Recurrent Pericarditis Post-Pericardiectomy: "Does a Stitch in Time Save Nine?"

A 40-year-old female presents to the office with recurrent symptoms of pericarditis. Her symptoms are typical for a flare of her recurrent pericarditis, which include pleuritic chest pain radiating to her left shoulder, shortness of breath and constitutional symptoms of feeling unwell and lethargic.

In the last five years, she has had approximately 16 flares of chest pain, three emergency department visits for chest pain, two of which required overnight admissions, and a pericardiocentesis. Her symptoms began six weeks post mitral valve surgery. Her autoimmune serology workup was weakly positive for antinuclear antibodies. No relevant family history of connective tissue disease or vasculitis.

She was initially treated with ibuprofen and colchicine, with the addition of prednisone and azathioprine commonly referred to as quadruple therapy. Due to an intolerance of azathioprine, she was then commenced on anakinra as a steroid minimizing immunosuppressant.

Despite various combinations of immunosuppressant therapies, her symptoms remained debilitating, with ongoing steroid dependence. The decision was made to undergo a radical pericardiectomy. Pathology of the excised pericardium exhibited fibrous adhesions macroscopically, with neovascularization and sparse acute and chronic inflammatory cell infiltrate microscopically.

She enjoyed a brief symptom free period post-pericardiectomy, however experienced further flares when her anakinra and colchicine were tapered.

She had a repeat cardiac magnetic resonance imaging (MRI) (CMR) during a presentation to the hospital with a flare post-pericardiectomy, which demonstrated circumferential inflammation in a region that was defined on T1 black blood imaging (Figure 1), Black Blood STIR Imaging, (Figure 2a), PSIR sequence post-gadolinium contrast (Figure 2b),  and PSIR Fat Sat LGE sequence (Figure 2c). A novel T1 MOLLI mapping sequence that is specific for fibrosis and edema confirmed the findings of the previous sequences with the demonstration of increased values circumferentially (Figure 2d).

Her dose of anakinra was up titrated and she is on intermittent ibuprofen in addition as ongoing maintenance therapy. A repeat CMR demonstrated normalization of her T1 MOLLI mapping values in the sub visceral pericardial region that was previously elevated, suggestive of resolution of the previously demonstrated inflammation and edema. (Figure 2e)

Figure: Panel of CMR Sequences

Figure 1
The arrows indicate: 1a. Epicardial Fat in T1 Black Blood Short Axis Sequence; 2a. Increased signal intensity in the myocardium on T2 STIR Sequence; 2b. Late gadolinium enhancement in the epicardial region in PSIR 4 chamber Sequence; 2c. Late gadolinium enhancement in PSIR Fat Suppressed mid ventricular short axis Sequence; 2d. Increased T1 mapping values in the region of the Epicardial fat in a T1 map of the mid ventricle in short axis; 2e. Normalization of T1 mapping values in the region of the Epicardial fat in a T1 map of the mid ventricle in short axis in a CMR study performed.

What is the potential cause of ongoing inflammation and/or symptoms of pericarditis in this patient post pericardiectomy?

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