Pericardial Disease Program

Overview

The Pericardial Disease Program at the Massachusetts General Hospital Corrigan Minehan Heart Center is a multidisciplinary team of cardiologists, imaging specialists and surgeons with expertise in taking care of conditions involving the pericardium, the protective sac around the heart. Patients most commonly seek a pericardiectomy for acute pericarditis, an uncommon condition that causes inflammation of the normally thin and flexible membrane.

Our physicians are international leaders in the diagnosis and treatment of this uncommon condition, and we have treated more than 200 cases since 2010. As a multidisciplinary team, we collectively diagnose the condition, define the treatment plan and make recommendations to patients and families to engage them as partners in their care, and help patients make informed decisions. Education is a cornerstone of our care.

Conditions We Treat

Recurrent Pericarditis

Acute Pericarditis

The pericardium is a thin, sac-like structure that surrounds and protects the heart. Acute pericarditis is a condition in which the pericardium becomes irritated and inflamed, causing chest pain. In approximately 85% of cases, the cause of acute pericarditis isn’t known, but viral infections are thought to be responsible for the vast majority. Other rarer causes include autoimmune conditions, bacterial infections, tuberculosis, malignancy, radiation therapy, drugs and toxins, cardiac surgery, and chest trauma.

While acute pericarditis may cause a variety of symptoms, by far the most common symptom is chest pain, which occurs in over 95% of patients and is often described as sharp and stabbing in nature. It typically radiates to the back of the neck, worsens with deep breaths or coughing, and improves when sitting up and leaning forward. Other potential symptoms of acute pericarditis include shortness of breath, fatigue, low-grade fever, palpitations, and viral symptoms such as cough, sore throat, and runny nose, although symptoms may vary depending on the underlying cause. Additionally, acute pericarditis is commonly associated with pericardial effusion, which refers to a buildup of fluid within the pericardium. The majority of pericardial effusions are small and do not cause symptoms or require treatment; in rare cases, however, a pericardial effusion may become large and/or compromise the heart’s ability to pump blood to the body. In these circumstances, a pericardiocentesis is performed to remove the fluid from within the pericardium.

The diagnosis of acute pericarditis is typically made based on a patient’s symptoms and clinical history in combination with an electrocardiogram (EKG), blood work, and echocardiogram (cardiac ultrasound). Treatment consists of anti-inflammatory medications, most commonly with non-steroidal anti-inflammatory drugs (NSAIDs), such as ibuprofen or aspirin, in combination with colchicine, a second anti-inflammatory medication. Most cases of pericarditis are mild, and the vast majority of patients recover fully within a few weeks.

Overview of Recurrent Pericarditis

Occasionally, patients who experience an episode of acute pericarditis may develop recurrent symptoms weeks to months after their initial episode, a condition called recurrent pericarditis. Recurrent pericarditis occurs when symptoms of acute pericarditis return after a symptom-free period of at least four to six weeks. A related condition, called incessant pericarditis, occurs when symptoms of acute pericarditis persist for more than four to six weeks without a symptom-free period. Both conditions are challenging to treat and require a multidisciplinary team of pericardial experts to guide therapy.

Diagnosis

When patients with a history of acute pericarditis present with recurrent or incessant pericarditis, we undertake a comprehensive clinical evaluation to evaluate for possible underlying causes and to determine the extent of ongoing inflammation within the pericardium. Potential diagnostic tests may include:

• Blood work
• Echocardiogram (cardiac ultrasound)
• Cardiac CT scan to look for pericardial thickening or calcification
• Cardiac MRI to look for inflammation within the pericardium

Treatment

Our multidisciplinary team of experts devises a care plan uniquely tailored to each individual patient. For patients with evidence of ongoing inflammation, a number of different anti-inflammatory medications may be trialed, including NSAIDs and colchicine, glucocorticoids (steroids), and newer injectable agents that target novel pathways to suppress inflammation. In addition, a variety of analgesic medications may be employed to help alleviate the chest pain associated with pericarditis, as well. For select patients with persistent chest pain who do not experience relief with medication therapy, surgical pericardiectomy may be considered, in which the entire pericardium is surgically removed from the surface of the heart.

Prognosis

Recurrent pericarditis is a difficult condition to manage as it may recur over time. Reassuringly, while symptoms may wax and wane, most cases eventually resolve without causing permanent damage to the heart or pericardium. In rare circumstances, patients with recurrent pericarditis may develop constrictive pericarditis, which is a condition in which the pericardium becomes fibrotic and scarred, leading to symptoms of heart failure. Patients with recurrent pericarditis are thus followed by our team of pericardial specialists over time, even after symptoms resolve, to monitor for the development of rare complications such as constrictive pericarditis.

Constrictive Pericarditis

Constrictive pericarditis is a condition characterized by thickening and scarring of the pericardium and, in some cases, deposition of calcium. The inflammation can cause pain, sometimes severe. It often has an unknown cause but may be related to a virus or other systemic illness. On occasion, this inflammation leads to thickening of the pericardium which, in the acute initial phase of pericarditis, may resolve with medications alone. However, in some instances, the pericardium becomes permanently thickened and calcified. When this happens, the heart is unable to fill normally with blood from the body and therefore cannot pump normally.

In many instances the cause of this condition is unknown, or what we call “idiopathic.” In other cases it may be caused by:

• Autoimmune disease
• Radiation therapy to the chest
• Diseases such as tuberculosis and mesothelioma
• Infections
• Prior cardiac surgical procedures

Common symptoms associated with constrictive pericarditis are similar to those of heart failure, including:

• Difficulty breathing and chest pain
• Fatigue
• Swelling in the legs, feet and abdomen
• Engorgement of the veins, including the neck veins
• Muscle weakness

You should seek medical attention when experiencing any of the above symptoms.

Diagnosis

The diagnosis of pericardial constriction is often difficult as it is uncommon, so it is important to seek advanced care at a specialized medical center. Many patients go undiagnosed for months or even years. However, our specialists are aware and attuned to the possible diagnosis.

Your initial visit at Mass General may be with any member of our multidisciplinary team, including cardiac surgeons and cardiologists. If you have been referred to a surgeon by your primary care physician or another institution, your surgeon will review your information and discuss surgical options with you.

In instances where the diagnosis may be less clear, clinical cardiologists will be engaged to obtain or review images from echocardiograms. We can perform the necessary diagnostic tests with expert interpretation, including:

• Echocardiography
• CT scanning to look for pericardial thickening
• Magnetic resonance imaging to look for both pericardial thickening and tethering of the heart tissue to the pericardium
• Right and left heart cardiac catheterization with fluid challenges, which is the most definitive test to demonstrate the response of the heart to changing conditions

Frequently, the imaging studies alone are sufficient to make the diagnosis and discuss with you your treatment options. Our cardiologists, surgeons and imaging specialists communicate with each other directly to ensure well-coordinated care.

If the inflammation is acute and the pericardium is swollen, anti-inflammatory agents may help the pericardium return to normal. However, if the condition is longstanding, there is no medical therapy and surgery is required.

Surgical Treatment

Surgery to remove all of the pericardium, called radical pericardiectomy, is the best option for chronic cases of constrictive pericarditis and, in most instances, results in return to normal heart function since the heart itself is unaffected by the condition. The surgical procedure requires removal of the pericardial sac from around the front and back of the heart. The procedure requires surgical expertise to ensure that all of the pericardium and all of the layers of the pericardium are removed.

There are normally two layers of pericardium, both of which have fluid in between them to lubricate the motion of the heart. In the case of constriction, these two layers are thickened and fused together. It is important that the surgeon removes both layers from the heart in order to relieve the constriction. Removing only the outer of the two layers will not solve the problem.

Remarkably perhaps, there are no long-term problems associated with removing the pericardium. In fact, there is even a congenital condition in which children can be born without a pericardium and live a normal life. Its normal function is to protect the heart and optimize its performance; however, in day-to-day life, this is unnecessary.

The primary risk associated with the procedure is damage to the phrenic nerves, which are the nerves that cause the diaphragm to move when you breathe. Phrenic nerve injury is an uncommon complication. With any surgery, there are risks. Speak with your care team about any concerns or questions.

What to Expect for Surgery

A pericardiectomy is performed most commonly through a median sternotomy, an incision in the front of the chest that divides the breastbone and gives complete access to the heart. Small incisions and minimally invasive approaches cannot provide complete removal of the pericardium which is necessary to treat the condition. In about 50% of cases, use of the heart-lung machine is required; however, in the other 50%, the procedure can be done without it.

Either way, the procedure takes about four hours in the operating room and overnight recovery in the intensive care unit. The length of hospital stay will depend on the individual's condition. Very frequently, patients have a great deal of extra fluid built up because of the longstanding constriction, and this may take several days to correct.

What to Expect Following Surgery

Patients typically feel an improvement to their heart symptoms very quickly following surgery and before leaving the hospital. Your vital signs will be closely monitored by your care team to ensure they’re functioning as expected. The recovery time is unique for each patient, but the average patient is typically discharged and able to return home one week following surgery.

In the weeks following surgery, patients will have a gradual return in their overall level of energy and appetite. You may be sent home with new medications, including diuretic pills to help eliminate the extra water your body has retained caused by constriction. You may also be set up on a beta-blocker to prevent atrial fibrillation, a common minor complication after heart surgery. Ask your doctor about how soon after your surgery you can return to daily activities, such as exercise and work.