Refractory Recurrent Pericarditis After Pericardiectomy in a Young Woman

Pericardiectomy is the recommended treatment for patients with recurrent pericarditis and refractory symptoms despite optimal anti-inflammatory therapy. We present a case of a 40-year-old woman who underwent total pericardiectomy after multiple episodes of pericarditis that was refractory to optimal guideline-derived medical therapy, including anti-inflammatory and biologic agents, who continued to have relapsing symptoms even after pericardiectomy. (Level of Difficulty: Intermediate.)


MEDICAL HISTORY
In 2015, the patient underwent mitral valve repair for mitral valve prolapse. Six weeks after cardiac surgery, she experienced a fever of 102 F, sharp pleuritic chest pain that worsened on lying flat, and shortness of breath. Her NT-proBNP on initial visit was 87 pg/mL (normal value <125 pg/mL).

LEARNING OBJECTIVES
To understand the role of serial cardiac magnetic resonance imaging in the treatment of patients with complex recurrent pericarditis. To understand the role of pericardiectomy in patients with refractory recurrent pericarditis without the presence of physiologic constriction.
To be able to understand that a small subset of patients may experience recurrent flares of pericarditis after pericardiectomy and may require novel immunosuppressive therapies and cardiac magnetic resonance imaging for optimal management.

DIFFERENTIAL DIAGNOSIS
On the basis of the patient's presenting symptoms and the relevant investigations, she received a diagnosis of acute pericarditis.
Differential diagnoses included, but were not limited to, acute coronary syndrome, pulmonary embolism, gastroesophageal reflux disease, and costochondritis, all of which were systematically excluded.       Patient reported worsening pleuritic chest pain; elevation in inflammatory markers to ESR (15 mm/h) and US-CRP (6.95 mg/dL) while tapering anakinra CMR in 2019 demonstrated increased gadolinium uptake on DHE, and T2 STIR sequence showed increased signal intensity; anakinra was increased to once daily again, but patient continued to experience worsening symptoms; colchicine 0.6 mg orally twice daily was added to anakinra along with ibuprofen 400 mg three times daily as needed April 2019 to September 2020 Repeat CMR in September 2020 demonstrated qualitative reduction in the intensity of gadolinium uptake on DHE imaging and normalization of signal intensity on T2 STIR imaging CMR ¼ cardiac magnetic resonance imaging; DHE ¼ delayed hyperenhancement; ESR ¼ erythrocyte sedimentation rate; STIR ¼ short T1 inversion recovery; US-CRP ¼ ultrasensitive C-reactive protein.
T1 mapping may be of utility to detect active inflammation and/or gauge the adequacy of response to therapy. At present, these techniques are novel and are used primarily for research purposes; however, they are promising and require further validation in prospective outcome studies.
TREATMENT. Surgical pericardiectomy is indicated in patients with symptomatic pericardial constriction, those with refractory recurrent pericarditis despite optimal medical therapy, and those with severe intolerance to medical therapy (11,12).

FOLLOW-UP
The dose of anakinra was increased to once daily again, but the patient continued to experience worsening symptoms. Colchicine 0.6 mg orally twice daily Refractory Recurrent Pericarditis After Pericardiectomy in a Young Woman was added to anakinra along with ibuprofen 400 mg three times daily as needed. Her symptoms were moderately controlled. A repeat cardiac MRI in 2020 demonstrated a qualitative reduction in the intensity of the gadolinium uptake on DHE imaging ( Figure 2C) with normalization of signal intensity on T2 STIR imaging ( Figure 3B).

CONCLUSIONS
We present the case of a 40-year-old woman who experienced refractory recurrent pericarditis associated with PPS after mitral valve surgery, with a complex clinical course resulting ultimately in pericardiectomy. She continued to experience chest pain suggestive of a recurrence after pericardiectomy and was again given anti-inflammatory therapy for symptomatic relief. Twitter: @AllanLKleinMD1.