CardioExchange Archive

For our latest case, we are trying something new. We asked five esteemed clinicians for their differing perspectives on a challenging condition. Enjoy the discussion – and please let us know what you think.

A 35-year-old previously healthy physician with no risk factors for ischemic heart disease presented 4 months ago with symptoms of an upper respiratory infection, followed by severe chest pain. He was diagnosed with myopericarditis. His erythrocyte sedimentation rate and C-reactive protein level were within their reference ranges. An electrocardiogram revealed ST-segment elevations in the inferior and lateral leads. The patient’s troponin I level peaked at 30 ng/mL and then trended down to the normal range. MRI on presentation showed a patchy, subepicardial pattern of atypical late gadolinium enhancement (LGE) in the mid to apical lateral wall and the inferior wall. The left-ventricular ejection fraction (LVEF) was normal.

Four months later the patient returns reporting a chest-pain level of 2 out of 10. The pain is intermittent (once or twice daily) and pleuritic; it occurs during both rest and activity (no correlation). The patient has been taking ibuprofen 800 mg three times daily for the past month after two attempts to wean, as well as colchicine 0.6 mg twice daily for the past 3 months. His pain increases whenever he stops the ibuprofen. An echocardiogram documents an LVEF of 65% with no wall-motion abnormalities. Repeat MRI shows resolution of LGE in the inferior wall, and no evidence of pericarditis or pericardial enhancement was appreciated. Text from the MRI report appears below:

Impression: 1. Compared to prior exam from four months ago there has been interval improvement in the extent of myocarditis. No regions of elevated signal intensity were seen in the T2-weighted edema imaging to suggest acute myocarditis. Patchy, subepicardial pattern of atypical LGE is again noted in the mid to apical lateral wall. However, it has decreased in both intensity and extent since the prior study. The atypical LGE in the inferior wall is no longer present. LGE of the pericardium is not appreciated.

However, the study was reviewed by an independent outside reader who reported: “There is evidence of pericarditis with pericardial enhancement. There are no images to look for edema. The wall motion, chamber size, and EF are normal. There is one small area of myocardial enhancement.” The patient is concerned about persistent pain and wonders about therapeutic options.

Questions for Our Esteemed Clinicians — and Their Answers

1. What management approach would you recommend for this patient? Why?

James Fang, MD: Pericarditis is generally idiopathic (e.g., viral) in etiology, but when it becomes recurrent, it is important to exclude other systemic conditions such as lupus, tuberculosis, etc. Such an evaluation should take place before entertaining corticosteroid therapy for this patient. One should also be careful to recognize recurrent pericarditis as a condition associated with pain, not merely an effusion or an abnormal electrocardiogram. Interestingly, the case is described as myopericarditis rather than perimyocarditis. I would favor perimyocarditis, as it is likely that the persistent pericardial disease, rather than myocardial disease, is causing the patient’s symptoms to persist. The case is unlikely to be due to myocardial ischemia, although it can never be completely discounted in a middle-aged man.

James de Lemos, MD: This is a tough one — my initial inclination is to run the other way! We have a physician patient with a recalcitrant problem and no easy “next steps.” Fortunately, the myocarditis component seems to have almost completely resolved, but his symptoms persist and he cannot be weaned from high-dose NSAID therapy. I don’t see the need for additional testing, but I am aware of no good “evidence-based” treatments. The options include continued therapy with colchicine and NSAIDs, systemic corticosteroids, local (intrapericardial) administration of corticosteroids, or immunosuppressive therapy. Unfortunately, each of these approaches comes with safety concerns.

Kanu Chatterjee, MD: I have seen a number of patients like this one. They had myopericarditis, as troponin was elevated, and there was also evidence for pericarditis (pericardial friction rub, pericardial effusion). The patient in this case has now developed “recurrent pericarditis syndrome.” This syndrome is characterized by recurrent pericardial pain. During recurrence, pericardial rub or pericardial effusion may or may not be present. It is of interest that this patient’s erythrocyte sedimentation rate and C-reactive protein level remain normal. One should start colchicine, 0.6­­–1.2 mg daily, for about a year. This patient is already taking an adequate dose of colchicine.

Thomas Ryan, MD: This is quite consistent with my experience over the years, largely college students with viral pericarditis, a fair percentage with recurrent and refractory chest pain. Dave Spodick, who was here in Boston before going to Worcester, introduced me to colchicine when he first discovered its usefulness. Fortunately, not much has changed in approaching the management of these patients, even with all the cardiac magnetic resonance and slicker imaging techniques we have today.

I would use colchicine 0.6 mg twice daily for about 3 months, and if the patient develops signs of chest pain while on that, I would give ibuprofen 800 mg three times daily for about 2 weeks and then taper that down. If he broke through and had pain while tapering, I would start with corticosteroids, about 40 mg of prednisone. I would give that for 2 weeks before I reduced it to 30 mg for 2 weeks and then 20 mg for 2 weeks. Then at 20 mg, I would reduce it by 1 mg every 2 weeks.

Be careful: If he breaks through twice during the steroid weaning, he will break through a third time. If that happens, I would use pericardial stripping, as recently published Mayo data support. In these patients, in whom medical management has failed, pericardial stripping was found to be safe and effective in relieving symptoms, with minimal complications.

Rick A. Nishimura, MD:  This patient probably has a residual “chronic relapsing pericarditis.” Management involves either a high-dose nonsteroidal anti-inflammatory drug (NSAID) or a high-dose salicylate, in addition to the colchicine. In this particular case, the NSAID appears to be ineffective, so a high-dose salicylate (650–1300 mg) should be given every four hours. Checking that the salicylate level is adequate (10–20 mg/dL) will help to calibrate the dosage. After complete relief of symptoms, the salicylates should be tapered very slowly over several months, with the colchicine maintained for at least 3 months after symptoms cease.

2. Would you offer corticosteroid therapy? Would you offer other immunosuppressive therapy?

James Fang: The patient appears to have had a reasonable trial of both NSAID therapy and colchicine. At this point, corticosteroid therapy can reasonably be offered, but weaning can take a long time often must be done very slowly. Other immunosuppressive therapies (e.g., aziothioprine, mycophenolate mofetil, cyclosporine) have been used, but their risks are probably greater than those from corticosteroids in most patients; therefore, they should be reserved for patients who do not tolerate corticosteroids. In rare instances, direct intrapericardial injection of corticosteroids or pericardiectomy is warranted.

James de Lemos: I would offer a trial of intrapericardial administration of corticosteroids — if I could talk one of our interventionalists or electrophysiologists into doing it! Interestingly, our electrophysiologists do several procedures in which they enter a pericardium without an effusion and now have more experience with it than our interventionalists do. I would try to avoid systemic corticosteroids, as weaning a pericarditis patient from them is difficult. I would avoid immunosuppressive therapy at this point, as the patient has no heart failure and the myocarditis has improved.

Kanu Chatterjee: It is better to avoid corticosteroids, as the recurrence rate is quite high (about 30% to 50%). To control inflammation, ibuprofen is preferable, initially 1200–1600 mg/day, with the dose tapered slowly each week. Rather than ibuprofen, one may use indomethacin or, instead, aspirin 325–600 mg/day. The doses should be tapered quickly. Corticosteroid therapy should be considered as a last resort. Other immunosuppressive therapy such as melphalan has been used without any benefit. Pericardiectomy has been done to prevent recurrence, but the results have been disappointing.

Thomas Ryan: I would offer corticosteroid therapy. All the scanning that these patients undergo (with cardiac magnetic resonance of one kind and another) does not seem to prevent the breakthrough. You give the steroid wean two good tries, and if the patient breaks though again, there is no point in trying a third time and you consider stripping.

Rick A. Nishimura: I would be very reluctant to offer corticosteroid therapy. It has been shown that patients with acute pericarditis who receive steroids tend to have multiple relapses when weaned off them. Thus, if at all possible, steroid therapy should be avoided. One could consider immunosuppressive therapy if this were giant-cell myocarditis, which would respond to such therapy; however, the patient’s clinical presentation does not suggest that. An agent used to treat autoimmune disorders, such as an interleukin-1 blocker, might be considered, but this would be on an investigational basis. If all of those treatments fail, complete pericardiectomy would be effective.

3. How would you counsel this patient about his prognosis?

James Fang: The overall prognosis is generally good, and progression to large effusions, tamponade, and/or constriction is relatively uncommon. Chronic pain is a possibility, though also uncommon, but can wax and wane over time. Careful attention to corticosteroid therapy and appropriate weaning usually resolves the issue.

James de Lemos: I would tell him that his prognosis is good with respect to survival and “major events,” but I would be honest that I can’t promise that medications will take his pain away completely. I would also tell him that pericardiectomy could be considered as a last resort but that I hope we would not get to that point.

Kanu Chatterjee: The prognosis of patients with recurrent pericarditis is excellent. The patient in this case should be assured that his prognosis is very good. However, given the evidence of myocarditis, he should be followed for arrhythmias. There have been rare reports of sudden cardiac death in patients with myocarditis. This patient’s MRI suggests resolving myocarditis. He should be told that symptom recurrence is certain, initially frequent with gradual lessening. Upon recurrence, ibuprofen should be restarted. Colchicine should be continued.

Thomas Ryan: I would simply tell him, “Don’t sweat it. If you break through after the steroid wean, we can always go through a stripping, and the statistics are really favorable. You just have to be patient.”

Rick A. Nishimura: Overall, this patient should have an excellent prognosis. His ventricular function is normal following his myocarditis. The major limiting problem would be a “relapse” of the pericarditis, which occurs in 30% to 50% of patients.

Now share your response to this nuanced case.