Recurrent pericarditis and constrictive pericarditis — structured written assessment

SAQ 1 (10 marks)

Practice scenario. A 36-year-old woman (64 kg, normal renal function) has her third episode of pericarditis in 14 months, with pleuritic pain and a raised C-reactive protein. Each earlier episode was treated with ibuprofen for 2 weeks without colchicine, and she was symptom-free and off treatment between episodes. Her sister has had recurrent pericarditis.^[1]

1. How does ESC 2025 define recurrent pericarditis, and how does it differ from incessant pericarditis? (2)^[1]
2. Which ESC 2025 row supports colchicine for her, and what dose and duration does ESC 2025 give? (2)^[1]
3. Give two features of her history that ESC 2025 links to recurrence, or to a genetic predisposition. (2)^[1]
4. A year later she has corticosteroid dependence, colchicine resistance and raised C-reactive protein at each flare. Which ESC 2025 row applies? Give class and level. (2)^[1]
5. Summarise one randomized trial of an anti-IL-1 agent in recurrent pericarditis: design, population and main result. (2)^[8][9]

Model answers — SAQ 1

1. Recurrent pericarditis is a relapse after a documented first episode, a symptom-free interval, complete discontinuation of anti-inflammatory therapy, and evidence of subsequent recurrence (1 mark).^[1] Incessant pericarditis means persistent symptoms without a symptom-free interval of >4 weeks despite full-dose guideline-directed medical therapy (including corticosteroids), or early relapse during tapering (1 mark).^[1]
2. Colchicine is recommended as first-line therapy as an adjunct to aspirin/NSAID or corticosteroid therapy to reduce subsequent recurrences (Class I, Level A) (1 mark).^[1] Dose 0.5 mg once daily if <70 kg or with severe renal impairment, otherwise 0.5 mg twice daily, so 0.5 mg once daily for her at 64 kg; in incessant or recurrent cases it is maintained for at least 6 months (1 mark).^[1]
3. Inadequate treatment of the first episode, such as no colchicine, can raise recurrence to 50% after a first recurrence (1 mark).^[1] Familial occurrence of pericarditis has been reported among the relatives of idiopathic recurrent pericarditis patients, ranging from 4% to 10%, and mutations associated with autoinflammatory disorders in up to 10% of patients with multiple recurrences, especially with a positive family history and a poor response to colchicine (1 mark).^[1]
4. Anti-IL-1 agents (anakinra or rilonacept) are recommended for recurrent pericarditis after failure of first-line therapies and corticosteroids and elevation of C-reactive protein, to reduce recurrences and allow corticosteroid withdrawal (1 mark).^[1] Class I, Level A (1 mark).^[1]
5. AIRTRIP: double-blind, placebo-controlled randomized withdrawal trial in 21 consecutive patients at 3 Italian referral centres (enrolled June to November 2014) with ≥3 previous recurrences, raised C-reactive protein, colchicine resistance and corticosteroid dependence; open-label anakinra for 2 months, then responders were randomized to anakinra or placebo for 6 months or until a recurrence (1 mark); recurrence in 9 of 10 (90%) on placebo versus 2 of 11 (18.2%) on anakinra (1 mark).^[8] Or RHAPSODY: phase 3 double-blind randomized-withdrawal trial; 86 patients with a recurrence and raised C-reactive protein entered a 12-week rilonacept run-in and 61 were randomized to rilonacept or placebo; recurrence 7% versus 74% (2 marks).^[9]

SAQ 2 (10 marks)

Practice scenario. A 61-year-old man has had progressive ankle swelling, abdominal distension and breathlessness for 3 months after coronary bypass surgery. His jugular venous pressure is raised, NT-proBNP is near normal, and left and right ventricular systolic function are preserved. He is haemodynamically stable.^[1]

1. What is the likely diagnosis, and which ESC 2025 features support it? (2)^[1]
2. Give the ESC 2025 row on imaging in suspected constriction and the echocardiographic criteria it quotes. (2)^[1]
3. C-reactive protein is raised and CMR shows pericardial late gadolinium enhancement. What does ESC 2025 recommend? Give class and level. (2)^[1]
4. Four months later constriction persists, C-reactive protein is normal and CMR shows no enhancement. What does ESC 2025 recommend, and what kind of operation? (2)^[1]
5. He has severe tricuspid regurgitation. What does ESC 2025 recommend at surgery? Give class and level. (2)^[1]

Model answers — SAQ 2

1. Constrictive pericarditis; post-cardiac surgery is a common reported cause in developed countries (11%–37%) (1 mark).^[1] ESC 2025 says the classic clinical picture is usually characterized by isolated right heart failure with normal or nearly normal natriuretic peptide levels, and the classical presentation comprises signs and symptoms of right heart failure with preserved biventricular function without previous or concomitant myocardial disease (1 mark).^[1]
2. Multimodality imaging is recommended in all patients with suspected constrictive pericarditis to make the diagnosis and assess pericardial thickening, calcifications and active inflammation (Class I, Level C) (1 mark).^[1] Mayo Clinic criteria: septal bounce or ventricular septal shift with either medial e' >8 cm/s or hepatic vein expiratory diastolic reversal ratio >0.78 (sensitivity 87%, specificity 91%) (1 mark).^[1]
3. Anti-inflammatory therapy is recommended in haemodynamically stable patients with a transient or new diagnosis of constriction with concomitant evidence of pericardial inflammation, to prevent progression to constriction and avoid pericardiectomy (1 mark).^[1] Class I, Level C (1 mark).^[1]
4. Pericardiectomy is recommended in permanent constriction if there is no active inflammation or anti-inflammatory treatment is not successful after 3–6 months (Class I, Level C) (1 mark).^[1] Complete (radical) pericardiectomy at a high-volume centre with the required expertise, rather than partial anterior or anterophrenic resection (1 mark).^[1]
5. Tricuspid valve repair is recommended in pericardial constriction with severe tricuspid regurgitation to improve symptoms and survival (1 mark).^[1] Class I, Level C; ESC 2025 says supporting evidence is limited (1 mark).^[1]