Constrictive Pericarditis - Clinical Features and Causes - UpToDate

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Constrictive pericarditis: Clinical features and causes

AUTHOR: Brian D Hoit, MD
SECTION EDITOR: Martin M LeWinter, MD
DEPUTY EDITOR: Susan B Yeon, MD, JD
All topics are updated as new evidence becomes available and our peer review process is complete.
Literature review current through: Jan 2024.

This topic last updated: Dec 02, 2022.
INTRODUCTION
The clinical presentation and pathophysiology of constrictive pericarditis and effusive-

constrictive pericarditis are reviewed here. Related issues are discussed separately.
● (See "Constrictive pericarditis: Diagnostic evaluation".)
● (See "Constrictive pericarditis: Management and prognosis".)
● (See "Pericardial effusion: Approach to diagnosis" and "Cardiac tamponade".)
● (See "Acute pericarditis: Clinical presentation and diagnosis" and "Acute pericarditis:
Treatment and prognosis" and "Recurrent pericarditis".)
DEFINITIONS
● Constrictive pericarditis – Constrictive pericarditis is a clinical syndrome in which an

inelastic thickened pericardium restricts cardiac filling [1-3]. The course of this condition
may be chronic or transient:
• Chronic constrictive pericarditis – Most constrictive pericarditis has a chronic

course, with constriction persisting for greater than three to six months. Pericardial
effusion is rarely a prominent feature of chronic constrictive pericarditis. (See
'Constrictive pericarditis' below.)
https://www-uptodate-com.bibliotecavirtual.udla.edu.ec/contents/constrictive-pericarditis-clinical-features-and-causes/print?search=pericarditis&… 1/20
• Transient constrictive pericarditis – In a minority of cases of constrictive

pericarditis, the constriction resolves spontaneously or with medical therapy, with
most such cases resolving within three to six months. Cases of transient constrictive
pericarditis commonly present as effusive-constrictive pericarditis. (See 'Transient
constrictive pericarditis' below.)
● Effusive-constrictive pericarditis – Effusive-constrictive pericarditis is a clinical

syndrome caused by constrictive pericarditis and coexisting (often hemodynamically
significant) pericardial effusion [4-8]. This syndrome overlaps with transient constrictive
pericarditis. (See 'Effusive-constrictive pericarditis' below.)
PERICARDIAL PHYSIOLOGY
The pericardium consists of a visceral monolayer adherent to the epicardial surface of the
heart, and the parietal pericardium, a fibroelastic layer that is continuous with the visceral
pericardium and forms a sac surrounding the heart that contains a thin layer of fluid. In the
absence of pericardial disease, the pericardium stretches to accommodate physiologic
changes in cardiac volume (but is noncompliant if its limited reserve volume is exceeded). In
the absence of pericardial disease, intrathoracic and right heart pressures decrease during
inspiration, leading to an increase in venous return to the right heart and a 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 (LV) filling.
PATHOPHYSIOLOGY
Constrictive pericarditis — With pericardial constriction, cardiac filling is impeded by an

external force (ie, the virtually inelastic parietal and/or visceral pericardial tissue, which is
thickened, fibrotic, and sometimes calcified). This results in a markedly impaired ability to
accommodate cardiac volume changes.
The key pathophysiologic features of constrictive pericarditis, which are largely responsible
for physical examination, hemodynamic, and imaging findings, are elevated venous
pressure, greatly enhanced ventricular interdependence, and the dissociation of intracardiac
and intrathoracic pressures.
● Limited late diastolic filling – With constrictive pericarditis, early diastolic filling is
even more rapid than in the absence of pericardial disease. Compression does not
occur until the cardiac volume approaches the limits of the inelastic pericardium, which
begins in middiastole. 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.
● Dissociation of intracardiac and intrathoracic pressures – The thickened, rigid

pericardium prevents the normal inspiratory decrease in intrathoracic pressure from
being transmitted to the heart chambers, although this is not present in all cases. The
rigid pericardium also does not expand to accommodate increased venous return to
the right heart during inspiration. Thus, pulmonary venous pressure, but not LV
pressure, declines during inspiration, leading to a reduction in LV volume because of a
decrease in the transpulmonary gradient.
● Enhanced ventricular interdependence – The right and left ventricles are

interdependent, as these chambers share a septum, epicardial circumferential
myocytes, and pericardial space [9]. In the setting of constrictive pericarditis, the
external constraint of the pericardium enhances ventricular interdependence (ie, the
hemodynamics of the left and right ventricles are influenced by each other to a much
greater degree than in the nondiseased state). With enhanced ventricular
interdependence, the right ventricular volume expands via shifting of the
interventricular septum toward the LV. (See "Constrictive pericarditis: Diagnostic
evaluation", section on 'Cardiac catheterization' and "Constrictive pericarditis:
Diagnostic evaluation", section on 'Doppler findings'.)
The hemodynamic findings and distinction between constrictive pericarditis and restrictive
cardiomyopathy ( algorithm 1 and table 1) are discussed in more detail elsewhere. (See
"Differentiating constrictive pericarditis and restrictive cardiomyopathy".)
Effusive-constrictive pericarditis — In effusive-constrictive pericarditis, the noncompliant

pericardium abnormally restrains the cardiac volume and also elevates the pressure in the
pericardial fluid, such that tamponade can occur with a relatively small pericardial effusion
[4-7,10]. This results in a variable hemodynamic picture with features ranging from
predominantly those of cardiac tamponade to those of constrictive pericarditis. Most cases
present with mixed features. (See 'Effusive-constrictive pericarditis' below.)
INCIDENCE AND CAUSES
Constrictive pericarditis — Constrictive pericarditis can occur after virtually any pericardial
disease process [1,2,11]. The etiology of constrictive pericarditis varies widely depending on
the population studied, with idiopathic or posttreatment (eg, postoperative or postradiation)
causes being more common in resource-abundant countries and infectious etiologies being
more prominent in resource-limited countries. The following causes of constrictive
pericarditis were identified in case series from tertiary care centers primarily in resource-
abundant countries [5,12-16]:
● Idiopathic or viral – Constrictive pericarditis was attributed to idiopathic or viral causes

in 42 to 61 percent of cases.
While constrictive pericarditis and recurrent pericarditis are both commonly attributed
to idiopathic or viral causes, constrictive pericarditis is rarely a sequela of recurrent
idiopathic pericarditis.
● Post-cardiac surgery – 11 to 37 percent. Cases of constrictive pericarditis following

orthotopic heart transplant have also been reported [17].
● Post-radiation therapy – 2 to 31 percent, primarily after Hodgkin disease or breast

cancer.
● Systemic rheumatic diseases (also known as autoimmune diseases) – 3 to 7

percent. Constrictive pericarditis is a rare complication of these disorders. (See
"Pericardial involvement in systemic autoimmune diseases".)
● Postinfectious (purulent pericarditis including tuberculosis) – 3 to 15 percent.
Tuberculosis is the leading cause of constrictive pericarditis in resource-limited

countries; it is now a rare cause in resource-abundant countries [3]. Tuberculosis should
be considered as a possible etiology among patients at high risk of developing active
tuberculosis, including those in or from endemic areas and those with human
immunodeficiency virus (HIV) infection. (See "Tuberculous pericarditis".)
Whipple disease (caused by Tropheryma whipplei infection) is an uncommon cause of

constrictive pericarditis [18].
● Miscellaneous causes (malignancy, trauma, drug-induced, asbestosis, sarcoidosis,

uremic pericarditis) – 1 to 10 percent. These are all rare causes of constrictive
pericarditis.
In addition, patients with a newly recognized systemic multiorgan fibroinflammatory

disease associated with elevated serum levels of immunoglobulin G4 (IgG4) may
present with constrictive pericarditis; histopathologically, the pericardium is fibrotic
with IgG4-positive lymphoplasmacytic infiltration [19].
Another rare cause of constrictive pericarditis is Myhre syndrome [20].
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)
[21]. Constrictive pericarditis developed in 2 of 416 patients with idiopathic/viral pericarditis

(0.48 percent) and 7 of 84 patients with a nonviral/nonidiopathic etiology (8.3 percent). The
incidence rates of constrictive pericarditis were:
● Idiopathic/viral – 0.76 cases per 1000 person-years

● Autoimmune/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
● Other purulent pericarditis – 52.75 cases per 1000 person-years
Transient constrictive pericarditis — A subset of patients with constrictive pericarditis

undergoes spontaneous resolution of constrictive pericarditis or responds to medical
therapy [1,22,23]. As an example, in a series 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 [23]. Some degree of effusion is common in patients
with transient constrictive pericarditis, so there is some overlap between this syndrome and
effusive-constrictive pericarditis.
The causes of transient constrictive pericarditis are similar to those for effusive-constrictive
pericarditis (see 'Effusive-constrictive pericarditis' below), as there is overlap between these
conditions. In a series of 36 patients with transient constrictive pericarditis, the most
common causes were pericardial inflammation after pericardiotomy (25 percent of cases);
infection (viral, bacterial, or tuberculous), idiopathic, systemic rheumatic disease, trauma,
and malignancy accounted for the remaining cases [23]. An effusive-constrictive process was
identified in the majority (67 percent) of these cases.
Effusive-constrictive pericarditis — Effusive-constrictive pericarditis appears to be

relatively uncommon but may be increasing in prevalence, although there are only limited
published data.
● In a series of 95 patients undergoing surgery for constrictive pericarditis, 24 percent

were diagnosed with effusive-constrictive pericarditis [5].
● In series of patients undergoing pericardiocentesis (of which all [6] or many had cardiac
tamponade [24]), coexistent effusive-constrictive pericarditis was diagnosed in 8 [6] and
16 percent [24].
The prevalence of effusive-constrictive pericarditis differs among the various etiologies of

pericarditis. Effusive-constrictive pericarditis is much more common among cases of
tuberculous pericarditis than among cases of idiopathic pericarditis [25,26]. In a series of 68
patients with tuberculous pericardial effusion, effusive-constrictive disease was present in
just over half [27].
In studies from resource-abundant countries, the etiology of effusive-constrictive pericarditis

has most commonly been idiopathic, reflecting the frequency of idiopathic pericardial
disease in these countries [5,6,26]. Other causes include radiation, malignancy,
chemotherapy, postsurgical and post-cardiac injury pericardial disease, infection
(tuberculosis and other purulent pericarditis), and end-stage kidney disease [26].
However, the relative frequency of causes of effusive-constrictive pericarditis has varied

considerably among individual case series. Thus, in one report of 33 cases of effusive-
constrictive pericarditis, the most frequent cause was procedure-related hemopericardium
(33 percent), followed by idiopathic (27 percent), post-cardiac surgery (18 percent), postviral
(9 percent), malignancy (6 percent), and other (6 percent) [24].
CLINICAL MANIFESTATIONS
Constrictive pericarditis
Clinical presentation — Patients with constrictive pericarditis typically present with one or
both of the following constellations of symptoms and signs [1]:
● Volume overload – Symptoms related to volume overload range from peripheral

edema to anasarca.
● Decreased cardiac output – Symptoms related to diminished cardiac output in

response to exertion include fatigability and dyspnea on exertion.
In a 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 5 percent with symptoms ascribed to decreased cardiac output (eg,
dyspnea, fatigue, and reduced exercise capacity) [12]. (See "Cardiac tamponade" and "Heart
failure: Clinical manifestations and diagnosis in adults", section on 'Clinical presentation'.)
The history elicited from a patient with suspected constrictive pericarditis should include
responses to focused questions directed at potential underlying causes (eg, history of prior
malignancy with thoracic radiation treatment, prior cardiothoracic surgery, chest trauma,
etc).
Key signs of constrictive pericarditis — 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 sign, a pericardial knock, peripheral edema, ascites, and/or cachexia.
● Elevated JVP – Elevated JVP has been reported in as many as 93 percent of patients
with surgically confirmed constrictive pericarditis [12]. The characteristics of the jugular
venous waveforms in patients with constrictive pericarditis are different from those in
healthy patients and in 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. In patients in normal sinus
rhythm, the resulting waveform is typically W- or M-shaped. (See "Examination of the
jugular venous pulse" and "Constrictive pericarditis: Diagnostic evaluation", section on
'Cardiac catheterization'.)
JVP may be in the normal range 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, JVP may rise only after volume expansion [28]. (See 'Occult
constrictive pericarditis' below.)
● Pulsus paradoxus – Pulsus paradoxus (an exaggerated drop in systolic blood pressure
greater than 10 mmHg during inspiration) occurs in less than 20 percent of patients
with constrictive pericarditis ( waveform 1) [12,29]. It is more common in patients
with effusive-constrictive pericarditis with coexisting pericardial effusion and cardiac
tamponade and it may be more common in patients with coexisting pulmonary
disease. (See "Pulsus paradoxus in pericardial disease".)
● Kussmaul sign – Kussmaul 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 incidence rates of 13 to 21 percent, Kussmaul sign is much more common in our
experience [5,12]. (See "Examination of the jugular venous pulse".)
● Pericardial knock – A medium frequency accentuated heart sound occurring slightly

earlier than an S3 may be audible and rarely is palpable. A pericardial knock was
reported in 47 percent of patients with constrictive pericarditis in one series [12].
Sixteen percent of patients in the same series had a pericardial friction rub.
● Other findings – 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.
Occult constrictive pericarditis — A syndrome of occult constrictive pericarditis has been

described, but few reports are available. The largest series, published in 1977, described 19
patients with chest pain, dyspnea, and fatigue presenting with initially normal physical and
hemodynamic findings [28]. Most of these patients had prior history and/or clinical findings
suggestive of pericardial disease, and subsequent hemodynamic evaluation after saline
infusion ( waveform 2) was consistent with unmasking of constrictive pericarditis with
volume loading.
Effusive-constrictive pericarditis — Patients with effusive-constrictive pericarditis can

present with clinical features of pericardial effusion or constrictive pericarditis or both [6,26].
Cardiac tamponade may or may not be present.
Patients with effusive-constrictive pericarditis generally have a subacute presentation with

symptoms lasting days or weeks [26], although more acute or chronic presentations have
been described [6]. Acute cases may present with symptoms and signs of cardiac tamponade
(such as hypotension) while subacute cases may present with fatigue, shortness of breath,
and lower extremity edema [26]. Pleuritic chest pain and fever are common [6]. Common
physical signs include jugular venous distention and hepatomegaly [6].
A number of clinical clues suggest that a patient with suspected constrictive pericarditis may
have effusive-constrictive pericarditis:
● Pulsus paradoxus is often present; this finding is relatively uncommon in classic

constrictive pericarditis because the inspiratory decline in intrathoracic pressure is not
transmitted to the right heart chambers. (See "Constrictive pericarditis: Diagnostic
evaluation", section on 'Echocardiography'.)
● A pericardial knock is absent.
● The y descent is less marked than expected. (See "Constrictive pericarditis: Diagnostic
evaluation", section on 'Cardiac catheterization'.)
● Kussmaul sign is frequently absent.
SOCIETY GUIDELINE LINKS
Links to society and government-sponsored guidelines from selected countries and regions
around the world are provided separately. (See "Society guideline links: Pericardial disease".)
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
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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
● Pathophysiology
• Constrictive pericarditis – With pericardial constriction, cardiac filling is impeded

by an external force (ie, the virtually inelastic parietal and/or visceral pericardial
tissue, which is thickened, fibrotic, and sometimes calcified). The key
pathophysiologic features of constrictive pericarditis, which are largely responsible
for physical examination, hemodynamic, and imaging findings, are elevated venous
pressure, greatly enhanced ventricular interdependence, and dissociation of
intracardiac and intrathoracic pressures. Pericardial effusion is not a prominent
feature of constrictive pericarditis. (See 'Constrictive pericarditis' above.)
• Effusive-constrictive pericarditis – In this condition, a variable-size pericardial

effusion is present, and the noncompliant pericardium both constricts the cardiac
volume and also elevates the pressure of the pericardial fluid, such that tamponade
can occur with a relatively small pericardial effusion. This results in a variable
hemodynamic picture with most cases presenting with mixed features of cardiac
tamponade and constrictive pericarditis. (See 'Effusive-constrictive pericarditis'
above.)
● Etiology
• Constrictive pericarditis – This condition can occur after virtually any pericardial
disease process, with the etiologies varying widely depending on the population
studied, with idiopathic or posttreatment (eg, postoperative or postradiation) causes
being more common in resource-abundant countries and infectious etiologies (eg,
tuberculosis) being more prominent in resource-limited countries. The risk of
constrictive pericarditis is highest after purulent or tuberculous pericarditis. (See
'Constrictive pericarditis' above.)
• Transient constrictive pericarditis – The causes of this condition are similar to

those for effusive-constrictive pericarditis, as there is overlap between these
conditions. (See 'Transient constrictive pericarditis' above.)
• Effusive-constrictive pericarditis – This condition has a wide variety of causes; the

frequency distribution differs between resource-abundant versus resource-limited
countries. In resource-abundant countries, idiopathic cases are the most common.
In resource-limited countries, tuberculosis is most common.
Effusive-constrictive pericarditis is more common among cases of tuberculous

pericarditis than among cases of idiopathic pericarditis. Pericardial inflammation
after pericardiotomy is also a common cause of transient constrictive pericarditis
and/or effusive-constrictive pericarditis. (See 'Effusive-constrictive pericarditis'
above.)
● Clinical presentation
• Constrictive pericarditis – Patients with constrictive pericarditis typically present

with symptoms related to fluid overload (eg, peripheral edema, anasarca) and/or
diminished cardiac output (eg, fatigability, dyspnea on exertion). Most patients have
elevated jugular venous pressure (JVP). Other important but less common features
include pulsus paradoxus, Kussmaul sign, a pericardial knock, edema, ascites,
and/or cachexia. (See 'Constrictive pericarditis' above.)
• Effusive-constrictive pericarditis – Patients with this condition usually present with

clinical features of pericardial effusion (with or without cardiac tamponade) or
constrictive pericarditis or both. In a patient with suspected constrictive pericarditis,
clinical findings suggestive of effusive-constrictive pericarditis include: presence of
pulsus paradoxus, absence of a pericardial knock, a less marked y descent than
typical for pericardial constriction, and absence of Kussmaul sign. (See 'Effusive-
constrictive pericarditis' above and "Examination of the jugular venous pulse".)
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Topic 140205 Version 3.0
GRAPHICS
Algorithmic approach to the differentiation of constrictive pericarditis versu

restrictive cardiomyopathy
ECG: electrocardiogram; BNP: B-type natriuretic peptide; CP: constrictive pericarditis; CMR:
cardiovascular magnetic resonance; CT: computed tomography; RCM: restrictive cardiomyopathy.
Graphic 99929 Version 3.0
Differentiating constrictive pericarditis and restrictive cardiomyopathy
Favors constrictive Favors restrictive

Parameter
pericarditis cardiomyopathy
Biomarkers
BNP <100 pg/mL >400 pg/mL
Echocardiographic parameters
Ventricular septal shift Present Absent

(bounce)*
Inspiratory change in IVRT Increased No change
Pericardial thickness Increased Normal
Respirophasic changes in Increased (as much as 30 to 40% Minimal (usually <10%)

ventricular filling velocity ¶ Δ variation)
Hepatic venous flow Reversal of forward flow during Reversal of forward flow during
expiration inspiration
Color M-mode flow >100 cm/second <45 cm/second

propagation
Early diastolic Doppler tissue Normal or increased (>12 Normal or decreased (<8
velocity E' at mitral annulus cm/second) cm/second)
"Annulus reversus" ◊ Yes No
Strain analysis (absolute >16% ≤10%

global longitudinal strain)
CMR parameters
Relative atrial volume ratio ≥1.5 ≤1.1

(LA/RA volume)
Late gadolinium Pericardial enhancement Myocardial enhancement

enhancement common in constrictive frequently noted in amyloidosis
pericarditis
Global longitudinal strain Normal Reduced
Ventricular interdependence >11% <8%

(cine CMR maximal septal
respiratory excursion)
Cardiac catheterization parameters
Respiratory change in LV and Discordant Concordant

RV pressures
RAP/PCWP ratio >0.77 ≤0.77
BNP: B-type natriuretic peptide; IVRT: isovolumic relaxation time; CMR: cardiovascular magnetic
resonance imaging; LA: left atrial; RA: right atrial; LV: left ventricular; RV: right ventricular; RAP: right
atrial pressure; PCWP: pulmonary capillary wedge pressure.
* When septal shift is combined with either medial E' ≥9 cm/s or hepatic vein expiratory reversal
≥79%, the sensitivity and specificity for constrictive pericarditis are 87 and 91%, respectively.
¶ The ventricular filling velocity is highly influenced by the level of LA pressure. When LA pressure is
greatly elevated in a patient with constrictive pericarditis, respiratory variation in ventricular filling
may not be observed, whereas patients with lower LA pressure (ie, due to volume depletion or earlier
stage of disease) may have more noticeable changes in ventricular filling velocities with respiration.
Δ The ability to assess respirophasic changes in ventricular filling velocities is challenging in patients
with atrial fibrillation due to the presence of variable cardiac cycle length from beat to beat.
◊ Unlike in normal individuals, mitral lateral (and tricuspid lateral) annular E' velocities are often
relatively reduced in patients with constrictive pericarditis ("annular reversus"). This reduction may be
the result of lateral adhesion of the pericardium while the longitudinal movement of the septal
annulus is unimpeded. These mechanics are not evident in restrictive cardiomyopathy.
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 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.
CP: constrictive pericarditis.
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.
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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23/2/24, 14:11 Constrictive pericarditis: Clinical features and causes - UpToDate

Official reprint from UpToDate®

Constrictive pericarditis: Clinical features and causes

Literature review current through: Jan 2024.

The clinical presentation and pathophysiology of constrictive pericarditis and effusive-

● (See "Constrictive pericarditis: Diagnostic evaluation".)

● (See "Constrictive pericarditis: Management and prognosis".)

● (See "Pericardial effusion: Approach to diagnosis" and "Cardiac tamponade".)

● Constrictive pericarditis – Constrictive pericarditis is a clinical syndrome in which an

• Chronic constrictive pericarditis – Most constrictive pericarditis has a chronic

• Transient constrictive pericarditis – In a minority of cases of constrictive

● Effusive-constrictive pericarditis – Effusive-constrictive pericarditis is a clinical

Constrictive pericarditis — With pericardial constriction, cardiac filling is impeded by an

with little or no filling subsequently. As constrictive pericarditis becomes more severe,

● Dissociation of intracardiac and intrathoracic pressures – The thickened, rigid

● Enhanced ventricular interdependence – The right and left ventricles are

Effusive-constrictive pericarditis — In effusive-constrictive pericarditis, the noncompliant

INCIDENCE AND CAUSES

● Idiopathic or viral – Constrictive pericarditis was attributed to idiopathic or viral causes

● Post-cardiac surgery – 11 to 37 percent. Cases of constrictive pericarditis following

● Post-radiation therapy – 2 to 31 percent, primarily after Hodgkin disease or breast

● Systemic rheumatic diseases (also known as autoimmune diseases) – 3 to 7

● Postinfectious (purulent pericarditis including tuberculosis) – 3 to 15 percent.

Tuberculosis is the leading cause of constrictive pericarditis in resource-limited

Whipple disease (caused by Tropheryma whipplei infection) is an uncommon cause of

● Miscellaneous causes (malignancy, trauma, drug-induced, asbestosis, sarcoidosis,

In addition, patients with a newly recognized systemic multiorgan fibroinflammatory

Another rare cause of constrictive pericarditis is Myhre syndrome [20].

[21]. Constrictive pericarditis developed in 2 of 416 patients with idiopathic/viral pericarditis

● Idiopathic/viral – 0.76 cases per 1000 person-years

Transient constrictive pericarditis — A subset of patients with constrictive pericarditis

Effusive-constrictive pericarditis — Effusive-constrictive pericarditis appears to be

● In a series of 95 patients undergoing surgery for constrictive pericarditis, 24 percent

The prevalence of effusive-constrictive pericarditis differs among the various etiologies of

In studies from resource-abundant countries, the etiology of effusive-constrictive pericarditis

However, the relative frequency of causes of effusive-constrictive pericarditis has varied

● Volume overload – Symptoms related to volume overload range from peripheral

● Decreased cardiac output – Symptoms related to diminished cardiac output in

● Pericardial knock – A medium frequency accentuated heart sound occurring slightly

● Other findings – Profound cachexia, peripheral edema, ascites, pulsatile hepatomegaly

Occult constrictive pericarditis — A syndrome of occult constrictive pericarditis has been

Effusive-constrictive pericarditis — Patients with effusive-constrictive pericarditis can

Patients with effusive-constrictive pericarditis generally have a subacute presentation with

● Pulsus paradoxus is often present; this finding is relatively uncommon in classic

● A pericardial knock is absent.

● Kussmaul sign is frequently absent.

SOCIETY GUIDELINE LINKS

INFORMATION FOR PATIENTS

• Constrictive pericarditis – With pericardial constriction, cardiac filling is impeded

• Effusive-constrictive pericarditis – In this condition, a variable-size pericardial

• Transient constrictive pericarditis – The causes of this condition are similar to

• Effusive-constrictive pericarditis – This condition has a wide variety of causes; the

Effusive-constrictive pericarditis is more common among cases of tuberculous

• Constrictive pericarditis – Patients with constrictive pericarditis typically present

• Effusive-constrictive pericarditis – Patients with this condition usually present with

Use of UpToDate is subject to the Terms of Use.

4. Hancock EW. Subacute effusive-constrictive pericarditis. Circulation 1971; 43:183.

9. Domínguez F, Fuster V, Fernández-Alvira JM, et al. Association of Sleep Duration and

22. Sagristà-Sauleda J, Permanyer-Miralda G, Candell-Riera J, et al. Transient cardiac

Algorithmic approach to the differentiation of constrictive pericarditis versu

Graphic 99929 Version 3.0

Differentiating constrictive pericarditis and restrictive cardiomyopathy