Pericardial Effusions in People With Cancer

A pericardial effusion is defined as the buildup of fluid within the pericardium, the layer of tissues that line the heart. In roughly two-thirds of people, the effusion develops slowly (a chronic pericardial effusion) and may cause few symptoms. If it develops rapidly, however, an acute pericardial effusion often causes life-threatening symptoms and is a medical emergency. When enough fluid builds up in the pericardial space it can severely limit the motion of the heart, a condition known as cardiac tamponade.

Pericardial effusions occur in up to 21% of people with cancer, and are sometimes the first symptom. The test of choice for making the diagnosis is an echocardiogram, but a high index of suspicion is important in finding the effusion early. Thought to be a poor prognostic sign in the past, the mortality rate has decreased substantially in the past few decades due prompt diagnosis and management.

Pericardial effusions can be acute or chronic (present for at least 3 months). Ordinarily, there is 15 ml to 50 ml of fluid present in the pericardial space (1 tsp equals 5 ml), but large effusions may contain up to 2 liters of fluid.

If you have a pericardial effusion that is small and developed slowly, you may only know of its presence due to findings on an X-ray or CT scan. In contrast, if the pericardial effusion is large, or if it develops rapidly it can cause symptoms including:

• Shortness of breath (especially with lying down and often worse with inspiration)
• Lightheadedness and fainting (syncope)
• Chest pain, more commonly felt on the left side than the right
• Palpitations
• A rapid heart rate (since the heart is restricted and cannot beat as forcefully, heart rate increases to maintain the same blood flow to tissues)

Unfortunately, symptoms of a pericardial effusion can mimic those of the condition that often results in these effusions (such as lung cancer or metastatic breast cancer), delaying the diagnosis. It can also mimic heart disease.

There are many causes of pericardial effusions, and even if a person has cancer these conditions may be the culprit. Some of these include:

• Infections, including viral infections, bacterial infections (including tuberculosis), fungal infections, and parasitic infections. Viral infections are most common, especially Cocksackie A and B, HIV, hepatitis viruses, cytomegalovirus (CMV), and echoviruses.
• Autoimmune conditions (such as with rheumatoid arthritis and lupus)
• Cancers (both those that spread to the pericardium from other regions of the body such as lung cancer, breast cancer, sarcomas, and lymphomas, and those that begin in the pericardium, such as pericardial mesothelioma)
• Current or previous radiation therapy to the chest for lung cancer, breast cancer, esophageal cancer, lymphomas, and others
• Chemotherapy
• Kidney failure
• Hypothyroidism (usually when severe)
• Inflammation (including pericarditis)
• Chest surgery (including surgery for heart disease or lung cancer)
• Heart attacks
• Medications

Some of the medications that may cause pericardial effusions include:

• The chemotherapy medications Adriamycin (doxorubicin) and Cytoxan (cyclophosphamide)
• The tuberculosis drug Isoniazid
• The seizure medication Dilantin (phenytoin)
• The medication Rogaine (minoxidil) used for male pattern baldness and high blood pressure

Pericardial effusions may occur with any type of cancer, but the most common include lung cancer, breast cancer, and lymphomas. With lung cancer, pericardial effusions are very common, with roughly half of people undergoing radiation therapy for lung cancer developing some degree of an effusion. Adjuvant chemotherapy (chemotherapy given after surgery) appears to increase this risk this risk as well.

In about 50% of people with cancer who have a pericardial effusion, the pericardial effusion is the first sign and leads to the diagnosis of the cancer.

The diagnosis of a pericardial effusion begins with a careful history and physical exam, combined with a high index of suspicion in people who have cancer.

Physical examination may suggest the presence of a pericardial effusion, but is not diagnostic alone. Breath sounds may be decreased, usually due to a concurrent pleural effusion. The classic triad of findings includes low blood pressure (hypotension), enlargement or bulging of veins in the neck (jugular venous distention), and muffled heart sounds. A pericardial friction rub (a high pitched scratching sound over the heart) may also be heard.

Laboratory tests cannot diagnose a pericardial effusion but may be helpful in detecting some of the underlying causes, such as viral cultures, TB tests, and inflammatory markers. An EKG may show ST elevation in most leads.

In general, imaging tests alone cannot make a diagnosis of a pericardial effusion, but may suggest that one is present. On a chest X-ray, a radiologist may see an enlarged cardiac shadow known as the "water-bottle sign." A pericardial fat stripe may also be seen. CT and MRI are sometimes done, and may be helpful along with an echocardiogram in defining pericardial effusions found on the anterior side of the heart, or when pockets of fluids (a loculated effusion) are present.

An echocardiogram, or ultrasound of the heart is the test of choice for diagnosing a pericardial effusion, and there are several variations of this test that may be used (including 2-D echo, M-mode echo, doppler echo, transesophageal echo, and/or intracardiac echo).

Some of the conditions that can mimic a pericardial effusion include:

• Acute pericarditis
• Constrictive pericarditis
• Pulmonary edema (cardiogenic)
• Pulmonary embolism
• Heart attacks

The treatment of pericardial effusions is two-fold. First, the pericardial effusion needs to be treated, often on an emergency basis if it is compressing the heart. Secondly, the cause of the pericardial effusion needs to be addressed.

With infections, antibiotics or antiviral medications will be needed. With inflammation, anti-inflammatory medications or steroids may be used. If the effusion is due to medications, the drug is usually stopped and alternative medications used when possible. With heart failure, medications to treat the conditions, such as diuretics, may be used. With cancer, management of the cancer will vary depending on the extent of the cancer (see below).

Treatments to resolve a pericardial effusion may include:

• Pericardiocentesis: In a pericardiocentesis, a long, thin needle is inserted through the skin and into the pericardial sac (usually with ultrasound guidance) to withdraw the fluid.
• Inserting a shunt (from the pericardial effusion into the abdomen to continually drain the fluid) is often needed as these effusion often recur after they are drained.
• Balloon pericardiotomy
• Pericardiectomy (removing the pericardium) via either thoracoscopic or open surgery. A pericardial window (subxyphoid pericardiectomy) is a procedure in which a section ("window") of pericardial tissue is removed so that fluid can no longer accumulate in the pericardial sac.

If the effusion is large or symptomatic, a procedure known as video-assisted thoracoscopic (VATS) pericardiectomy may be performed. For people who have pericardial effusions due to benign causes (such as an infection) the prognosis with this procedure is good. Given the reasons behind the development of pericardial effusions in people with cancer, the prognosis is guarded regardless of the treatment chosen. That said, with better treatments now available for many advanced cancers, managing the effusion while beginning treatment to control the underlying cancer is likely to improve the prognosis.

Treatment can vary depending on the extend of the cancer. In some cases, chemotherapy and radiation therapy have reduced a pericardial effusion so that surgery can later be successful. Most of the time, however, a pericardial effusion is associated with other areas of cancer metastases (metastatic cancer) and systemic (whole-body) treatments such as chemotherapy, targeted therapy, and/or immunotherapy are the best options.

The prognosis of a pericardial effusion depends largely on the underlying cause. With infections, inflammatory disease, or kidney disease that can be managed, the prognosis may be very good, and it's important to remember that people who have cancer also develop these conditions.

In the past, the prognosis for a pericardial effusion was poor, with up to a third of people with metastases to the pericardium dying from pericardial tamponade. Better treatment options for metastatic cancer as well as prompt treatment of a developing pericardial effusion are critical to improve survival.

A pericardial effusion may be mild and temporary with conditions such as some viral infections, but can be serious and a poor prognostic sign for people living with cancer. That said, there are a number of methods that can be used to resolve an effusion to improve symptoms and prevent complications when diagnosed promptly.

If you or a loved one have cancer and develop a pericardial effusion, ask a lot of questions and be your own advocate. The treatment and prognosis of cancers, even lung cancer, is improving, and much of what you may have heard or read about metastatic cancer and pericardial effusions may not only be very disheartening, but inaccurate. Since it's challenging for even oncologists who specialize in one type of cancer to stay abreast of the latest treatment options, strongly consider getting an opinion at a large cancer center such as a National Cancer Institute designated cancer center. Even in the elderly, newer treatments are available that are much better tolerated, and performance status (how someone functions day to day) is more important than chronological age.