The pericardium is a thick piece of tissue that envelops the heart but is only loosely attached. When it becomes inflamed, it will weep fluid into the space between the pericardium and the heart muscle.
When this fluid accumulates, it impedes the heart's ability to expand and take on the normal amount of blood required for each beat. As a result, the patient has a decreased amount of pumping action and feels short of breath.
These fluid accumulations can be caused by quite a few relatively common problems - viral illnesses, bacterial infections such as strep and staph, lupus, rheumatoid arthritis, kidney failure, and some medications.
But none of them seemed to fit in this patient's case. Furthermore, the results of all his routine blood tests were normal.
While the doctors were searching for a cause, they addressed his symptoms.
A small "window" was cut out of the bottom of the pericardium to allow the built-up fluid to drain into the area around the lung. This relieved the pressure in his heart and eased his breathing. The small piece of pericardium that had been excised was sent to the pathologist, who found nothing abnormal in the tissue.
The patient went home. Three months later, his symptoms returned, far worse than before. He could barely walk a few steps without gasping for air, his legs had swollen dramatically and his voice was weak.
He returned to the hospital for another echocardiogram. This time, it showed more than just fluid in the pericardium. The doctors found a thick rind of tissue constricting the heart muscle, essentially choking it.
What was going on?
The doctors decided he must be suffering from constrictive pericarditis, a condition usually seen in patients with a prior history of tuberculosis.
The patient seemed to remember having had a positive TB skin test as a child, but he said he was never treated for the disease. So the hospital did another skin test for TB.
It came up negative.
(No one blamed him for his glitchy memory. Several of his doctors, also children of the '60s, sympathized.)
They ordered a newly developed and more sophisticated blood test for TB. That, too, was negative.
They wondered, could it be sarcoid? Sarcoid is a noninfectious TB look-alike disease that can cause constrictive pericarditis.
They ordered the one blood test for this disease.
Again, the results were negative.
To be thorough, the doctors had the patient undergo another battery of tests for lupus, rheumatoid arthritis, and vasculitis, all of which are autoimmune diseases.
Every one returned negative.
The answer was clearly something rare.
Occasionally, lymphomas, melanomas, breast, and some glandular cancers can wend their way into the pericardium. But this is very uncommon. Furthermore, these diseases take a heavy toll on people. Other than his heart condition, this patient appeared well.
Without a diagnosis and with the patient complaining of ongoing and severe shortness of breath, his physicians decided the best course of treatment was to remove the entire front side of his pericardium. This would relieve the pressure and give the pathologists another crack at figuring out the problem.
As soon as he opened the man's chest and saw the pericardium, the heart surgeon ruled out TB, which usually looks and feels like an eggshell, forming a calcified ring around the heart within the pericardial sac.
Instead, he found a thickened rind, almost like a grapefruit peel.
Two days later, the pathologist reported that the patient had a glandular cancer. Most likely, it was an adenocarcinoma that had originated in the gastrointestinal tract.
The patient is now being treated with chemotherapy for this tumor.
John Stern is an infectious-disease doctor at Pennsylvania Hospital.