Recurrent pericarditis and constrictive pericarditis — structured viva

Stem

Practice viva. The examiner asks how you define and treat pericarditis that keeps coming back, what you do when standard therapy fails, and how you recognise and manage constriction.^[1]

Branch A — Definitions

Examiner: What do incessant, recurrent and chronic pericarditis mean?^[1]

Strong answer:

• ESC 2025 Table 3 (Terminology and stages) uses ≤4 weeks for acute, >4 weeks to ≤3 months for subacute/incessant, and >3 months for chronic pericarditis; recurrent means new symptoms or disease activity after clinical remission.^[1]
• Incessant pericarditis describes persistent symptoms without a symptom-free interval of >4 weeks despite full-dose guideline-directed therapy, including corticosteroids, or early relapse during tapering.^[1]
• Recurrence after a first episode is 15%–30%, usually within 18 months, and may reach 50% after a first recurrence when the first episode was inadequately treated.^[1]
• Recognising incessant cases matters because they can progress directly to constrictive pericarditis within a few months.^[1]

Branch B — Treating a recurrence

Examiner: A 30-year-old man of 80 kg with normal renal function has his first recurrence. What do you prescribe?^[1]

Strong answer:

• High-dose aspirin or an NSAID with a proton pump inhibitor, recommended as first-line therapy to control symptoms and reduce recurrences (Class I, Level B); indomethacin is usually considered for incessant or recurrent cases.^[1]
• Colchicine, recommended as first-line therapy as an adjunct to aspirin/NSAID or corticosteroid therapy to reduce subsequent recurrences (Class I, Level A), at 0.5 mg twice daily for him (once daily is for <70 kg or severe renal impairment), maintained for at least 6 months in recurrent cases and withdrawn as the last drug only after stable remission.^[1]
• No corticosteroid first: it is not recommended as the first option without a specific indication (Class III, Level C).^[1]
• CORP (120 patients with a first recurrence, double-blind randomized trial) found recurrence at 18 months of 24% with colchicine versus 55% with placebo.^[6]

Follow-up: His response to the NSAID and colchicine is incomplete, so low- to moderate-dose prednisone is added as triple therapy. He then flares as prednisone is tapered. What now?^[1]

• Continue colchicine and add an NSAID instead of increasing the corticosteroid dose (ESC 2025).^[1]
• Every decrease in prednisone should be made only when he is asymptomatic and C-reactive protein is normal, particularly below 25 mg/day.^[1]

Branch C — When standard therapy fails

Examiner: A woman with recurrent pericarditis is corticosteroid-dependent and colchicine-resistant, with a raised C-reactive protein at every flare. What next?^[1]

Strong answer:

• Anti-IL-1 agents (anakinra or rilonacept) are recommended after failure of first-line therapies and corticosteroids with elevated C-reactive protein, to reduce recurrences and allow corticosteroid withdrawal (Class I, Level A).^[1]
• With pericardial inflammation on CMR after failure, contraindications and intolerance to first-line therapies and corticosteroids, they should be considered regardless of C-reactive protein (Class IIa, Level C).^[1]
• Anakinra is dosed at 1–2 mg/kg/day up to 100 mg/day in adults for at least 6 months, with tapering needed; rilonacept is not currently available in Europe, and anakinra may be preferable with increased infection risk.^[1]
• In RHAPSODY (phase 3 randomized-withdrawal trial in recurrent pericarditis with raised C-reactive protein), 2 of 30 patients (7%) on rilonacept recurred during the randomized-withdrawal period versus 23 of 31 (74%) on placebo; in AIRTRIP (double-blind, placebo-controlled randomized withdrawal trial among 21 consecutive patients enrolled at 3 Italian referral centres between June and November 2014, with ≥3 previous recurrences, raised C-reactive protein, colchicine resistance and corticosteroid dependence), 2 of 11 (18.2%) on anakinra recurred versus 9 of 10 (90%) on placebo.^[9][8]
• Hydroxychloroquine may be considered for disease refractory to standard therapy including corticosteroids and anti-IL-1 agents (Class IIb, Level B).^[1]

Branch D — Constriction

Examiner: A man has right heart failure, preserved ventricular function and a near-normal natriuretic peptide 2 months after cardiac surgery. How do you approach it?^[1]

Strong answer:

• Multimodality imaging is recommended in all patients with suspected constrictive pericarditis (Class I, Level C); cardiac catheterization for haemodynamic assessment should be considered when multimodality imaging is inconclusive (Class IIa, Level C).^[1]
• On echocardiography I look for the Mayo criteria: septal bounce or septal shift with either medial e' >8 cm/s or a hepatic vein expiratory diastolic reversal ratio >0.78.^[1]
• As this is a new diagnosis, if he is haemodynamically stable with raised C-reactive protein or pericardial enhancement on CMR, anti-inflammatory therapy is recommended to prevent progression to constriction and avoid pericardiectomy (Class I, Level C).^[1]
• If constriction is permanent without active inflammation, or anti-inflammatory treatment fails after 3–6 months, pericardiectomy is recommended (Class I, Level C), and it should be complete and done at a high-volume centre.^[1]
• Pericardial thickness can be normal in up to 20% of cases, so a normal thickness does not exclude constriction.^[1]