker's Bureau: Philips Medical [Heart valve disease (3D Transesophageal
echo)]. Martin M LeWinter, MD Nothing to disclose Gabriel S Aldea, MD Nothing to disclose Edward
Verrier, MD Nothing to disclose Brian C Downey, MD, FACC Nothing to disclose

Contributor disclosures are reviewed for conflicts of interest by the editorial group. When found, these are
addressed by vetting through a multi-level review process, and through requirements for references to be
provided to support the content. Appropriately referenced content is required of all authors and must
conform to UpToDate standards of evidence.

Conflict of interest policy

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