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Large chronic idiopathic pericardial effusion: to tap or not to tap

Case presented by Working Group on Myocardial and Pericardial Diseases



The case

Presented by: Massimo Imazio, MD, FESC.  

Cardiology Department, Maria Vittoria Hospital and Department of Public Health and Pediatrics, University of Torino, Torino, Italy.

E-mail: massimo_imazio@yahoo.it; massimo.imazio@unito.it

Case presentation

A 69-year-old Caucasian female patient, with a previous diagnosis of Rheumatoid Arthritis (RA) and Sjogren Syndrome (SS), was referred for a second opinion. She has a known large chronic idiopathic pericardial effusion since 2010.

In March 2011 and September 2011 she underwent 2 diagnostic pericardiocentesis without pericardial drainage. The pericardial effusion always reappeared after few days. She also attempted empiric anti-inflammatory therapies with NSAID and colchicine without significant changes of the size of the pericardial effusion. The aetiological testing was negative and the final diagnosis was large idiopathic pericardial effusion. She was always asymptomatic and the pericardial effusion was detected incidentally following a chest-x-ray and a transthoracic echocardiogram.
At the visit she is asymptomatic without evidence of distended neck veins. The ECG shows sinus rhythm with low QRS voltages (Figure 1).

Figure 1. Low QRS voltages on ECG.

Picture 1

On transthoracic echocardiography a large pericardial effusion (maximal telediastolic echo-free space of 36mm) is detected without clinical and echocardiographic signs of cardiac tamponade.

Figure 2. Large pericardial effusion (>20mm) without significant changes of mitral E velocities with respiratory phases and maintained changes of inferior vena cava size with respiration.

Picture 2

Questions

  1. What is your next diagnostic step?
  2. What medical therapy would you suggest?
  3. How would you plan the follow-up?

A large pericardial effusion (>20mm of telediastolic free-space) is defined chronic when lasting for > 3months. It is labelled “idiopathic” when the etiological evaluation is negative as in this case even after the analysis of the pericardial fluid.

In asymptomatic patients the finding may be incidental and the outcome is poorly known.

In the largest reported study from the Barcelona group (1), 28 cases were reported: 13 of 28 (46%) were asymptomatic. After a mean follow-up of 7 years cardiac tamponade developed in 8/28 (29%), pericardiocentesis was necessary in 24/28 (86%) and pericardiectomy was performed as final therapy in 20/28 (71%).

On this basis, the authors recommend to resort to pericardiectomy whenever a large pericardial effusion recurs after pericardiocentesis since cardiac tamponade may occur unexpectedly in about one third of cases.

This is a poorly studied condition with divergent opinions among experts since an alternative strategy of “wait and see” may be equally adopted (2,3).

In this case, since the patient was completely asymptomatic without any physical or echocardiographic sign of cardiac tamponade, we decided to wait and monitor the effusion within 3 months. Since it was unchanged we decided to perform another pericardiocentesis with prolonged drainage in order to check again the pericardial fluid and test the possible efficacy of prolonged drainage to prevent the reaccumulation of pericardial fluid.

The evolution of the pericardial effusion is reported in the figure below.

Picture 3

The effusion reappeared despite the pericardiocentesis and worsened after an episode of pericarditis. After this worsening the patient remained asymptomatic for > 1year.

Anti-inflammatory therapies, including colchicine,  have been shown not to be efficacious in the absence of inflammation and pericarditis (4) and thus were performed only to treat the episode of pericarditis.

This case shows that a chronic large effusion may be asymptomatic and stable for long period of time. Precipitating events such as pericarditis or trauma may be responsible of worsening and should be considered in the decision to treat. Careful echocardiographic follow-up are warranted every 3 to 6 months depending on symptoms, pericardial effusion size changes, and the management should be tailored to the single patient according to his/her preferences and evolution. Pericardiectomy is generally the last option to be carefully considered and offered in well-experienced surgical centres since it is a long and demanding cardiac surgery operation. A pericardial window is often an alternative option to be considered.

The solution of the previous case of the month: Chest pain, electrocardiographic changes and pericardial effusion. What is it ?

Angelica Peritore1, Massimo Imazio2, Alberto Roghi3 and Patrizia Pedrotti3
1 Bicocca University, Milano, Italy; 2 Ospedale Maria Vittoria, Cardiology, Torino, Italy; 3 Ospedale Niguarda Cà Granda, Cardiac Magnetic Resonance Laboratory, Milano, Italy

  1. What is your diagnosis?
  2. Would you have performed cardiac MRI?
  3. How should this case be managed?
  1. Pericarditis is an inflammatory disorder affecting the pericardium, which is the membranous sac that encapsulates the heart. The diagnosis of acute pericarditis is exclusively based on clinical criteria. It should meet at least two of the following criteria: typical chest pain, pericardial friction rub, suggestive electrocardiogram (ECG) and/or new or worsening pericardial effusions. An elevated C-reactive protein level on high-sensitivity testing, although nonspecific, is also supportive. Although the differential diagnosis of chest pain is extensive, certain features point strongly to pericarditis, especially pleuritic pain that is relieved by sitting forward and that radiates to the trapezius ridge. In the case described, the patient presented with two of the above-mentioned criteria namely, typical chest pain and new appearing pericardial effusion. Patient had sinus tachycardia and fever that are also common features.
    Acute pericarditis has numerous causes. However, in developed countries, roughly 80 to 90% of cases are idiopathic. It is assumed that these cases are viral. The remaining 10 to 20% of cases are most commonly associated with post–cardiac injury syndromes, connective-tissue diseases or cancer.
    The final diagnosis was acute idiopathic pericarditis because of the negativity of all cultural, serological and autoimmune exams. Moreover, the diagnosis of recurrent pericarditis was excluded because it requires the documentation of a first episode of acute pericarditis in the patient’s medical history, which actually not applies in this patient. Finally, constrictive pericarditis was excluded too because it is a chronic process that can result from a stiff pericardium that prevents satisfactory diastolic filling and these features were not present.
  2. Multimodality imaging is an integral part of modern management for pericardial diseases. Transthoracic Echocardiography (TTE) is most often the first-line test, followed by cardiac magnetic resonance imaging (MRI) or computed tomography (CT). TTE is recommended as an initial noninvasive imaging test for all patients with acute suspected pericarditis because of its ease of use, wide and bedside availability, cost-effectiveness, and comprehensive assessment of both anatomy and physiology. Cardiac MRI and CT can be helpful, because pericardial thickening, enhanced pericardial gadolinium uptake on MRI or both support the diagnosis, especially in limited clinical scenarios with complex clinical presentation or inconclusive echocardiographic findings.
    Our patient underwent cardiac MRI in order to exclude the evolution towards a constrictive form and to assess the degree of increased pericardial thickness and functional effects of the pericarditis. Black blood T1-weighted spin echo cardiac MRI was used for morphological assessment of the pericardium, the intrathoracic and mediastinal structures. Increased signal intensity of the pericardium on T2W-STIR images confirmed active inflammation. Late gadolinium enhancement of the pericardium is coherent with the acute inflammatory state. Real time cine images during free-breathing demonstrated the presence of a modest septal bounce, indicating accentuation of interventricular interdependence. This finding is related to reduced distensibility of the inflamed and thickened pericardium and it regresses with the resolution of the inflammatory process. Rare cases of rapid progression to constrictive pericarditis requiring surgical intervention have been described.
    Pericardial adhesion between inflamed visceral and parietal pericardial surfaces was assessed using dynamic tagging. The normal discontinuity of the tagging stripes with the slip of the inner and outer layers is absent in case of adhesion between the layers and the tag lines remain continuous across the pericardium.
    Cardiac MRI at 6 months showed that the signs of active pericardial inflammation had significantly reduced and there were no signs of pericardial constriction. Late gadolinium enhancement of the pericardium persisted. Late gadolinium enhancement of the pericardium in the absence of active inflammation indicates previous injury of the pericardium and/or a chronic fibrotic pericarditis, characterized by avascular pericardial layers with an abundance of collagen fibers and fibroblasts in the absence of vascularized granulation tissue.
  3. Based on recent landmark trials, if no intolerances or contraindications are present, the initial treatment of acute idiopathic pericarditis should consist of NSAID/ASA in combination with colchicine. The choice of NSAID-type or ASA therapy should be guided by patient-specific factors such as cost, dosage form, route of administration, tolerability and comorbidity (NSAIDs such as indomethacin or ibuprofen should be avoided in patients with ischemic heart disease due to the potential for coronary vasoconstriction and scar formation interference after a coronary event). An attack dose of NSAIDs or ASA therapy should always be used until symptom resolution and normalization of hs-CRP (usually in 1 wk) and should be tapered over 3–4 weeks. Our patient was allergic to acetylsalicylic acid, so oral ibuprofen and colchicines were used. Colchicine therapy can be continued for 6–12 months based on the clinician’s discretion and patient’s comorbidities.
    Early guidelines and published reviews recommend limiting use of corticosteroids to those patients with contraindications or intolerances to NSAID/ASA or colchicines. However these drugs are still commonly used, especially for recurrent pericarditis because of rapid symptom control and initial remission of symptoms. Tapering of the dose of steroidis necessary in order to avoid systemic adverse effects.

References


Case references:

  1. Sagristà-Sauleda J, Angel J, Permanyer-Miralda G, Soler-Soler J. Long-term follow-up of idiopathic chronic pericardial effusion. N Engl J Med. 1999;341(27):2054-9.
  2. Imazio M, Mayosi BM, Brucato A, Markel G, Trinchero R, Spodick DH, Adler Y. Triage and management of pericardial effusion. J Cardiovasc Med (Hagerstown).2010;11(12):928-35.
  3. Imazio M, Adler Y. Management of pericardial effusion. Eur Heart J. 2013;34(16):1186-97.
    Adler Y, Guindo J, Finkelstein Y, Khouri A, Assali A, Bayes-Genis A, Bayes de  Luna A. Colchicine for large pericardial effusion. Clin Cardiol. 1998;21(2):143-4.
  4. Imazio M, Adler Y. Pharmacological therapy of pericardial diseases. Curr Pharm Des. 2015;21(4):525-30.

Solution of the previous case references:

  1. Task Force members, Bernhard Maisch, Chairperson* (Germany), Petar M. Seferovi_c (Serbia and Montenegro), Arsen D. Risti_c (Serbia and Montenegro), Raimund Erbel (Germany), Reiner Rienmuller (Austria), Yehuda Adler (Israel), Witold Z. Tomkowski (Poland), Gaetano Thiene (Italy), Magdi H. Yacoub (UK). Guidelines on the Diagnosis and Management of Pericardial Diseases. Executive Summary. The Task Force on the Diagnosis and Management of Pericardial Diseases of the European Society of Cardiology. European Heart Journal 2004; 25,587–610.
  2. Allan L. Klein, Suhny Abbara, Deborah A. Agler, Christopher P. Appleton, Craig R. Asher, Brian Hoit, Judy Hung, Mario J. Garcia, Itzhak Kronzon, Jae K Oh, E. Rene Rodriguez, Hartzell V. Schaff, Paul Schoenhagen, Carmela D. Tan, and Richard D. White, Cleveland and Columbus, Ohio.
  3. 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.
  4. Bernard Cosyns, Sven Plein, Petros Nihoyanopoulous, Otto Smiseth, Stephan Achenback, Maria Joao Andrade, Mauro Pepi, Arsen Ristic, Massimo Imazio, Bernard Paelinck, Patrizio Lancellotti. European association of cardiovascular imaging (EACVI) position paper: multimodality imaging in pericardial disease. European heart journal – cardiovascular imaging 2015; 16:12-31.
  5. Nicholas C Schwier, James C. Coons, Shivdev K. Rao. Pharmacotherapy Update of Acute Idiopathic Pericarditis. Pharmacotherapy 2015;35:99–111.
  6. Brucato A, Brambilla G, Moreo A, et al. Long-term outcomes in difficult-to treat patients with recurrent pericarditis. Am J Cardiol 2006;98:267-271
  7. Imazio M, Brucato A, Adler Y, et al. Prognosis of idiopathic recurrent pericarditis as determined from previously published reports. Am J Cardiol 2007;100:1026-28
  8. Francone M, Dymarkoswski S, Kalantzi M. et al. Assessment of ventricular coupling with real time cine MRI and its value to differentiate constrictive pericarditis from restrictive cardiomyopathy. Eur J Radiol 2006;16:944-51
  9. Haley JH, Tajik AJ, Danielson GK, et al. Transient constrictive pericarditis: causes and natuaral history. J Am Coll Cardiol 2004;43:271-75

The content of this article reflects the personal opinion of the author/s and is not necessarily the official position of the European Society of Cardiology.