Constrictive Pericarditis - Management and Prognosis - UpToDate

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Constrictive pericarditis: Management and prognosis

AUTHOR: Brian D Hoit, MD
SECTION EDITORS: Martin M LeWinter, MD, Gabriel S Aldea, MD, Edward Verrier, 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: Mar 28, 2023.
INTRODUCTION
The management and prognosis of constrictive pericarditis and effusive-constrictive

pericarditis are reviewed here. Related issues are discussed separately.
● (See "Constrictive pericarditis: Diagnostic evaluation".)
● (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 condition may present
during subacute or chronic phases:
• Subacute constrictive pericarditis – Early stage (subacute) constrictive pericarditis

is suggested by the presence of chest pain, elevated erythrocyte sedimentation rate
or C-reactive protein, pericardial effusion, and pericardial delayed
hyperenhancement on cardiovascular magnetic resonance (CMR) imaging.
A subset of patients with subacute constrictive pericarditis have transient

constrictive pericarditis, meaning the constriction resolves spontaneously or with
medical therapy, with most such cases resolving within three to six months. Cases of
https://www-uptodate-com.bibliotecavirtual.udla.edu.ec/contents/constrictive-pericarditis-management-and-prognosis/print?search=pericarditis&… 1/22
transient constrictive pericarditis commonly present as effusive-constrictive

pericarditis. (See "Constrictive pericarditis: Clinical features and causes", section on
'Transient constrictive pericarditis'.)
• 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: Clinical features and causes", section on 'Constrictive
pericarditis'.)
● Effusive-constrictive pericarditis – Effusive-constrictive pericarditis is a clinical

syndrome caused by constrictive pericarditis and coexisting hemodynamically
significant pericardial effusion [4-9]. This syndrome overlaps with subacute and
transient constrictive pericarditis. (See "Constrictive pericarditis: Clinical features and
causes", section on 'Effusive-constrictive pericarditis'.)
PREVENTION
Measures to prevent constrictive pericarditis include prompt treatment of acute pericarditis

and recurrent pericarditis, drainage of pericardial effusions, and prophylactic colchicine
therapy in patients undergoing pericardiotomy.
● Promptly treat pericarditis – One goal of prompt effective treatment of acute and
recurrent pericarditis is to reduce the risk of subsequent constrictive pericarditis. The
risk of developing constrictive pericarditis after acute pericarditis varies depending
upon the cause of acute pericarditis [10]. The risk of constrictive pericarditis is highest
for purulent pericarditis (52.74 cases per 1000 person-years), tuberculous pericarditis
(31.65 cases per 1000 person-years), neoplastic pericarditis (6.33 cases per 1000
person-years), and systemic rheumatic disease/pericardial injury syndrome (4.40 cases
per 1000 person-years). When idiopathic/viral acute pericarditis is appropriately and
effectively treated, constrictive pericarditis rarely develops (0.76 cases per 1000 person-
years) [10]. (See "Acute pericarditis: Treatment and prognosis".)
Management of purulent pericarditis, tuberculous pericarditis, and neoplastic

pericarditis are discussed separately. (See "Tuberculous pericarditis", section on
'Treatment' and "Purulent pericarditis", section on 'Treatment'.)
● Prophylax against postpericardiotomy syndrome – In patients who have undergone

pericardiotomy, prophylactic colchicine therapy reduces the risk of postpericardiotomy
syndrome and thus reduces the number of patients at risk for late development of
constrictive pericarditis. Prevention of postpericardiotomy syndrome is discussed

separately. (See "Post-cardiac injury syndromes", section on 'Prevention'.)
● Drain effusions – Removal of infected pericardial fluid is a key component of the

treatment of some types of pericarditis.
• Purulent pericarditis – For purulent pericarditis, complete drainage of the infected

pericardial effusion is a key component of infection eradication and may reduce the
risk of development of pericardial constriction, as discussed separately. (See
"Purulent pericarditis", section on 'Pericardial drainage'.)
• Postpericardiotomy syndrome – Limited evidence suggests that draining

postpericardiotomy pericardial effusions may reduce the risk of subsequent chronic
constrictive pericarditis [11]. Observational studies suggest that surgical drainage of
postpericardiotomy pericardial effusions may decrease the risk of postoperative
atrial fibrillation by decreasing pericardial inflammation [12], but reduction in risk of
constrictive pericarditis has not been established. (See "Post-cardiac injury
syndromes".)
MANAGEMENT OF CONSTRICTIVE PERICARDITIS
The management of constrictive pericarditis differs depending upon whether the clinical
presentation suggests early (subacute) or chronic disease.
Treatment of early (subacute) disease — For patients with signs of early stage (subacute)
constrictive pericarditis who are hemodynamically stable and have no evidence of late
(chronic) constrictive pericarditis, we initiate a trial of medical therapy rather than immediate
pericardiectomy ( algorithm 1). As noted above, the presence of chest pain, elevated
erythrocyte sedimentation rate (ESR) or C-reactive protein (CRP), pericardial effusion, and
pericardial delayed hyperenhancement on CMR suggests a subacute process that may
respond to antiinflammatory therapy [13,14]. (See "Constrictive pericarditis: Diagnostic
evaluation", section on 'CMR'.)
The rationale for this approach is that some patients with a subacute presentation have
transient constrictive pericarditis which will resolve with medical therapy (or spontaneously)
without need for pericardiectomy [15].
Approach to subacute disease — Our approach to treatment is as follows:
● Treatment of specific cause – Medical therapy should be directed at the underlying

cause of effusive-constrictive pericarditis (eg, tuberculosis, neoplasm, or systemic
rheumatic diseases) whenever possible. (See "Purulent pericarditis", section on

'Treatment' and "Tuberculous pericarditis", section on 'Treatment'.)
● Treatment of inflammation – Antiinflammatory therapy is the mainstay of treatment

for patients with subacute constrictive pericarditis; our approach is as follows
( algorithm 1).
Doses of the antiinflammatory agents described below are the same as the doses used
in acute or recurrent pericarditis ( table 1), but the duration of drug therapy is
generally longer for constrictive pericarditis, although limited data are available on
treatment duration.
• Initial therapy – Initial treatment of subacute constrictive pericarditis is similar to

that of acute pericarditis, typically utilizing a combination of a nonsteroidal
antiinflammatory drug (NSAID) plus colchicine ( table 1). If there is a good
response, this regimen is continued for two to three months before attempting to
wean therapy. Glucocorticoid therapy ( table 1) may be used instead of the NSAID
in patients who have relative or absolute contraindications to NSAID therapy (eg,
pregnancy beyond 20 weeks, renal failure, concern for bleeding risk when combined
with oral anticoagulant).
Patients with marked improvement in symptoms and signs of constrictive

pericarditis, including normalization of CRP level, resolution of inflammation on
CMR, or normalization of pericardial thickness on computed tomography (CT) or
CMR following two to three months of initial therapy, can typically be slowly weaned
from therapy by tapering the NSAID to off and then discontinuing colchicine
( table 1). Surgical intervention generally is not necessary in these patients.
• Refractory disease – Patients with refractory symptoms and signs of subacute

constrictive pericarditis despite initial NSAID plus colchicine therapy are treated
similarly to patients with recurrent pericarditis. Such patients are generally treated
with a glucocorticoid plus colchicine for two to three months ( table 1) prior to
attempting to wean therapy. An interleukin 1 (IL-1) inhibitor (anakinra or rilonacept)
is a reasonable alternative to glucocorticoid plus colchicine in this setting.
Patients with marked improvement in symptoms and signs of constrictive

pericarditis, including normalization of CRP level, resolution of inflammation on
CMR, or normalization of pericardial thickness on CT or CMR after two to three
months on glucocorticoid plus colchicine therapy, can typically be slowly weaned by
tapering the glucocorticoid to off and then discontinuing colchicine ( table 1).
For patients with refractory symptoms and signs of subacute constrictive pericarditis
despite glucocorticoid plus colchicine therapy, we suggest treatment with anakinra
or rilonacept. If there is a good response, treatment with an IL-1 inhibitor is

generally continued for at least 4 to 12 months prior to attempting to taper therapy.
● Treatment of progression to chronic disease – Patients who progress and develop

evidence of late (chronic) constrictive pericarditis (eg, anasarca, atrial fibrillation,
hepatic dysfunction, or pericardial calcification), should be evaluated by cardiothoracic
surgery for pericardiectomy. This includes patients who have persistent symptoms and
signs of constrictive pericarditis despite at least 4 to 12 months of IL-1 inhibitor therapy.
(See 'Treatment of late (chronic) disease' below.)
Efficacy of treatment of subacute disease — The efficacy of antiinflammatory therapy in

the treatment of subacute constrictive pericarditis is supported by small observational
studies and indirect evidence from patients with acute or recurrent pericarditis. The limited
available literature does not strongly support a specific antiinflammatory regimen in patients
without a specific inflammatory disorder [15-18].
● Glucocorticoids and other antiinflammatory agents – Small single-institution case

series have described the use of antiinflammatory therapy (including glucocorticoids,
NSAIDs, and colchicine) in patients with subacute constrictive pericarditis [14-17]. In the
available reports, 35 to 100 percent of affected patients received antiinflammatory
therapies, with response rates ranging from approximately 45 to 60 percent.
As an example, in a series of 29 patients with constrictive pericarditis who were treated

with antiinflammatory medications (glucocorticoids in 62 percent, NSAIDs in 28
percent, and colchicine in 31 percent), 48 percent of treated patients had resolution of
constrictive pericarditis within 13 months of follow-up [16]. Causes of constrictive
pericarditis in this study included postsurgical (31 percent), systemic rheumatic disease
(10 percent), radiation-related (7 percent), and idiopathic or other (52 percent). After
antiinflammatory therapy, CRP and ESR improved considerably in patients with
reversible constrictive pericarditis, whereas there was little to no change in these
markers among patients with persistent constrictive pericarditis.
The use of glucocorticoids, NSAIDs, and colchicine in this setting is also supported by
indirect evidence from patients with acute and recurrent pericarditis. These data are
discussed separately. (See "Acute pericarditis: Treatment and prognosis", section on
'Medical therapies' and "Recurrent pericarditis", section on 'Pharmacologic therapy'.)
● IL-1 inhibitors – The efficacy of IL-1 inhibitor therapy in early constrictive pericarditis
was assessed in a prospective study of 39 patients with recurrent or incessant
glucocorticoid-dependent, colchicine-resistant pericarditis [18]. During follow-up,
constrictive pericarditis was diagnosed in eight patients (20 percent) and was more
common in those with an incessant course. Treatment with the anakinra at 100 mg/day
produced complete resolution in five patients within a median of 1.2 months. In the
remaining three patients, constrictive pericarditis became chronic, requiring
pericardiectomy within 2.8 months.
Additional indirect evidence supporting IL-1 inhibitor therapy comes from studies in
patients with recurrent pericarditis. These data are discussed separately. (See
"Recurrent pericarditis", section on 'Interleukin 1 inhibitors'.)
Treatment of late (chronic) disease — Since the symptoms and signs of chronic constrictive
pericarditis (eg, anasarca, atrial fibrillation, hepatic dysfunction, or pericardial calcification)
are generally progressive, most patients require surgical pericardiectomy.
Medical therapy — Medical therapy to relieve congestion (eg, cautious use of diuretics) is
used as a temporizing measure while preparing for pericardiectomy and is also indicated for
patients who are not candidates for surgery. Diuretics should be used sparingly with the goal
of reducing elevated venous pressure, ascites, and edema while awaiting surgical
intervention. This approach can help to optimize the patient's hemodynamics prior to
surgery and may improve their functional status. In patients who are candidates for
pericardiectomy, medical therapy should not delay proceeding with this procedure, as
outcomes may be worse if pericardiotomy is delayed [1,19].
Pericardiectomy
Indication — We suggest pericardiectomy for patients with chronic constrictive

pericarditis who meet all of the following criteria:
● Symptoms and signs have persisted despite at least 4 to 12 months of IL-1 inhibitor
therapy.
● Symptoms are moderate to severe (New York Heart Association [NYHA] functional class
III or IV ( table 2)).
● Constrictive pericarditis has not progressed to "end-stage." (See 'Patients unlikely to

benefit from pericardiectomy' below.)
● There is no concomitant restrictive cardiomyopathy (eg, due to radiation-induced

injury). (See "Restrictive cardiomyopathies".)
● There are no other comorbid conditions that would place the patient at high risk (eg,
end-stage kidney disease or ventricular systolic dysfunction). (See 'Patients unlikely to
benefit from pericardiectomy' below.)
Patients unlikely to benefit from pericardiectomy — Due to the complex nature of

the surgery and the associated operative mortality, surgery should be carefully considered in
patients who may not benefit from the procedure. This includes patients with either mild or
very advanced disease, in those with mixed constrictive-restrictive disease (including
radiation-induced constrictive pericarditis), and in other patients at high risk for mortality
after pericardiectomy.
● Mild disease – In patients with constrictive pericarditis, mild symptoms (NYHA

functional class II), 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 with antiinflammatory agents is an option for
such patients, with reassessment for pericardiectomy should symptoms progress [15].
(See 'Approach to subacute disease' above.)
● End-stage disease – 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, markedly
reduced resting cardiac output (cardiac index ≤1.2 L/m2 per min), hypoalbuminemia
due to protein-losing enteropathy, and/or congestive hepatopathy including cardiac
cirrhosis. (See "Congestive hepatopathy".)
● Mixed constrictive-restrictive disease – Symptoms may persist after successful

pericardiectomy in patients with mixed constrictive pericarditis and restrictive
cardiomyopathy (as occurs with radiation-induced disease) because of abnormalities in
intrinsic myocardial compliance, and long-term survival is limited (32 percent at 10
years in one series [20]). In patients who have received prior mediastinal irradiation, it
is important to assess the extent of myocardial damage with tissue Doppler and/or
endomyocardial biopsy.
Other patients with persistent symptoms may have had insufficient removal of
pericardial tissue, perhaps due to involvement of the visceral pericardium.
● Other high risk groups – Mortality after pericardiectomy is also high in individuals
with end-stage kidney disease or ventricular systolic dysfunction [1].
Procedure — Pericardiectomy involves removal of as much of the constricting parietal

and epicardial (visceral) layers as possible, including layers over the right atrium, superior
vena cava, inferior vena cava and the inferior part of the right ventricle adjacent to the
diaphragm, while preserving the bilateral phrenic nerves [1]. Complete pericardiectomy is
generally preferred to partial pericardiectomy since observational data suggest better
operative and long-term outcomes with complete pericardiectomy [21]. Redo
pericardiectomy is associated with high operative mortality (eg, 12.2 percent) [21,22].
The median sternotomy approach is commonly preferred as it enables more complete

removal of pericardium overlying the right atrium and vena cavae [21,23]. For purulent
pericarditis with effusive-constrictive pericarditis, the left anterolateral thoracotomy may be

preferred given the risk of sternal infection [23].
Pericardiectomy is performed with or without cardiopulmonary bypass (CPB). While some

observational studies have found an association between CPB use and risk of mortality,
other studies have found no association [21].
Outcomes — Most patients have relief of symptoms after pericardiectomy, with some
evidence that earlier surgical intervention is associated with better outcomes [19]. In one
series, NYHA functional class improved markedly among long-term survivors (mean follow-
up four years), with 69 percent free of clinical symptoms [24].
However, surgical removal of the pericardium is associated with a significant risk of operative
mortality, with reported rates ranging from 0 to 19 percent [22]. In one center, the 30-day
mortality rate fell from a historic rate of 13 percent to 5 percent after 1990 [25]. Some studies
suggest that outcomes after pericardiectomy for idiopathic constrictive pericarditis are
better than for pericardiectomy for radiation-induced or postpericardiotomy constrictive
pericarditis, although others have found no relationship between cause of constriction and
outcomes [21]. Outcomes are best at high-volume surgical centers with greater experience
performing pericardiectomy.
MANAGEMENT OF EFFUSIVE-CONSTRICTIVE PERICARDITIS
Management of effusive-constrictive pericarditis is similar to that for constrictive pericarditis

except that pericardiocentesis is generally the initial step.
● Pericardiocentesis – For patients with effusive-constrictive pericarditis,

pericardiocentesis alone may produce at least temporary relief of symptoms, although
it does not fully reverse the underlying condition. Overt cardiac tamponade has been
reported in approximately one-half of patients with effusive-constrictive pericarditis
[26] (see "Cardiac tamponade"). In a series of 15 patients, marked improvement after
pericardiocentesis was noted in five, mild improvement in eight, and no benefit in two
[7].
● Treatment of causes, inflammation, and symptoms – As for constrictive pericarditis,

treatment includes treatment of specific causes (eg, infection) and antiinflammatory
agents for patients with symptoms or signs suggestive of pericardial inflammation. In
cases of known inflammatory diseases (eg, rheumatoid arthritis), antiinflammatory
therapy should be tailored to the specific disorder. For patients without a known
specific disease (ie, idiopathic constrictive pericarditis), NSAIDS, colchicine, and
glucocorticoids have all been used with variable success, but data are limited. Diuretic
therapy is administered as needed for symptom control. (See 'Treatment of early

(subacute) disease' above.)
Since there is overlap between effusive-constrictive pericarditis and transient

pericarditis, a substantial number of patients with effusive-constrictive pericarditis do
not require pericardiectomy. Reported rates of pericardiectomy among patients with
effusive constrictive pericarditis have varied widely from 6 to 65 percent, with lower
rates in some later series, likely reflecting differences in patient populations [9].
● Pericardiectomy – Surgical removal of the pericardium is reserved for patients with

refractory symptoms and signs of chronic constrictive pericarditis (eg, anasarca, atrial
fibrillation, hepatic dysfunction, or pericardial calcification) who do not have an
excessive risk profile, as discussed above.
Removal of the thickened and inflamed pericardium in the setting of effusive-

constrictive pericarditis can be technically challenging. In effusive-constrictive
pericarditis, the visceral rather than the parietal layer of pericardium is often a
significant cause of the constrictive symptoms and physiology. Thus, if surgery is
performed, a visceral pericardiectomy may be required [8]. Efforts should be made to
remove as much of the pericardium as is technically feasible [1,27-29]. Removal of the
visceral pericardium is often difficult, requiring sharp dissection of many small
fragments until an improvement in ventricular motion is observed [7]. Thus,
pericardiectomy for effusive-constrictive pericarditis should be performed only at
centers with experience in pericardiectomy for constrictive pericarditis.
PROGNOSIS
Long-term survival after pericardiectomy for constrictive pericarditis is inferior to that of an

age- and sex-matched population, although in many patients this is likely related to
comorbid conditions [19,24,27]. In one series, the 5- and 10-year survival rates were 78 and
57 percent, respectively [24]. Independent adverse predictors of long-term outcome included
older age and worse New York Heart Association class. In other series, independent adverse
predictors have included older age, renal dysfunction, pulmonary hypertension, right
ventricular (RV) dysfunction, left ventricular (LV) dysfunction, concomitant coronary artery
disease, chronic obstructive pulmonary disease, and hyponatremia [27,30].
The etiology of the pericardial disease is also an important determinant of survival

[24,27,31,32]. Prior ionizing radiation, because it may induce myocardial injury as well as
pericardial disease, is associated with poorer long-term outcomes following surgery [25]. 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 [27].
In two subsequent case series, the five-year survival rates after surgery for patients with
idiopathic, postsurgical, and postradiation constrictive pericarditis were 80, 56, and 11
percent, and 81, 50, and 0 percent, respectively [31,32].
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 LV 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 had significantly lower long-term survival (median follow-up 2.4 years) than patients
with either two normal values or one abnormality [33]. Preoperative RV systolic dysfunction
also appears associated with postoperative morbidity and mortality [34].
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
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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
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● Beyond the Basics topic (see "Patient education: Pericarditis (Beyond the Basics)")
SUMMARY AND RECOMMENDATIONS
● Definitions (See 'Definitions' above.)
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• Constrictive pericarditis is a clinical syndrome in which an inelastic thickened

pericardium restricts cardiac filling. The condition may be transient or chronic.
• Effusive-constrictive pericarditis is a clinical syndrome caused by constrictive

pericarditis and coexisting hemodynamically significant pericardial effusion. This
syndrome overlaps with transient constrictive pericarditis.
● Prevention – Measures to prevent constrictive pericarditis include prompt treatment of

acute pericarditis and recurrent pericarditis, prophylactic colchicine therapy in patients
undergoing pericardiotomy, and complete drainage of purulent pericardial effusions.
(See 'Prevention' above.)
● Treatment of the underlying condition – Medical therapy should be directed at the

underlying cause of effusive-constrictive pericarditis (eg, tuberculosis, neoplasm, or
systemic rheumatic diseases) whenever possible. (See "Purulent pericarditis", section
on 'Treatment' and "Tuberculous pericarditis", section on 'Treatment'.)
● Management of subacute constriction – Antiinflammatory therapy is the mainstay of

treatment for patients with signs of early stage (subacute) constrictive pericarditis.
Subacute constrictive pericarditis is generally manifested by chest pain, elevated C-
reactive protein (CRP) or erythrocyte sedimentation rate (ESR), and pericardial effusion
or pericardial delayed hyperenhancement on cardiovascular magnetic resonance (CMR)
imaging, in addition to symptoms and signs of pericardial constriction.
Our suggested treatment approach for such patients is as follows (see 'Treatment of
early (subacute) disease' above). Doses of antiinflammatory agents are the same as
those used for acute pericarditis, but the duration of therapy is generally longer for
constrictive pericarditis ( table 1 and algorithm 1):
• For initial treatment of patients with subacute constrictive pericarditis, we suggest a

combination of a nonsteroidal antiinflammatory drug (NSAID) plus colchicine (Grade
2C). If there is a good response, treatment is continued for two to three months
before attempting to taper therapy.
• For patients with refractory signs and symptoms despite initial NSAID plus colchicine
therapy, we suggest treatment with a glucocorticoid plus colchicine (Grade 2C). An
interleukin 1 (IL-1) inhibitor (anakinra or rilonacept) is a reasonable alternative. If
there is a good response, NSAID plus colchicine treatment is continued for two to
three months before attempting to taper therapy.
• For patients with refractory signs and symptoms despite glucocorticoid plus
colchicine therapy and those who have a prior history of recurrent or incessant
pericarditis, we suggest treatment with an IL-1 inhibitor (anakinra or rilonacept)
( table 1) (Grade 2C). If there is a good response, the IL-1 inhibitor is generally
continued for at least 4 to 12 months prior to attempting to taper therapy.
● Management of chronic constriction – Pericardiectomy is the only definitive

treatment for patients with late (chronic) constrictive pericarditis. Diuretic therapy is
used only as a temporizing measure and for patients who are not candidates for
surgery. (See 'Treatment of late (chronic) disease' above.)
We suggest pericardiectomy for patients with chronic constrictive pericarditis who meet
all of the following criteria (Grade 2C) (see 'Pericardiectomy' above):
• Symptoms and signs have persisted or recurred despite at least 4 to 12 months of

IL-1 inhibitor therapy.
• Symptoms are moderate to severe (New York Heart Association [NYHA] functional
class III or IV ( table 2)).
• Constrictive pericarditis has not progressed to "end-stage." (See 'Patients unlikely to

benefit from pericardiectomy' above.)
• There is no concomitant restrictive cardiomyopathy (eg, due to radiation-induced

injury). (See "Restrictive cardiomyopathies".)
• There are no other comorbid conditions that would place the patient at high risk (eg,
end-stage kidney disease or ventricular systolic dysfunction). (See 'Patients unlikely
to benefit from pericardiectomy' above.)
● Management of effusive constrictive pericarditis – Management of effusive-

constrictive pericarditis is similar to that for constrictive pericarditis except that
pericardiocentesis is generally the initial step. In effusive-constrictive pericarditis, the
visceral pericardium is often a significant contributor to constriction, so
pericardiectomy can be technically challenging. (See 'Management of effusive-
constrictive pericarditis' above.)
● Prognosis – Long-term survival after pericardiectomy for constrictive pericarditis is

impacted by concurrent conditions (such as kidney and lung disease), older age, and
the etiology of pericardial disease. Prior ionizing radiation is associated with worse
long-term outcomes because it is associated with myocardial as well as pericardial
disease. (See 'Prognosis' above.)
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14. Sato K, Ayache A, Kumar A, et al. Improvement in left ventricular mechanics following
medical treatment of constrictive pericarditis. Heart 2021; 107:828.
15. Haley JH, Tajik AJ, Danielson GK, et al. Transient constrictive pericarditis: causes and
natural history. J Am Coll Cardiol 2004; 43:271.
16. Feng D, Glockner J, Kim K, et al. Cardiac magnetic resonance imaging pericardial late
gadolinium enhancement and elevated inflammatory markers can predict the
reversibility of constrictive pericarditis after antiinflammatory medical therapy: a pilot
study. Circulation 2011; 124:1830.
17. Chang SA, Choi JY, Kim EK, et al. [18F]Fluorodeoxyglucose PET/CT Predicts Response to
Steroid Therapy in Constrictive Pericarditis. J Am Coll Cardiol 2017; 69:750.
18. Andreis A, Imazio M, Giustetto C, et al. Anakinra for constrictive pericarditis associated
with incessant or recurrent pericarditis. Heart 2020; 106:1561.
19. Vistarini N, Chen C, Mazine A, et al. Pericardiectomy for Constrictive Pericarditis: 20
Years of Experience at the Montreal Heart Institute. Ann Thorac Surg 2015; 100:107.
20. Pahwa S, Crestanello J, Miranda W, et al. Outcomes of pericardiectomy for constrictive
pericarditis following mediastinal irradiation. J Card Surg 2021; 36:4636.
21. Liu VC, Fritz AV, Burtoft MA, et al. Pericardiectomy for Constrictive Pericarditis: Analysis
of Outcomes. J Cardiothorac Vasc Anesth 2021; 35:3797.
22. Cho YH, Schaff HV, Dearani JA, et al. Completion pericardiectomy for recurrent
constrictive pericarditis: importance of timing of recurrence on late clinical outcome of
operation. Ann Thorac Surg 2012; 93:1236.
23. Depboylu BC, Mootoosamy P, Vistarini N, et al. Surgical Treatment of Constrictive

Pericarditis. Tex Heart Inst J 2017; 44:101.
24. Ling LH, Oh JK, Schaff HV, et al. Constrictive pericarditis in the modern era: evolving
clinical spectrum and impact on outcome after pericardiectomy. Circulation 1999;
100:1380.
25. Murashita T, Schaff HV, Daly RC, et al. Experience With Pericardiectomy for Constrictive
Pericarditis Over Eight Decades. Ann Thorac Surg 2017; 104:742.
26. Kim KH, Miranda WR, Sinak LJ, et al. Effusive-Constrictive Pericarditis After
Pericardiocentesis: Incidence, Associated Findings, and Natural History. JACC Cardiovasc
Imaging 2018; 11:534.
27. Bertog SC, Thambidorai SK, Parakh K, et al. Constrictive pericarditis: etiology and cause-
specific survival after pericardiectomy. J Am Coll Cardiol 2004; 43:1445.
28. Talreja DR, Edwards WD, Danielson GK, et al. Constrictive pericarditis in 26 patients with
histologically normal pericardial thickness. Circulation 2003; 108:1852.
29. 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.
30. 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.
31. Szabó G, Schmack B, Bulut C, et al. Constrictive pericarditis: risks, aetiologies and
outcomes after total pericardiectomy: 24 years of experience. Eur J Cardiothorac Surg
2013; 44:1023.
32. George TJ, Arnaoutakis GJ, Beaty CA, et al. Contemporary etiologies, risk factors, and
outcomes after pericardiectomy. Ann Thorac Surg 2012; 94:445.
33. 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.
34. 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.
Topic 140886 Version 4.0
GRAPHICS
Approach to treatment of subacute constrictive pericarditis in adults
This figure summarizes our suggested general approach to treating subacute constrictive pericarditis
in adult patients. Subacute constrictive pericarditis is generally manifested by chest pain, elevated CRP
or ESR, pericardial effusion, and pericardial delayed hyperenhancement on CMR in addition to
symptoms and signs of pericardial constriction. The treatment approach outlined above does not
apply to patients with chronic constrictive pericarditis, which is manifested by anasarca, atrial
fibrillation, hepatic dysfunction, and/or pericardial calcification. Refer to UpToDate topics on
constrictive pericarditis for additional detail, including guidance on dosing for the medications in this
figure and discussion of the evidence supporting their efficacy. Limited data are available on the
duration of drug therapies for constrictive pericarditis. Refer to UpToDate topics also for discussion of
treatment of effusive-constrictive pericarditis including pericardiocentesis and treatment of specific
causes of this condition (eg, tuberculosis, neoplasm, or systemic rheumatic disease).
NSAID: nonsteroidal antiinflammatory drug; IL-1: interleukin-1; CRP: serum C-reactive protein level;
ESR: erythrocyte sedimentation rate; CMR: cardiovascular magnetic resonance; CCT: cardiac computed
tomography.
* For patients with relative or absolute contraindications to NSAID, a glucocorticoid is an alternative to

an NSAID. In addition, it is reasonable to proceed directly to treatment with glucocorticoid plus
colchicine if the patient has recently been treated with a course of an NSAID plus colchicine (eg, for
treatment of acute or recurrent pericarditis).
¶ For patients with prior recurrent or incessant pericarditis, an IL-1 inhibitor is a reasonable
alternative to NSAID plus colchicine or glucocorticoid plus colchicine therapy.
Δ Treatment response is assessed by evaluating symptoms and CRP (with or without repeat imaging).
A good response is indicated by improvement or resolution of symptoms, reduction or normalization
of the CRP, and, if assessed, improvement or resolution of inflammation on CMR or normalization of
pericardial thickness on CCT or CMR.
◊ The IL-1 inhibitor used in this setting may be anakinra or rilonacept.
Graphic 141073 Version 1.0
Drug therapy in acute and recurrent pericarditis for adult patients
Duration of initial
Antiinflammatory
Drug or maintenance Tapering regimen ¶
dose
dose*
First-line therapy for most patients: Δ
Aspirin ◊ 650 to 1000 mg orally 3 1 to 2 weeks Decrease dose by

times daily about 250 mg per week
or
Ibuprofen ◊ 600 to 800 mg orally 3 1 to 2 weeks Decrease dose by 200

times daily § mg per week
or
Indomethacin ◊ 25 to 50 mg orally 3 1 to 2 weeks Decrease dose by 25

times daily mg per week
plus
Colchicine ¥ ‡ 0.5 to 0.6 mg orally 2 3 months (acute) Usually not tapered

times daily
6 months or more
(recurrent)
Second-line therapy (for refractory cases or patients with a contraindication to NSAID therapy):
Prednisone 0.2 to 0.5 mg/kg daily 2 to 4 weeks (acute or Gradual tapering over 2
recurrent † ) to 3 months; refer to
UpToDate topic review
of treatment of acute
pericarditis, section on
glucocorticoids
plus
Colchicine ¥ ‡ 0.5 to 0.6 mg orally 2 3 months or more Usually not tapered

times daily (acute)
6 months or more
(recurrent)
Colchicine is generally
continued for 4 weeks
or more after
discontinuation of
glucocorticoid
Third-line therapy: Second-line therapy plus NSAID dosed as for first-line therapy
Fourth-line therapy: One of the following agents (or pericardiectomy)
Rilonacept Loading dose of 320 160 mg SC weekly for Slow taper over 3
mg delivered as 2 SC several months months or more
doses of 160 mg on the
same day at 2 different

sites
Anakinra 1 to 2 mg/kg SC daily Several months Slow taper over 3

(maximum dose 100 months or more
mg daily)
Azathioprine 1 mg/kg orally daily Several months Not tapered

increasing to 2 to 3
mg/kg daily (maximum
dose 150 mg daily)
IVIG 400 to 500 mg/kg IV 5 days (may repeat Not tapered

daily after 1 month)
NSAID: nonsteroidal antiinflammatory drug (includes ibuprofen, indomethacin, and aspirin); SC:
subcutaneous injection; IVIG: intravenous immunoglobulin; IV: intravenous; CRP: C-reactive protein.
* This column describes the typical duration of full-dose therapy for symptom control. Except for
colchicine, the duration of full-dose therapy and subsequent tapering should be tailored according to
resolution of symptoms and normalization of markers of inflammation; refer to topic reviews for
approach.
¶ Tapering is begun once symptoms have resolved for at least 24 hours and CRP level has normalized.
Tapering is continued only if the patient remains asymptomatic with normal CRP levels. Some
clinicians taper more slowly than shown in the table by reducing the total daily dose (rather than each
individual dose) by the taper dose amount indicated.
Δ For patients treated with aspirin as an antiplatlet agent (including patients with peri-infarction
pericarditis), NSAIDs (such as ibuprofen and indomethacin) are avoided. Glucocorticoid therapy is also
avoided in patients with peri-infarction pericarditis. Refer to UpToDate content on pericardial
complications of myocardial infarction.
◊ Proton pump inhibitor (eg, omeprazole) gastrointestinal protection may be indicated.
§ Some patients may require ibuprofen every 6 hours (4 times daily), in which case the dose should
not exceed 600 mg every 6 hours.
¥ 0.5 mg colchicine is not available in the United States. It is widely available elsewhere.
‡ Colchicine dose should be reduced to 0.5 to 0.6 mg once daily in patients <70 kg. Refer to UpToDate
content on colchicine dosing for other indications for dosage reduction.
† Patients with acute pericarditis are generally treated with prednisone for a duration at the lower end
of this range, while patients with recurrent pericarditis are generally treated for a duration at the
upper end of this range.
Data from:
1. Lange RA, Hillis LD. Clinical practice. Acute pericarditis. N Engl J Med 2004; 351:2195.
2. Maisch B, Seferovic PM, Ristic AD, et al. Guidelines on the diagnosis and management of pericardial disease: The task
force on the diagnosis and management of pericardial disease of the European Society of Cardiology. European Heart
Journal 2004; 25:587.
3. Imazio M, Brucato A, Trinchero R, et al. Individualized therapy for pericarditis. Expert Rev Cardiovasc Ther 2009; 7:965.
NYHA and other classifications of cardiovascular disability
Canadian
NYHA functional Cardiovascular Specific activity
Class
classification [1] Society functional scale [3]
classification [2]
I Patients with cardiac Ordinary physical Patients can perform to

disease but without activity, such as walking completion any activity
resulting limitations of and climbing stairs, requiring ≥7 metabolic
physical activity. does not cause angina. equivalents (ie, can
Ordinary physical Angina with strenuous carry 24 lb up 8 steps;
activity does not cause or rapid prolonged do outdoor work
undue fatigue, exertion at work or [shovel snow, spade
palpitation, dyspnea, or recreation. soil]; do recreational
anginal pain. activities [skiing,
basketball, squash,
handball, jog/walk 5
mph]).
II Patients with cardiac Slight limitation of Patients can perform to

disease resulting in ordinary activity. completion any activity
slight limitation of Walking or climbing requiring ≥5 metabolic
physical activity. They stairs rapidly, walking equivalents (eg, have
are comfortable at rest. uphill, walking or stair- sexual intercourse
Ordinary physical climbing after meals, in without stopping,
activity results in cold, in wind, or when garden, rake, weed,
fatigue, palpitation, under emotional stress, roller skate, dance
dyspnea, or anginal or only during the few foxtrot, walk at 4 mph
pain. hours after awakening. on level ground) but
Walking more than 2 cannot and do not
blocks on the level and perform to completion
climbing more than 1 activities requiring ≥7
flight of ordinary stairs metabolic equivalents.
at a normal pace and in
normal conditions.
III Patients with cardiac Marked limitation of Patients can perform to

disease resulting in ordinary physical completion any activity
marked limitation of activity. Walking 1 to 2 requiring ≥2 metabolic
physical activity. They blocks on the level and equivalents (eg, showe
are comfortable at rest. climbing 1 flight in without stopping, strip
Less-than-ordinary normal conditions. and make bed, clean
physical activity causes windows, walk 2.5 mph
fatigue, palpitation, bowl, play golf, dress
dyspnea, or anginal without stopping) but
pain. cannot and do not
perform to completion
any activities requiring
>5 metabolic
equivalents.
IV Patients with cardiac Inability to carry on any Patients cannot or do

disease resulting in physical activity without not perform to
inability to carry on any discomfort. Anginal completion activities
physical activity without syndrome may be requiring >2 metabolic
discomfort. Symptoms present at rest. equivalents. Cannot
of cardiac insufficiency carry out activities
or of the anginal listed above (specific
syndrome may be activity scale III).
present even at rest. If
any physical activity is
undertaken, discomfort
is increased.
NYHA: New York Heart Association.
References:
1. The Criteria Committee of the New York Heart Association. Nomenclature and Criteria for Diagnosis of Diseases of the
Heart and Great Vessels, 9 th ed, Little, Brown & Co, Boston 1994. p.253.
2. Campeau L. Grading of angina pectoris. Circulation 1976; 54:522.
3. Goldman L, Hashimoto B, Cook EF, Loscalzo A. Comparative reproducibility and validity of systems for assessing
cardiovascular functional class: Advantages of a new specific activity scale. Circulation 1981; 64:1227.

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23/2/24, 14:11 Constrictive pericarditis: Management and prognosis - UpToDate

Official reprint from UpToDate®

Constrictive pericarditis: Management and prognosis

Literature review current through: Jan 2024.

The management and prognosis of constrictive pericarditis and effusive-constrictive

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

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

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

• Subacute constrictive pericarditis – Early stage (subacute) constrictive pericarditis

A subset of patients with subacute constrictive pericarditis have transient

transient constrictive pericarditis commonly present as effusive-constrictive

• Chronic constrictive pericarditis – Most constrictive pericarditis has a chronic

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

Measures to prevent constrictive pericarditis include prompt treatment of acute pericarditis

Management of purulent pericarditis, tuberculous pericarditis, and neoplastic

● Prophylax against postpericardiotomy syndrome – In patients who have undergone

constrictive pericarditis. Prevention of postpericardiotomy syndrome is discussed

● Drain effusions – Removal of infected pericardial fluid is a key component of the

• Purulent pericarditis – For purulent pericarditis, complete drainage of the infected

• Postpericardiotomy syndrome – Limited evidence suggests that draining

MANAGEMENT OF CONSTRICTIVE PERICARDITIS

Approach to subacute disease — Our approach to treatment is as follows:

● Treatment of specific cause – Medical therapy should be directed at the underlying

rheumatic diseases) whenever possible. (See "Purulent pericarditis", section on

● Treatment of inflammation – Antiinflammatory therapy is the mainstay of treatment

• Initial therapy – Initial treatment of subacute constrictive pericarditis is similar to

Patients with marked improvement in symptoms and signs of constrictive

• Refractory disease – Patients with refractory symptoms and signs of subacute

Patients with marked improvement in symptoms and signs of constrictive

or rilonacept. If there is a good response, treatment with an IL-1 inhibitor is

● Treatment of progression to chronic disease – Patients who progress and develop

Efficacy of treatment of subacute disease — The efficacy of antiinflammatory therapy in

● Glucocorticoids and other antiinflammatory agents – Small single-institution case

As an example, in a series of 29 patients with constrictive pericarditis who were treated

Indication — We suggest pericardiectomy for patients with chronic constrictive

● Constrictive pericarditis has not progressed to "end-stage." (See 'Patients unlikely to

● There is no concomitant restrictive cardiomyopathy (eg, due to radiation-induced

Patients unlikely to benefit from pericardiectomy — Due to the complex nature of

● Mild disease – In patients with constrictive pericarditis, mild symptoms (NYHA

● End-stage disease – In our experience, patients with "end-stage" constrictive

● Mixed constrictive-restrictive disease – Symptoms may persist after successful

Procedure — Pericardiectomy involves removal of as much of the constricting parietal

The median sternotomy approach is commonly preferred as it enables more complete

pericarditis with effusive-constrictive pericarditis, the left anterolateral thoracotomy may be

Pericardiectomy is performed with or without cardiopulmonary bypass (CPB). While some

MANAGEMENT OF EFFUSIVE-CONSTRICTIVE PERICARDITIS

Management of effusive-constrictive pericarditis is similar to that for constrictive pericarditis

● Pericardiocentesis – For patients with effusive-constrictive pericarditis,

● Treatment of causes, inflammation, and symptoms – As for constrictive pericarditis,

therapy is administered as needed for symptom control. (See 'Treatment of early

Since there is overlap between effusive-constrictive pericarditis and transient

● Pericardiectomy – Surgical removal of the pericardium is reserved for patients with

Removal of the thickened and inflamed pericardium in the setting of effusive-

Long-term survival after pericardiectomy for constrictive pericarditis is inferior to that of an

The etiology of the pericardial disease is also an important determinant of survival

Preoperative indices of contraction and relaxation also predict postoperative prognosis. An

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