Management of Refractory Constrictive Pericarditis: A Challenge of Timing

A 62-year-old male with a history of hypertension, hyperlipidemia, coronary artery disease with prior percutaneous coronary intervention to the left anterior descending artery, hypothyroidism, and stage III chronic kidney disease presented to cardiology clinic with several months of poor exercise tolerance, bilateral lower extremity edema, and fatigue.

He was initially diagnosed with acute pericarditis over 1 year ago, when he presented to an outside facility with fevers, chills, and pleuritic chest pain. Inflammatory markers were elevated, with C-reactive protein 157 mg/dL on admission. Extensive infectious workup including testing for Epstein-Barr virus, cytomegalovirus, ehrlichiosis, Legionella, Rickettsia, and Borrelia burgdorferi was negative. Antinuclear antibodies (ANA) was negative as well. Ultimately the etiology of pericarditis was labeled as idiopathic. He improved with a prolonged course of indomethacin 15 mg twice daily and colchicine 0.6 mg every other day, but 6 months he later returned with dyspnea and weight gain. Workup at the time was notable for cardiac magnetic resonance imaging (MRI) demonstrating a moderate pericardial effusion, circumferential pericardial thickening, diffuse delayed pericardial enhancement, no delayed myocardial enhancement, and diastolic septal bounce. Right heart catheterization showed equalization of diastolic filling pressures. He was diagnosed with constrictive pericarditis. He was continued on colchicine and started on a slow prednisone taper along with low dose furosemide. Despite an additional 3 months of medical therapy, his heart failure symptoms worsened, and he was referred to our facility for further management.

His medications at this visit included: colchicine 0.6 mg every other day, prednisone 40 mg daily, furosemide 40 mg daily, levothyroxine 150 mcg daily, amiodarone 200 mg daily, metoprolol succinate 50 mg daily, aspirin 81 mg daily, rivaroxaban 20 mg daily, and double strength sulfamethoxazole-trimethoprim 800 mg – 160 mg three times per week. His vital signs were normal, with heart rate 57 bpm, blood pressure 116/66 mmHg, oxygen saturation 97% on room air, and no pulsus paradoxus. Physical examination was notable for elevated jugular venous pressure, Kussmaul's sign, a pericardial knock, and 1+ pitting bilateral lower extremity edema. Laboratory evaluation showed elevated creatinine (1.40 mg/dL), elevated NTproBNP (289 pg/mL), normal troponin T (<0.010), normal ultra-sensitive C-reactive protein (0.9 mg/L), and normal Westergren sedimentation rate (5 mm/hr). Electrocardiogram demonstrated normal sinus rhythm with T wave inversions in the inferior leads.

Cardiac MRI showed near concentric pericardial thickening, diastolic septal bounce, and mild respirophasic septal shift (Image A). There was no increased signal intensity of the pericardium on T2-weighted short-tau inversion recovery (STIR) sequences (Image B). Mild delayed enhancement of the pericardium adjacent to the basal anterior and lateral walls of the left ventricle was noted.

Images A & B

Left heart catheterization showed no angiographically significant coronary artery disease. Right heart catheterization demonstrated the following: mean RA pressure 17 mmHg, RV 40/20 mmHg, PA 36/24 mmHg with mean 26 mmHg, PCWP 24 mmHg, and LVEDP 22 mmHg. Cardiac index by Fick method was 4.17 L/min/m2.

The patient stated his symptoms were significantly impacting his quality of life and asked what else could be done.

What is the most appropriate next step in management?

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