Transient Constrictive Pericarditis Releasing the Grip
Transient constrictive pericarditis (CP) is an increasingly recognized sub-type of the more typically known CP. It was first described back in 1987, when a small group of subjects with CP demonstrated spontaneous and permanent resolution of constrictive physiology on serial echocardiograms.1 This month, the European Society of Cardiology has formally recognized it as a variant of constrictive pericarditis in their updated Guidelines for management of pericardial diseases.2 Transient CP usually presents with symptoms of heart failure due to limited biventricular diastolic filling, however, transient CP can be reversible and hence may have a better prognosis. The incidence of transient CP is estimated to be 9-17% of all CP cases.1,3 Most cases of transient CP are described as idiopathic, but are presumably related to underlying viral pericarditis. It can also be precipitated by pericardiotomy for cardiac surgery, collagen vascular disease, bacterial pericarditis or chemotherapy.4-7 Of those who develop CP subsequent to acute pericarditis, approximately 15% will have transient CP and have resolution of symptoms within 3 months.8 Given the sometimes brief manifestation of this condition, it seems likely that it is under-recognized.
Pathologically, transient CP is usually a result of fluctuating pericardial edema, inflammation and fibrin deposition, rather than more fixed pericardial fibrosis or calcification.9 The resultant pericardial thickening leads to constrictive physiology. It may resolve spontaneously or with the assistance of anti-inflammatory therapy. It is likely that in some cases transient CP may actually represent an early manifestation in the spectrum of CP disease, before chronic inflammation leads to accumulation of fibroblasts and collagen and constrictive physiology becomes irreversible. Hence, recognition of this condition is crucial, to enable institution of pharmacological therapy, which may prevent progression to more chronic fibrotic CP. Like traditional CP, those with transient CP may present with signs and symptoms of predominantly right heart failure. However, due to the inflammatory etiology, there may be accompanying features of pericarditis including: fever, pleuritic chest pain and a frictional rub.3
Echocardiography remains the mainstay for identification of subjects with transient CP. A persistent pericardial effusion is a common feature and may be described as effusive CP or inflammatory epicarditis.10 This can be accompanied by elevated inflammatory markers such as: white cell count, erythrocyte sedimentation rate and C reactive protein. Other diagnostic echocardiographic features of constrictive physiology including: interventricular septal shift, septal bounce/shudder inferior vena cava plethora with impaired inspiratory collapse, increased respirophasic variation of mitral and tricuspid inflow velocities, and hepatic vein expiratory flow reversal. Additionally, Doppler imaging of the mitral annular tissue velocities may demonstrate a preferential reduction in the lateral early diastolic tissue velocity (e') compared with the septal value, referred to as "annulus reversus".11 This pattern also results in a relative increase in the mitral early diastolic inflow velocity (E wave) to the e', thereby resulting in a falsely elevated E/e' ratio, termed annulus paradoxus.12
Cardiac magnetic resonance imaging (CMR) has revolutionized our ability to diagnose and monitor both constrictive physiology and pericardial inflammation in transient CP. Standard cine steady-state-free-procession (SSFP) sequences can assess for pericardial effusions or thickening (>3mm), a diastolic septal bounce/shudder, conical deformity of the ventricles, epicardial tethering and IVC enlargement. A free breathing sequence can also enable identification of a respirophasic septal shift, a hallmark of ventricular interdependence and constrictive physiology. Active pericardial inflammation can also be readily identified with specific sequences. T2-weighted STIR spin-echo sequences demonstrate regions of increased water content, relative to surrounding tissue. Hence, increased pericardial signal suggests edema due to relatively acute pericardial inflammation. Gadolinium contrast also accumulates in regions of inflammation and fibrosis compared with normal tissue. This prolonged wash-out is exploited by CMR delayed enhancement (DE) imaging, where increased pericardial DE signal has been histologically validated against organizing pericarditis and neovascularization.13 Differentiation of pericardial DE from adjacent epicardial/pericardial fat can also be further optimized with the inclusion of a fat suppression pre-pulse. Pericardial DE and associated pericardial thickening >3mm has been found to be 86% sensitive and 80% specific for identification of transient CP. Of these patients, 93% had moderate to severe pericardial DE, along with more elevated inflammatory markers (ESR and CRP) than those with typical chronic CP.5 Recent imaging recommendations from the American Society of Echocardiography advocate multimodality imaging for assessment of pericardial disease such as transient CP.14 [Figure 1] Quantitative methods for assessment of pericardial DE have also been recently described.15
Typically, transient CP is managed with anti-inflammatory pharmacological therapy. This may involve a combination of NSAIDs and colchicine, plus steroids in refractory cases. Colchicine and NSAIDs reduce recurrence and decrease inflammation in the setting of acute pericarditis.16 Longer term use of NSAIDs in transient CP may warrant gastric protection with proton pump inhibitors. Although persistent, refractory cases of transient CP may necessitate oral steroid therapy, there is obvious caution recommended due to the association with pericarditis recurrence and systemic effects at higher doses (>1mg/kg/d).17 Refractory inflammation resulting in constrictive physiology may necessitate "triple therapy". Although this regimen can provide symptomatic improvement, outcome data regarding impact on recurrence remains lacking. Likewise, the use of steroid sparing agents, such as azathioprine, is becoming more widespread but remains largely un-validated in the non-rheumatological population. Recently, anakinra, which is a recombinant human interleukin-1 receptor blocker, has also shown promise in the treatment of recurrent pericarditis.18
Pericardiectomy is occasionally required for patients with refractory transient CP. However, active inflammation with associated exudative adhesions may make this challenging procedure even more technically difficult. Therefore, aggressive up-titration of anti-inflammatory therapy is recommended to try and avoid surgery or at least reduce the degree of active inflammation pre-operatively.
Transient CP is an increasingly recognized variant of classic chronic CP. It presents with heart failure due to impaired pericardial distensibility and thickening. However, the underlying pathology is typically one of acute or sub-acute inflammation, rather than fibrosis or calcification. Etiologies are multi-factorial. Echocardiography remains the mainstay for identification of constrictive physiology, however CMR now provides complimentary data regarding functional constriction and evidence of active inflammation on DE and T2 STIR weighted imaging. Systemic inflammatory markers can also be useful to follow disease activity. By definition, transient constriction will typically resolve spontaneously or with anti-inflammatory therapy. However, the optimal pharmacological regimen to prevent recurrence remains elusive. As diagnosis improves, NSAIDs, colchicine, steroids and immunomodulating agents are being increasingly prescribed. Pericardiectomy remains technically challenging and is reserved for refractory cases.
- Sagrista-Sauleda J, Permanyer-Miralda G, Candell-Riera J, Angel J, Soler-Soler J. Transient cardiac constriction: an unrecognized pattern of evolution in effusive acute idiopathic pericarditis. Am J Cardiol 1987;59:961-6.
- Adler Y, Charron P, Imazio M et al. 2015 ESC Guidelines for the diagnosis and management of pericardial diseases: The Task Force for the Diagnosis and Management of Pericardial Diseases of the European Society of Cardiology (ESC)Endorsed by: The European Association for Cardio-Thoracic Surgery (EACTS). Eur Heart J 2015.
- Haley JH, Tajik AJ, Danielson GK, Schaff HV, Mulvagh SL, Oh JK. Transient constrictive pericarditis: causes and natural history. J Am Coll Cardiol 2004;43:271-5.
- Maeda K, Saito S, Toda T, Ueno T, Kuratani T, Sawa Y. Transient constrictive pericarditis following cardiac surgery. Ann Thorac Cardiovasc Surg 2014;20 Suppl:897-900.
- Feng D, Glockner J, Kim K et al. Cardiac magnetic resonance imaging pericardial late gadolinium enhancement and elevated inflammatory markers can predict the reversibility of constrictive pericarditis after antiinflammatory medical therapy: a pilot study. Circulation 2011;124:1830-7.
- Allaria A, Michelli D, Capelli H, Berri G, Gutierrez D. Transient cardiac constriction following purulent pericarditis. Eur J Pediatr 1992;151:250-1.
- Woods T, Vidarsson B, Mosher D, Stein JH. Transient effusive-constrictive pericarditis due to chemotherapy. Clin Cardiol 1999;22:316-8.
- Imazio M, Brucato A, Maestroni S et al. Risk of constrictive pericarditis after acute pericarditis. Circulation 2011;124:1270-5.
- Taylor AM, Dymarkowski S, Verbeken EK, Bogaert J. Detection of pericardial inflammation with late-enhancement cardiac magnetic resonance imaging: initial results. Eur Radiol 2006;16:569-74.
- Syed FF, Ntsekhe M, Mayosi BM, Oh JK. Effusive-constrictive pericarditis. Heart Fail Rev 2013;18:277-87.
- Reuss CS, Wilansky SM, Lester SJ et al. Using mitral 'annulus reversus' to diagnose constrictive pericarditis. Eur J Echocardiogr 2009;10:372-5.
- Ha JW, Oh JK, Ling LH, Nishimura RA, Seward JB, Tajik AJ. Annulus paradoxus: transmitral flow velocity to mitral annular velocity ratio is inversely proportional to pulmonary capillary wedge pressure in patients with constrictive pericarditis. Circulation 2001;104:976-8.
- Bogaert J, Francone M. Cardiovascular magnetic resonance in pericardial diseases. J Cardiovasc Magn Reson 2009;11:14.
- 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.
- Cremer PC, Tariq MU, Karwa A et al. Quantitative assessment of pericardial delayed hyperenhancement predicts clinical improvement in patients with constrictive pericarditis treated with anti-inflammatory therapy. Circ Cardiovasc Imaging 2015;8.
- Imazio M, Brucato A, Cemin R et al. A randomized trial of colchicine for acute pericarditis. N Engl J Med 2013;369:1522-8.
- Imazio M, Brucato A, Cumetti D et al. Corticosteroids for recurrent pericarditis: high versus low doses: a nonrandomized observation. Circulation 2008;118:667-71.
- Jain S, Thongprayoon C, Espinosa RE et al. Effectiveness and Safety of Anakinra for Management of Refractory Pericarditis. Am J Cardiol 2015.
Keywords: Anti-Inflammatory Agents, Non-Steroidal, Azathioprine, Blood Sedimentation, C-Reactive Protein, Cardiac Surgical Procedures, Cell Count, Chest Pain, Colchicine, Collagen, Constriction, Echocardiography, Edema, Fibrin, Fibroblasts, Gadolinium, Heart Failure, Hepatic Veins, Inflammation, Interleukin 1 Receptor Antagonist Protein, Magnetic Resonance Imaging, Pericardial Effusion, Pericardiectomy, Pericarditis, Pericarditis, Constrictive, Pericardium, Proton Pump Inhibitors, Interleukin 1 Receptor Antagonist Protein, Vascular Diseases, Vena Cava, Inferior
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