Constrictive pericarditis: Management and prognosis

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 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 Basics." The Basics patient education pieces are written in plain language, at the 5^th to 6^th 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 10^th to 12^th 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

●Definitions (See 'Definitions' above.)

•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.)