An Overview of Constrictive Pericarditis

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Constrictive pericarditis is a chronic condition in which the pericardium (the membrane-like sac that encloses the heart), becomes stiffened and inelastic. As a result, cardiac function deteriorates. This condition, which fortunately is quite uncommon, always has serious consequences. 

Symptoms of constrictive pericarditis tend to be similar to symptoms seen with other kinds of heart disease, and their onset is insidious.

This means that there is often a substantial delay in making the correct diagnosis. Most typically, by the time the diagnosis is made, surgical treatment is the only viable option.

What Is Constrictive Pericarditis?

The pericardial sac—or pericardium—is the fibrous, elastic, fluid-filled sac that encloses and protects the heart. The pericardium limits excessive movement of the beating heart in the chest lubricates the heart to reduce friction as it works and protects it from infection. 

Certain diseases can produce inflammation within the pericardium (a condition called pericarditis), and if the inflammation persists long enough or becomes particularly severe, eventually the pericardial sac may become scarred and thickened, causing it to lose its elasticity. The stiffened pericardial sac can have the effect of “squeezing” the heart, thus restricting its ability to fill with blood. This condition is called constrictive pericarditis.

With constrictive pericarditis, the amount of blood the heart is able to pump with each heartbeat can become significantly limited, due to the heart’s inability to fill completely. Because the blood returning to the heart from the body can become backed up within the vascular system, people with constrictive pericarditis often develop significant fluid retention, which can lead to severe edema (swelling).

Constrictive pericarditis is usually a chronic, progressive disorder; that is, it begins relatively gradually and becomes worse over time. So its symptoms also tend to worsen gradually and can be ignored or written off (say, as “old age”), for months or even years. However, the symptoms get relentlessly worse as time passes. The symptoms caused by constrictive pericarditis are very similar to the symptoms produced by other, far more common types of heart disease—so the correct diagnosis can be delayed. Once the diagnosis of constrictive pericarditis is made, surgery to relieve the cardiac constriction is usually required.

Causes

Constrictive pericarditis can result from almost any disease or disorder that can cause inflammation in the pericardium. In particular, just about any medical problem that can cause acute pericarditis can also result in constrictive pericarditis. However, constrictive pericarditis is not a common consequence of acute pericarditis—in a study of 500 people who had acute pericarditis, only 1.8 percent of them developed constrictive pericarditis during six years of follow-up.

Constrictive pericarditis has been seen to follow acute pericarditis caused by all of the following:

For reasons that are not at all clear, constrictive pericarditis appears to be a rare event in people who have chronic or recurrent pericarditis. When it occurs, it appears to follow an episode of acute pericarditis.

Symptoms

The onset of constrictive pericarditis is often insidious, with symptoms very gradually worsening over a period of months or years.

Because the symptoms can worsen gradually, because the symptoms are often similar to those caused by other, much more common forms of heart disease, and because constrictive pericarditis can be difficult to diagnose unless the doctor specifically looks for it, the diagnosis of this condition is often delayed.

The symptoms of constrictive pericarditis tend to feature symptoms common with heart failure, especially dyspnea (shortness of breath), paroxysmal nocturnal dyspnea, poor exercise tolerance and easy fatiguability, rapid heart rate (tachycardia), and palpitations. People with constrictive pericarditis can also develop chest pain, steering doctors toward a diagnosis of coronary artery disease with angina.

Constrictive pericarditis can also produce fluid retention, which can become quite severe. This fluid retention often causes edema in the legs and abdomen. The abdominal edema may become severe enough to cause nausea, vomiting, and liver dysfunction, sending the doctor in the direction of trying to diagnose a gastrointestinal disorder. In fact, there have been cases in which patients referred for liver transplantation because of presumed primary liver failure turned out to have undiagnosed constrictive pericarditis as the underlying cause.

Diagnosis

As we have seen, constrictive pericarditis is pretty rare and its onset is often gradual, so it is common for doctors to think first of other medical problems that can cause the same kinds of symptoms, such as heart failure, coronary artery disease, liver disease, or other gastrointestinal conditions. 

To complicate things even further, when the doctor becomes focused on the idea that restricted cardiac filling may be the main issue, it turns out that there are several other cardiac conditions that also restrict cardiac filling. These conditions can be quite difficult to distinguish from constrictive pericarditis. They include diastolic heart failurerestrictive cardiomyopathy and cardiac tamponade.

The real key to diagnosing constrictive pericarditis is, first, for the doctor to think of the possibility in the first place, and then to do the necessary testing to specifically look for it. 

Echocardiography often gives several important clues to the presence of constrictive pericarditis and is often the first test that is done to screen for this condition. A thickened or calcified pericardium can be detected in almost half the people who have constrictive pericarditis, and dilation of the major veins that empty into the heart can also be seen frequently. The dilation is caused by the “backing up” of the blood returning to the heart. 

CT scanning is useful in clinching the diagnosis. Thickening of the pericardium is easier to detect with a CT scan than with echocardiography. Also, the CT scan often provides information that can be quite useful in planning surgical treatment. 

Cardiac MRI imaging is even more reliable than CT scanning in detecting abnormal thickening of the pericardium and is regarded by many experts as the study of choice when constrictive pericarditis is suspected. Furthermore, MRI imaging can be particularly helpful in revealing detailed anatomic information that is important in the surgical treatment of this condition. 

In some cases, however, even with the availability of modern non-invasive techniques, a cardiac catheterization may be necessary to help confirm the diagnosis of constrictive pericarditis. 

Again, the main point is that constrictive pericarditis is correctly diagnosed when the right tests are done, and the doctors performing the tests are alerted to the suspicion that constrictive pericarditis may be present.

Treatment

By the time it is diagnosed, constrictive pericarditis is almost always a chronic disorder that has been getting progressively worse over time. In people who have had constrictive pericarditis for at least several months by the time of diagnosis, the condition is permanent and is very likely to keep getting worse. So, in the majority of people diagnosed with constrictive pericarditis, surgical treatment is recommended right away. 

However, in some cases, constrictive pericarditis is diagnosed very early in its course. When this is the case, the possibility exists that aggressively treating the underlying cause of the problem can reverse the constrictive pericarditis, and render it a transient condition.

So, if constrictive pericarditis is newly diagnosed in a person whose cardiac symptoms appear to be mild and stable, and in whom the underlying medical condition producing the constrictive pericarditis is judged to be treatable, surgical therapy can be delayed for a few months, while aggressive treatment of the underlying medical disorder is undertaken.

When this course of action is undertaken, with luck the damage to the pericardium can be stopped and even reversed. However, during this period of time the patient needs to be carefully monitored for signs of deterioration. And, if no improvement is seen within two or three months, surgery should be done. The longer surgery is delayed, the more difficult the treatment is likely to become.

Surgery

The only effective treatment for most people who have constrictive pericarditis is to surgically remove a substantial portion of the thickened, fibrous pericardial sac—a procedure called pericardiectomy. By removing the thickened pericardium, the heart is no longer constricted, the restriction on cardiac filling is relieved, and the heart itself is freed to begin functioning normally again.

Pericardiectomy is very often a difficult and challenging procedure. This is the case partly because the diseased pericardial sac is often adherent to the cardiac muscle, making the procedure technically very difficult. (This is why the anatomic information provided by CT scanning and cardiac MRI can be so helpful in planning the surgery.)

Pericardiectomy surgery also tends to be difficult because the diagnosis of constrictive cardiomyopathy is often missed until the patient is extremely sick, and therefore has a greatly elevated surgical risk. In fact, in people who have end-stage constrictive pericarditis, surgery is more likely to hasten death than to improve things.

Because pericardiectomy is so difficult to perform, and because it is a relatively uncommon procedure, whenever possible it ought to be performed in major cardiac centers where the surgeons have substantial experience with this challenging procedure.

A Word From Verywell

Constrictive pericarditis is a chronic, progressive disorder in which the pericardial sac becomes thick and stiffened, and restricts the filling of the heart. Mild cases can sometimes be treated by aggressively addressing the underlying medical disease, but usually, surgical treatment is necessary. Early diagnosis is the key to successful treatment.

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Article Sources
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