C A S E R E P O R T

Effusive Constrictive Pericarditis

E ffusive constrictive pericarditis(ECP) is a relatively rare pericardial

condition. It is considered somewhat ofa middle stage between acute pericardi-tis and chronic constriction.1 Visceraland parietal pericardial stripping is anextensive procedure and is generallyconsidered the only effective treatment.Recent studies suggest up to 10% ofconstriction may be reversible and theremay be a specific role for medically man-aging these patients.2 Extrapolating fromthe response of acute pericarditis to col-chicine, we propose that colchicine maybe another option in management ofECP, if initiated early in the course ofthe disease. Here we report 2 patientswith varied effusion-constriction presen-tation and review the current under-standing of the reversible pericardialpathology and suggest a unified manage-ment approach with medical therapy.

Case 1A 66-year-old white man presented withrapidly worsening shortness of breathand band-like chest tightness of 5 days’duration. He had diabetes, hypertension,and stage 3 non–small cell lung cancertreated with radiation and chemother-apy 3 years ago. The patient had tachy-cardia with a heart rate of 110 beats perminute and systolic blood pressure of100 mm Hg; 2-dimensional echocardio-graphic examination demonstrated amoderate pericardial effusion. Mitralinflow velocities suggested ventricularinterdependence, but chamber collapsewas not evident (Figure 1). Right heartcatheterization performed 24 hours afterechocardiography revealed low cardiacoutput of 4.0 L ⁄min, pulmonary arterypressure (PAP) of 34 ⁄11 ⁄22 mm Hg,right atrial pressure (RAP) of 18 mmHg, right ventricular diastolic pressure(RVDP) of 18 mm Hg, and pulmonarywedge pressure (PCWP) of 20 mm Hg,

with left ventricular end-diastolic pres-sure (LVEDP) of 18 mm Hg suggestingconstrictive physiology. Indomethacinwas started along with a loading dose of1.2 mg of colchicine that was main-tained at 0.6 mg daily. Chest discomfortwas relieved in 1 to 2 days, with resolu-tion of tachycardia and hypotension bythe third day. With continued medicalmanagement, repeat echocardiography2 weeks later revealed resolution ofpericardial effusion and mitral inflowrespiratory variation. At 3 months, thepatient remains asymptomatic withbaseline functionality.

Case 2A 39-year-old woman presented withHenoch-Schonlein purpura–related re-nal failure and a prior failed renal trans-plant who was back on hemodialysis forthe past year. She presented to us withincreasing shortness of breath of2 weeks’ duration. Echocardiography

revealed a small circumferential pericar-dial effusion and pericardial thickeningof 1.5 cm noted on computed tomogra-phy (CT) of the chest. After 48 hours,the patient underwent right and leftheart catheterization, which revealedelevated filling pressures with equaliza-tion of diastolic pressures (RAP, 28 mmHg; RVDP, 27 mm Hg; LVEDP,30 mm Hg; PCWP, 26 mm Hg; PAP,43 ⁄27 mm Hg) and dip and plateau inright and left ventricular diastolic pres-sures suggestive of constrictive physio-logy (Figure 2). She was managedconservatively with prednisone, withmarked improvement in her symptoms.Two blinded readers independently cor-roborated significant reduction in peri-cardial thickening on follow-up chestCT study to 0.8 cm within a few weeks.The patient was seen in an out-patientclinic 2 months later with improvementin her symptoms, and repeat echocardio-graphy showed no pericardial effusion.

Effusive constrictive pericarditis (ECP) is a relatively infrequent pericardial condition. Thediagnosis is typically made when symptoms and right heart pressure elevation persistdespite drainage of pericardial effusion. Visceral and parietal pericardial stripping is anextensive procedure with significant morbidity and mortality but is widely considered theonly effective treatment. Recent studies suggest that up to 10% of constriction may bereversible, and a newer series has reported spontaneous complete resolution of symptomsin a subset of ECP patients. In this review, the authors describe 2 patients with ECP whowere managed successfully with steroids and colchicine, respectively, thus obviating theneed for surgery. The authors also review the current understanding of this reversiblepericardial pathology and explore the possible role for colchicine in treating thiscondition. Congest Heart Fail. 2009;15:199–201. �2009 Wiley Periodicals, Inc.

Rajeev Garg, MD;1 Avneet Singh, MBBS, MRCP;2 Anand Chockalingam, MD1

From the Department of Internal Medicine, Division of Cardiology, University ofMissouri-Columbia, Columbia, MO;1 and the Department of Internal Medicine,Long Island Jewish Medical Center, New Hyde Park, NY2

Address for correspondence:Avneet Singh, MBBS, MRCP, Department of Internal Medicine, Long IslandJewish Medical Center, 270-05 76th Avenue, New Hyde Park, NY 11040E-mail: avneetkathpal@gmail.comManuscript received July 16, 2008; revised November 4, 2008;accepted November 6, 2008

doi: 10.1111/j.1751-7133.2008.00032.x

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DiscussionIncidence and Natural History. In thelargest series to date reported bySagrista-Sauleda and colleagues,3 about1.3% of patients with any pericardialdisease and up to 7.9% of those withcatheterized tamponade showed evi-dence of ECP. This diagnosis can be eas-ily overlooked if simultaneous rightheart catheterization is not routinely per-formed at the time of pericardiocentesis.

Diagnosis. The reason both our casesare ECP is that both had evidence ofpericardial effusion and thickening onechocardiographic and CT scan with

simultaneous demonstration of constric-tive physiology on right heart catheteri-zation. However, since pericardiocentesiswas never performed in either case, theclassical ECP finding—failure of normal-ization of right heart pressures withremoval of pericardial fluid—could notbe demonstrated.

This also highlights that in theabsence of simultaneous right heartcatheterization with pericardiocentesis,combined data obtained from multiplemodalities may help point to this diag-nosis—pericardial thickening, effusion,ventricular septal bounce on echocar-diography and CT, and constrictivephysiology on right heart catheteriza-

tion, namely high right atrial pressureswith prominent x and y descents,respiratory discordance in peak rightand left ventricular systolic pressures,and square-root sign on ventriculardiastolic tracings.

Management. Hancock’s1 report in1971 reported only transient benefitwith anti-inflammatory agents anddiuretics. Surgery provided definite andsustained relief in 10 of 13 patients, with2 deaths (15% mortality) perioperative-ly. More recent, Sagrista-Sauleda andcolleagues3 documented complete spon-taneous resolution of ECP in 3 of 15patients. All 3 had idiopathic ECP.Reviewing their 10-year experience withconstrictive pericarditis, a study con-ducted at the Mayo Clinic reportedspontaneous resolution of constrictionin 36 (15%) of 212 patients.2 About56% were treated with nonsteroidalanti-inflammatory drugs (NSAIDs),44% with steroids, and about 11% withantibiotics. Importantly, except withpost-radiation constriction, all otherscould resolve spontaneously, and meantime to improvement was 8.3 weeks inthis study.

NSAIDs may reduce the inflamma-tion sufficiently in some patients withearly mild ECP to effect spontaneousresolution. Colchicine is effective intreating and preventing recurrent peri-carditis.4 One randomized trial demon-strated significant reduction in therecurrence rate (from 32.3% to 10.7%)using colchicine for 3 months followinga first episode of acute pericarditis.5

Colchicine has been reported to beuseful in managing recurring pericardi-tis-related large pericardial effusions.6

There are no studies using colchicine inECP. We propose that patients with sig-nificant pericardial effusions and thosesuspected of having pericardial inflam-mation be treated with colchicine toreduce recurrence of effusions andimprove symptoms in ECP. Althoughthis needs to be proven in future clinicaltrials, it may be difficult given the lowincidence of the disease. The clinicalsuccess with the NSAID-colchicinecombination in our first patient suggestsa small but definite role for aggressive

Figure 1. Mitral valve Doppler showing 28% respiratory variation in the left ventricularinflow velocities suggestive of ventricular interdependence.

Figure 2. Simultaneous left ventricular (LV) and right ventricular (RV) diastolic tracingshowing the classic square-root sign, equalization of RV and LV end-diastolic pressure, aswell as respiratory variation in RV and LV systolic pressures.

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early medical management in ECP andthe possibility of averting downstreamsurgical stripping. Our second patientwas undergoing hemodialysis, whichis considered a contraindication forcolchicine, and was therefore treatedwith prednisone.

Our report of only 2 patients is a limi-tation of our study. Echocardiographicresolution of effusion and clinical

improvement of symptoms suggestthat absence of constrictive physiol-ogy by a repeat catheterization wouldhave helped confirm right heartpressure reduction.

ConclusionsECP has to be entertained as a possibil-ity in all patients presenting with peri-

carditis or pericardial effusions.Diagnosis can be suspected from echo-cardiography and CT imaging but cath-eterization is definitive. There is a smallbut definite subset that resolves sponta-neously and, thus, an initial trial ofaspirin and colchicine appears reason-able. When symptoms are severe or per-sistent for months, pericardiectomy maybe needed.

REFERENCES

1 Hancock EW. Subacute effusive-constrictivepericarditis. Circulation. 1971;43:183–192.

2 Haley JH, Tajik AJ, Danielson GK, et al.Transient constrictive pericarditis: causes andnatural history. J Am Coll Cardiol. 2004;43:271–275.

3 Sagrista-Sauleda J, Angel J, Sanchez A, et al.Effusive–constrictive pericarditis. N Engl J Med.2004; 350:469–475.

4 Guindo J, Rodriguez de la Serna A,Ramio J, et al. Recurrent pericarditis: reliefwith colchicine. Circulation. 1990;82:1117–1120.

5 Imazio M, Bobbio M, Cecchi E, et al.Colchicine in addition to conventional therapyfor acute pericarditis: results of the COlchicinefor acute PEricarditis (COPE) Trial. Circulation.2005;112:2012–2016.

6 Adler Y, Guindo J, Finkelstein Y, et al. Colchi-cine for large pericardial effusion. Clin Car-diol. 1998;21(2):143–144.

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