A 47-year-old male presented with a one-week history of chest pain and shortness of breath. He had a past medical history significant for hypertrophic obstructive cardiomyopathy treated with septal myectomy one month before presentation. He described sharp, non-radiating chest pain that increased with deep inspiration and was relieved by leaning forward. He had a pericardial friction rub and elevated jugular venous pressure (JVP).
An echocardiogram was ordered and showed a moderate pericardial effusion, diastolic septal bounce with right atrial (RA) tethering and transmitral Doppler respiratory variation >45%. Cardiac magnetic resonance imaging (CMRI) was ordered and showed interventricular septal bounce, localized pericardial effusion adjacent to the right ventricle, thickened pericardium at 7 mm and circumferential pericardial late gadolinium enhancement (LGE) (Figure 1). Right and left heart catheterization showed pressure equalization in all chambers with a deep right atrial y descent. This confirmed the diagnosis of constrictive pericarditis (CP). Additionally, elevated inflammatory markers and marked late gadolinium enhancement represent active inflammation.
Figure 1
Left Panel: Four-chamber cardiac magnetic resonance image (CMRI) showing circumferential pericardial effusion, more prominent anterior to the right ventricle (white arrow).
Right Panel: Mild pericardial thickening and diffuse circumferential late-gadolinium enhancement due to inflammation.
Which of the following statements describes the best next step in the management of this patient?
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The correct answer is: C. Colchicine, ibuprofen, and refer for pericardiectomy if evidence of constriction persists and once LGE is less prominent.
Clearly the patient has acute pericarditis with hemodynamic features of constrictive pericarditis. This is a very difficult case since he had a sternotomy a month prior, which makes another open chest procedure very challenging. Furthermore, active pericardial inflammation indicated by high inflammatory markers, symptoms and LGE on CMRI signify friable pericardium (not organized), arguing against immediate pericardiectomy. Therefore, answer option D is incorrect. Steroids are not preferred as first-line agents for acute pericarditis due to the associated side effects and high association with recurrent pericarditis, which makes answer option A incorrect. Although colchicine is considered first-line therapy, it wouldn't be sufficient due to active severe inflammation as pointed out earlier. Answer option C, on the other hand, has a step-up approach with combined anti-inflammatory medications (colchicine and ibuprofen) for short period of time while the patient continues to be hemodynamically stable. This gives time for the active inflammation to subside and for the pericardium to get organized. If patient continues to have hemodynamic features of constrictive pericarditis after medical therapy, then pericardiectomy will be indicated and will be safer than if performed acutely.1-3
The patient had colchicine and ibuprofen for four weeks. During that time, he was hemodynamically stable. Repeat CMRI showed resolution of gadolinium enhancement but continued to show interventricular dependence with abnormal septal motion. At that point, the patient continued to need diuretics to maintain his dry weight. Therefore, he was referred to surgery and underwent total pericardiectomy with complete resolution of the hemodynamics changes. Six weeks later, he stopped anti-inflammatory medications.
References
Klein AL, Abbara S, Agler DA, et al. American Society of Echocardiography clinical recommendations for multimodality cardiovascular imaging of patients with pericardial disease: endorsed by the Society for Cardiovascular Magnetic Resonance and Society of Cardiovascular Computed Tomography. J Am Soc Echocardiogr 2013;26:965-1012.e15.
Imazio M, Brucato A, Cemin R, et al. A randomized trial of colchicine for acute pericarditis. N Engl J Med 2013;369:1522-28.
Alraies MC, Al Jaroudi W, Shabrang C, Yarmohammadi H, Klein AL, Tamarappoo BK. Clinical features associated with adverse events in patients with post-pericardiotomy syndrome following cardiac surgery. Am J Cardiol 2014;114:1426-30.