Pericardial disease associated with cancer: Management

INTRODUCTION — Cancer commonly involves the pericardium, with clinical presentations ranging from acute syndromes to more indolent conditions.

The management of pericardial disease associated with cancer is reviewed here. The clinical presentation and diagnosis of pericardial disease associated with cancer is presented separately. (See "Pericardial disease associated with cancer: Clinical presentation and diagnosis".)

GENERAL APPROACH — The management of pericardial disease in a patient with cancer is based upon multiple factors, including (see "Pericardial disease associated with cancer: Clinical presentation and diagnosis"):

●Symptoms and hemodynamic status

●Type of pericardial disease (eg, pericardial effusion with or without cardiac tamponade, acute pericarditis, constrictive pericarditis)

●Etiology of the pericardial disease (eg, malignancy, cancer therapy, infection, or other conditions)

●The type and stage of the cancer, with available treatments

●Cancer prognosis, patient comorbidities, performance status, and goals of care (see "Discussing goals of care")

Of note, some patients with malignant pericardial disease may not be appropriate candidates for aggressive therapy due to poor performance status, poor cancer prognosis, and/or multiple comorbidities. Patients who opt for best supportive care alone are not candidates for the treatment approaches discussed in this topic. Palliative care for patients at end of life is discussed separately. (See "Palliative care: The last hours and days of life".)

ACUTE MANAGEMENT OF CARDIAC TAMPONADE — For patients with cancer with clinical evidence of cardiac tamponade, the approach to acute management is similar to that for the general population (algorithm 1). (See "Pericardial effusion: Approach to diagnosis".)

Management details relevant to patients with cancer are as follows:

Supportive care — Volume repletion is imperative while preparing for pericardial fluid drainage. Inotropic support is rarely indicated. Positive pressure mechanical ventilation should be avoided, if possible. Further details on supportive care for cardiac tamponade are discussed separately. (See "Cardiac tamponade", section on 'Supportive care' and "Cardiac tamponade", section on 'Treatment of the underlying condition'.)

Patients on anticoagulation — Patients with cancer may also concurrently be anticoagulated for other clinical conditions, such as venous thromboembolism, which must be addressed prior to performing pericardial fluid drainage. The approach to pericardiocentesis in patients on anticoagulation or bleeding/clotting disorders is discussed separately. (See "Perioperative management of patients receiving anticoagulants" and "Pericardial effusion: Approach to diagnosis".)

Urgent drainage — For patients with cancer and cardiac tamponade, urgent pericardial fluid removal is required for therapeutic and diagnostic purposes. Removal of pericardial fluid is necessary to alleviate symptoms and prevent hemodynamic collapse. This approach generally results in rapid and dramatic improvement in symptoms and hemodynamics. Pericardial fluid or tissue sampling is also necessary to confirm the diagnosis of malignant pericardial effusion and, if indicated, to direct subsequent antineoplastic therapy once the patient is hemodynamically stable. (See "Pericardial disease associated with cancer: Clinical presentation and diagnosis", section on 'Diagnosis of malignant pericardial disease' and 'Cancer-directed therapy' below.)

●Procedure for initial acute pericardial fluid removal – Pericardial fluid is typically removed percutaneously (ie, using pericardiocentesis under echocardiographic guidance) or surgically (ie, using a pericardial window) (algorithm 1). Choosing between these approaches is discussed separately. (See "Pericardial effusion: Approach to diagnosis".)

●Preventing reaccumulation – At the time of or immediately after initial pericardial fluid removal, patients with suspected or confirmed malignant pericardial effusion should be treated with measures that reduce reaccumulation of pericardial effusion. (See 'Prevention and treatment of recurrent malignant pericardial effusion' below.)

For those who have had pericardial fluid removal, the development of symptoms such as fatigue, dyspnea, or chest heaviness should prompt repeat echocardiography to assess for recurrent pericardial effusion. (See "Cardiac tamponade" and 'Prevention and treatment of recurrent malignant pericardial effusion' below and "Pericardial effusion: Approach to diagnosis".)

●Complications of fluid removal – Pericardial fluid removal is generally tolerated. Rare complications of pericardiocentesis for cardiac tamponade include bleeding and heart failure (caused by posttap fluid shift into the central circulation). (See "Pericardial effusion: Approach to diagnosis".)

PREVENTION AND TREATMENT OF RECURRENT MALIGNANT PERICARDIAL EFFUSION

General principles — Patients with cardiac tamponade that is directly attributed to malignancy commonly reaccumulate pericardial fluid. Therefore, initial management with pericardial fluid removal is generally combined with or followed by one or more measures to prevent or treat recurrent malignant pericardial effusion, such as mechanical pericardial interventions (prolonged catheter drainage, repeated pericardiocentesis, surgical pericardiotomy, or, rarely, percutaneous balloon pericardiotomy) and cancer-directed systemic therapy. Selection of therapy by a multidisciplinary team (including a cardiologist, cardiac surgeon, and medical oncologist) is guided by the patient's overall clinical status (including symptoms, medical frailty, and comorbidities), prognosis, goals of care, care setting, and available treatment resources. Treatments should also focus on relieving symptoms, improving functional status, and minimizing repeat interventions.

The management of recurrent or chronic pericardial effusions in patients without cancer is discussed separately. (See "Pericardial effusion: Approach to diagnosis".)

Mechanical pericardial interventions after initial pericardiocentesis

Selection of intervention — For patients with malignant pericardial effusion who are initially treated with fluid removal using pericardiocentesis, further mechanical cardiac interventions are generally necessary to treat or prevent pericardial fluid reaccumulation. Options include catheter drainage, surgical decompression of the pericardium, and, rarely, percutaneous balloon pericardiotomy.

For patients with malignant pericardial effusion, our approach to managing recurrent pericardial effusion is as follows [1]:

●Initial prevention and treatment of recurrent effusion

•For medically fit patients with longer life expectancy, a catheter designed for extended pericardial fluid drainage (typically three to five days) can be placed at the time of initial drainage with pericardiocentesis (for prevention) or at the time of pericardial fluid recurrence (for treatment), commonly with inpatient monitoring. (See 'Extended catheter drainage' below.)

•Patients with limited life expectancy and a recurrent malignant pericardial effusion may be offered repeat pericardiocentesis, particularly if this is the option most compatible with the chosen care setting (eg, home or hospice rather than a monitored setting). Best supportive care is an appropriate alternative for patients who are not candidates for repeat pericardiocentesis; this situation may occur due to procedural limitations (eg, pericardial fluid that is drained repeatedly may become more loculated and difficult to access percutaneously) or the inability of the patient to tolerate repeat procedures.

●Refractory pericardial effusion – For those medically fit patients with recurrent or persistent malignant pericardial effusion who have undergone (or are expected to require) repeated pericardiocentesis or extended catheter drainage, surgical decompression of the pericardium is the standard approach (see 'Surgical decompression of the pericardium' below). We rarely use balloon pericardiotomy given its limitations, as discussed below (see 'Balloon pericardiotomy' below). The optimal treatment is not established since these approaches have not been directly compared in randomized trials.

●Avoid sclerosing agents – We do not use pericardial sclerosing agents, which are as effective as the other approaches in preventing pericardial effusion reaccumulation but commonly cause severe pain. (See 'Avoidance of pericardial sclerosing agents' below.)

Mechanical pericardial interventions are typically followed by systemic therapy directed at the specific cancer, as clinically indicated. (See 'Cancer-directed therapy' below.)

Although pericardiocentesis effectively relieves symptoms and improves hemodynamics, pericardial fluid reaccumulates in as many as 60 percent of patients [2,3]. As an example, one systematic review of 31 retrospective studies evaluated the effect of various percutaneous interventions on the recurrence rate of malignant pericardial effusion [4]. At follow-up ranging from two months to over one year, isolated pericardiocentesis demonstrated the highest rate of recurrent pericardial effusion (38 percent) relative to other mechanical interventions, including prolonged catheter drainage (12 percent), balloon pericardotomy (10 percent), and pericardial sclerosis (11 percent).

Extended catheter drainage — For patients with malignant pericardial effusion, extended catheter drainage is an effective treatment option that reduces the risk of recurrent pericardial effusion to approximately 10 to 20 percent [1,4-7]. However, the duration of pericardial effusion control is variable and ranges approximately between 1 to 12 months in observational studies [8,9].

●Treatment approach – Catheter drainage is typically required for at least three to five days [7,9]. The catheter should not be removed until drainage is minimal (<25 to 50 mL in a 24-hour period) to none. For patients who have had a catheter placed for three to five days whose rates of pericardial fluid drainage remains greater than 50 mL in a 24-hour period, we evaluate for a surgical pericardial window. (See 'Surgical decompression of the pericardium' below.)

In one observational series of 171 patients with cancer and pericardial effusion who underwent echocardiography-guided pericardiocentesis followed by extended catheter drainage, the average time to minimal catheter output (<50 mL in 24 hours) was three days [7].

●Complications – The reported incidence of complications from prolonged catheter drainage ranges from 7 to 17 percent [10]. Complications include pericarditic chest pain, catheter occlusion, infection, fever, pneumothorax, ventricular puncture, and cardiac arrest [3,8,11]. Intermittent rather than continuous drainage may help to maintain catheter patency and reduce risk for infection by decreasing the opportunity for retrograde migration of microbes or catheter tubing collapse that could cause occlusion.

Surgical decompression of the pericardium — Surgical decompression of the pericardium (via creation of a pericardial window) results in immediate hemodynamic benefit [6,8,12-17]. Surgical decompression is also associated with the lowest rates of recurrent pericardial effusion (0 to 7 percent) relative to other percutaneous interventions (ie, pericardiocentesis, catheter drainage, and balloon pericardotomy), but is the most invasive procedure. We prefer this approach in medically fit patients with recurrent or persistent malignant pericardial effusion who have undergone (or are expected to require) repeat pericardiocentesis or extended catheter drainage.

Surgical decompression of the pericardium has not been directly compared with other methods of pericardial drainage in randomized trials. In a systematic review of 59 observational studies, over a median follow-up of two months, surgical interventions were associated with better control of malignant pericardial effusion (93 to 100 percent) compared with percutaneous balloon pericardotomy (90 percent), single pericardiocentesis (67 percent), and extended catheter drainage (55 percent) [10].

●Treatment approach – For patients with a hemodynamically significant pericardial effusion, we generally perform pericardiocentesis prior to surgery to avoid further hemodynamic instability or cardiovascular collapse during administration of general anesthesia prior to surgery [17]. However, some loculated pericardial effusions may require surgical drainage. (See "Pericardial effusion: Approach to diagnosis".)

Surgical creation of a pericardial window can be accomplished using various surgical techniques (eg, open surgery, video assisted thoracoscopy [VATS]). While recurrence rates vary slightly according to surgical technique, recurrence of a large or symptomatic pericardial effusion following transthoracic (thoracotomy or VATS) pericardiostomy was seen in only 5 to 10 percent of patients, compared with an approximately 20 percent rate following pericardiocentesis alone. Recurrence rates are slightly higher when a subxiphoid approach is used because these "windows" do not drain to an absorptive mesothelial surface [17]. Moreover, approximately one-third of patients with malignant pericardial disease have coexisting pleural pathology, which may need to be addressed concurrently.

●Complications – The frequency of postoperative complications for surgical decompression ranges from approximately 5 to 10 percent [10]. The most common complications include hemorrhage, infection, pulmonary embolism, arrythmias, and myocardial infarction.

Balloon pericardiotomy — Due to its limitations, balloon pericardiotomy is a rarely used option to treat recurrent malignant pericardial effusion in patients previously treated with pericardiocentesis or catheter drainage. Balloon pericardiotomy causes pain and the size of the pericardial defect it creates is difficult to control, which increases the risk of early pericardial defect self-closure.

For patients treated with balloon pericardotomy, the recurrence rate for pericardial effusion ranges between 10 and 40 percent, with better outcomes seen in institutions with expertise in this procedure [4,10,18-22]. While there are no randomized trials comparing balloon pericardotomy with surgical decompression of the pericardium, observational data suggest that balloon pericardotomy is associated with lower rates of fluid recurrence (10 percent) relative to pericardiocentesis (38 percent) [4], but higher rates relative to surgical decompression (0 to 7 percent) [10]. (See 'Surgical decompression of the pericardium' above.)

●Treatment approach – Balloon pericardiotomy may be performed either immediately following initial pericardiocentesis or after pericardial effusion recurrence. Briefly, following catheter-based drainage, the pigtail catheter is exchanged over a wire for a larger balloon-tipped catheter that is inflated and extruded across the parietal pericardium to create a larger sized connection.

●Complications – Complications from balloon pericardotomy can occur in up to one-third of patients, including pneumothorax, pleural effusion, and infection [10,18,21]. Other, less common complications include hemorrhage and ventricular puncture.

Avoidance of pericardial sclerosing agents — We avoid the use of pericardial sclerosing agents due to significant toxicity. Pericardial sclerosing agents, such as tetracycline, platinum-based chemotherapy, and mitomycin C, induce inflammation and fibrotic adhesion of the pericardial layers to reduce the potential space for fluid reaccumulation. Although pericardial sclerosis is associated with similar rates of recurrent pericardial effusion relative to other mechanical cardiac interventions, this approach is associated with severe chest pain and constrictive pericarditis and is no longer used [4,23].

Cancer-directed therapy

Systemic therapy — Pericardial effusions and masses caused by cancer may respond to systemic antineoplastic therapy. This approach works best when combined with a mechanical method to promote pericardial drainage. In one observational study, systemic chemotherapy plus pericardial window was associated with a better treatment response (reduction in pericardial effusion or pericardial masses) compared with systemic chemotherapy plus pericardial drainage (92 versus 73 percent) [24]. Treatment with systemic chemotherapy alone was associated with the lowest response rate (54 percent).

The treatment of cancer types that are frequently associated with malignant pericardial effusions are discussed separately. (See "Pericardial disease associated with cancer: Clinical presentation and diagnosis", section on 'Malignancy'.)

●Lung cancer – (See "Overview of the initial treatment and prognosis of lung cancer".)

●Breast cancer – (See "Overview of the approach to metastatic breast cancer".)

●Esophageal cancer – (See "Initial systemic therapy for locally advanced unresectable and metastatic esophageal and gastric cancer".)

●Melanoma – (See "Overview of the management of advanced cutaneous melanoma".)

●Acute leukemia (AML and ALL) – (See "Treatment of acute lymphoblastic leukemia/lymphoma in children and adolescents" and "Acute myeloid leukemia in adults: Overview".)

●Hodgkin and non-Hodgkin lymphoma – (See "Overview of Hodgkin lymphoma in children and adolescents" and "Overview of non-Hodgkin lymphoma in children and adolescents" and "Clinical presentation and diagnosis of classic Hodgkin lymphoma in adults" and "Clinical presentation and initial evaluation of non-Hodgkin lymphoma".)

Other therapies

●Radiation therapy - Radiation therapy (RT) is used to treat radiosensitive cancers that may present adjacent to the heart, such as leukemias, lymphomas, lung, breast, and esophageal cancer. Multidisciplinary evaluation that includes the input of a radiation oncologist is necessary to manage patients who may benefit from the inclusion of radiation therapy and its sequencing with systemic therapy in this setting (eg, a patient with mediastinal lymphoma and pericardial effusion presumed to be caused by lymphatic obstruction). The decision on whether to include RT for patients with malignant pericardial effusion is based upon the potential benefits of cancer control and potential harms. These harms include acute side effects such as esophagitis and late toxicities such as pericardial, coronary, myocardial, and valve disease [25-29]. (See "Radiation therapy techniques in cancer treatment", section on 'Radiation side effects' and "Cardiotoxicity of radiation therapy for breast cancer and other malignancies" and "Cardiotoxicity of radiation therapy for Hodgkin lymphoma and pediatric malignancies".)

●Intrapericardial chemotherapy – Intrapericardial chemotherapy is a less preferred treatment to treat malignant pericardial effusion [25-29] due to the availability of more effective systemic therapies for most advanced cancer that are more easily administered.

Prognosis — Most patients with a symptomatic malignant pericardial effusion have a limited life expectancy (median two to four months), and many often develop recurrent pericardial effusion within this time [3,8,12-14,16,21,30-34]. Prognosis may be better in certain subsets of patients, such as those without malignant cells in the pericardium (at least in the setting of non-small cell lung cancer) [35,36], hematologic rather than solid tumors [32], breast cancer rather than some types of lung cancer [5,32,37,38], patients who are candidates for systemic therapy [24,39] or whose cancer is otherwise well-controlled with systemic therapy [31].

Many patients with cancer and pericardial disease will also benefit from palliative care services, which improves clinical outcomes and quality of life. (See "Discussing goals of care" and "Benefits, services, and models of subspecialty palliative care".)

PERICARDIAL EFFUSION WITHOUT CARDIAC TAMPONADE — Asymptomatic or minimally symptomatic pericardial effusions without cardiac tamponade can be managed conservatively with careful clinical and echocardiographic monitoring and avoidance of volume depletion. Malignant pericardial effusions may also be treated with systemic antineoplastic therapy directed at the causative cancer type, if indicated (see 'Cancer-directed therapy' above). Some patients will never develop symptoms or require intervention, particularly patients with small pericardial effusions.

Pericardial fluid or tissue sampling is necessary to confirm the diagnosis of malignant pericardial effusion, which is discussed separately. (See "Pericardial disease associated with cancer: Clinical presentation and diagnosis", section on 'Diagnosis of malignant pericardial disease'.)

The development of symptoms such as fatigue, dyspnea, or chest heaviness should prompt evaluation, including assessment for cardiac tamponade with physical examination and echocardiography. (See "Pericardial disease associated with cancer: Clinical presentation and diagnosis", section on 'Diagnostic evaluation for pericardial disease'.)

ACUTE PERICARDITIS — For acute symptomatic pericarditis, the goals of therapy are relief of pain, resolution of inflammation (and, if present, pericardial effusion), and the prevention of recurrence. Because limited data are available to guide management of acute pericarditis specifically in cancer patients, most treatment recommendations are extrapolated from observational studies, indirect evidence in patients without cancer, and expert opinion.

General approach — Nearly all patients with acute pericarditis associated with cancer receive combination therapy with colchicine plus nonsteroidal antiinflammatory drugs (NSAIDs) (table 1). Our general approach to treatment of symptomatic acute pericarditis is discussed in detail separately. (See "Acute pericarditis: Treatment and prognosis".)

Pericarditis caused by cytotoxic cancer therapies often resolves with the above standard therapy or after discontinuation of the cytotoxic agent. In this setting, the potential risks and benefits of cancer treatment interruption should be evaluated by a multidisciplinary cardio-oncology team.

Glucocorticoid therapy is generally reserved for refractory cases (or those for whom NSAIDs are contraindicated) (table 1). An exception is the treatment of patients with severe immune checkpoint inhibitor (ICI)-associated pericarditis, as discussed below. (See 'Immune checkpoint inhibitor-associated pericarditis' below.)

Immune checkpoint inhibitor-associated pericarditis — There are limited data to guide the management of ICI-associated pericarditis. Our approach is as follows:

●Trivial or small effusion – Patients with mild ICI-associated pericarditis and trivial or small pericardial effusion may tolerate continuation of the ICI and are treated with standard combination therapy for acute pericarditis (table 1) [40].

●Moderate or large effusion – For patients with severe ICI-associated pericarditis with moderate or large pericardial effusion, management consists of discontinuing the ICI and treating with antiinflammatory agents. We suggest glucocorticoid therapy (eg, methylprednisolone 1 mg/kg/day or prednisone 1 to 2 mg/kg/day) plus colchicine (rather than an NSAID plus colchicine) [40,41]. This approach is based on observational data in ICI-associated pericarditis and indirect evidence in patients with refractory pericarditis associated with other causes (although the glucocorticoid dose is generally higher for ICI-associated pericarditis). (See "Acute pericarditis: Treatment and prognosis", section on 'Glucocorticoids'.)

Rarely, ICI-pericarditis may be refractory to initial glucocorticoid doses and colchicine. Treatments that have been reported for refractory ICI-pericarditis include higher-dose glucocorticoid therapy (eg, methylprednisolone 1 g/day) along with other immunosuppressive agents (eg, infliximab [42] or azathioprine [43]).

●Cardiac tamponade – Patients with ICI-associated pericarditis and cardiac tamponade also require drainage of the pericardial fluid. (See 'Acute management of cardiac tamponade' above.)

●Management after resolution – After symptoms and signs of acute pericarditis resolve, patients may be evaluated by a multidisciplinary team for possible reinitiation of the ICI with close monitoring for recurrent symptoms [40].

The management of other cardiovascular toxicities related to ICI (including myocarditis) are discussed separately (table 2). (See "Toxicities associated with immune checkpoint inhibitors", section on 'Cardiovascular toxicity'.)

●Treatment of other causes – Other causes of pericarditis (such as infection) should be evaluated and treated appropriately, as discussed separately. (See "Purulent pericarditis".)

CONSTRICTIVE PERICARDITIS — Constrictive pericardial disease directly due to malignancy is uncommon. Most cases of cancer-associated pericardial constriction fall into the effusive-constrictive category where the pericardial effusion is often the primary cause of symptoms and signs and initial therapy is generally drainage of the pericardial fluid. (See "Constrictive pericarditis: Clinical features and causes", section on 'Incidence and causes' and "Constrictive pericarditis: Diagnostic evaluation", section on 'Effusive-constrictive pericarditis'.)

Radiation therapy to the chest (primarily treatment for Hodgkin disease, breast cancer, or lung cancer) is one of the causes of constrictive pericarditis. Measures to reduce the risk of radiation-induced pericardial disease (and other cardiac toxicity affecting the coronary arteries, valves, and myocardium) include techniques for more precise targeting of radiotherapy, respiratory gating, and prone positioning for patients with breast cancer [44]. Some patients with radiation-induced constrictive pericarditis also have restrictive cardiomyopathy, which may also be caused by exposure to radiation. (See "Constrictive pericarditis: Clinical features and causes", section on 'Constrictive pericarditis' and "Pericardial disease associated with cancer: Clinical presentation and diagnosis", section on 'Radiation' and "Restrictive cardiomyopathies".)

The rare patients with true malignant constrictive pericarditis may require surgical removal of the pericardium, if this intervention is justified by the overall prognosis. Pericardiectomy is the only definitive treatment option for patients with chronic constrictive pericarditis. Medical therapy (ie, diuretics) may be used as a temporizing measure and for patients who are not candidates for surgery. (See "Constrictive pericarditis: Management and prognosis", section on 'Management of constrictive pericarditis' and "Pericardial disease associated with cancer: Clinical presentation and diagnosis".)

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

●General approach – The management of pericardial disease in a patient with cancer is based upon multiple factors including symptoms, hemodynamic status, the type and etiology of pericardial disease, the type and stage of cancer, cancer prognosis, patient comorbidities, performance status, and goals of care. (See 'General approach' above.)

●Cardiac tamponade

•Urgent drainage – Patients with cardiac tamponade require urgent drainage. For patients with cancer and cardiac tamponade, acute management is similar to that for nonmalignant cardiac tamponade, including supportive care with fluid repletion while preparing for urgent pericardial fluid removal (via pericardiocentesis or surgical pericardial window) (algorithm 1). Measures to reduce the risk of reaccumulation are instituted at the time of or immediately after initial pericardial fluid removal. (See 'Acute management of cardiac tamponade' above.)

•Initial prevention and treatment of recurrent malignant pericardial effusion – For patients with malignant pericardial effusion with cardiac tamponade, initial management with pericardial fluid removal is usually combined with or followed by one or more measures to prevent or treat recurrent pericardial effusion. Options include mechanical pericardial interventions (repeated pericardiocentesis, prolonged catheter drainage or surgical pericardiotomy, or, rarely, percutaneous balloon pericardiotomy), cancer-directed systemic therapy, and palliative care. The choice of intervention is based upon factors including patient symptoms, medical frailty, prognosis, goals of therapy, comorbidities, and care setting. (See 'Prevention and treatment of recurrent malignant pericardial effusion' above and 'Cancer-directed therapy' above.)

Patients treated with systemic antineoplastic therapy still require mechanical management for prevention and treatment of recurrent malignant effusion. (See 'Cancer-directed therapy' above.)

•Refractory malignant pericardial effusion – For those medically fit patients with recurrent or persistent malignant pericardial effusion despite repeated pericardiocentesis or extended catheter drainage, we suggest surgical decompression of the pericardium rather than other pericardial interventions (such as balloon pericardiotomy or pericardial sclerosing agents) (Grade 2C). (See 'Surgical decompression of the pericardium' above.)

For frail patients with limited life expectancy, alternative management strategies include repeated pericardiocentesis and palliative care measures. (See 'Selection of intervention' above.)

●Pericardial effusion without cardiac tamponade – Pericardial effusions without cardiac tamponade can be managed with careful clinical and echocardiographic monitoring and avoidance of volume depletion. Malignant pericardial effusions may be treated with antineoplastic systemic agents, if indicated. (See 'Pericardial effusion without cardiac tamponade' above.)

●Acute pericarditis

•Non-ICI-associated pericarditis – For acute symptomatic pericarditis, the goals of therapy are relief of pain, resolution of inflammation (and, if present, pericardial effusion), and the prevention of recurrence. Nearly all patients with acute pericarditis associated with cancer not associated with immune checkpoint inhibitor (ICI) use are treated with combination therapy consisting of a nonsteroidal antiinflammatory drug (NSAID) plus colchicine (table 1). (See 'Acute pericarditis' above and "Acute pericarditis: Treatment and prognosis".)

•ICI-associated pericarditis

-Patients with mild ICI-associated pericarditis may tolerate continuation of ICI and are treated with standard therapy for acute pericarditis (table 1). (See "Acute pericarditis: Treatment and prognosis", section on 'Medical therapies'.)

-For patients with severe ICI-associated pericarditis with moderate or large pericardial effusion, management consists of discontinuing the ICI and treating with antiinflammatory agents. We suggest treating with glucocorticoid therapy (eg, methylprednisolone 1 mg/kg/day or prednisone 1 to 2 mg/kg/day) plus colchicine rather than NSAID plus colchicine (Grade 2C).

●Constrictive pericarditis – The rare patients with true malignant constrictive pericarditis may require surgical removal of the pericardium, if this intervention is justified by the overall prognosis. Pericardiectomy is the only definitive treatment option for patients with chronic constrictive pericarditis. Diuretic therapy may be used as a temporizing measure and for patients who are not candidates for surgery. (See 'Constrictive pericarditis' above.)

ACKNOWLEDGMENT — The UpToDate editorial staff acknowledges Howard (Jack) West, MD, who contributed to earlier versions of this topic review.