new case report chronic constrictive...

Hindawi Publishing CorporationCase Reports in CardiologyVolume 2013 Article ID 957497 4 pageshttpdxdoiorg1011552013957497

Case ReportChronic Constrictive Pericarditis

Hossein Doustkami1 Afshin Hooshyar2 Nasrollah Maleki2 Zahra Tavosi3 and Iraj Feizi4

1 Interventional Cardiology Department of Cardiology Imam Khomeini Hospital Ardabil University of Medical Sciences Iran2Department of Internal Medicine Imam Khomeini Hospital Ardabil University of Medical Sciences Ardabil Iran3Department of Internal Medicine Shohadaye Khalije Fars Hospital Bushehr University of Medical Sciences Bushehr Iran4Department of Cardiothoracic Surgery Imam Khomeini Hospital Ardabil University of Medical Sciences Ardabil Iran

Correspondence should be addressed to Nasrollah Maleki malekinasrollahyahoocom

Received 4 August 2013 Accepted 25 August 2013

Academic Editors K-R Chiou M-H Jim and M B Srichai

Copyright copy 2013 Hossein Doustkami et al This is an open access article distributed under the Creative Commons AttributionLicense which permits unrestricted use distribution and reproduction in any medium provided the original work is properlycited

Constrictive pericarditis (CP) is a rare clinical entity that can pose diagnostic problemsThe diagnosis of CP requires a high degreeof clinical suspicion The gold standard for diagnosis is cardiac catheterization with analysis of intracavitary pressure curveswhich are high and in end diastole equal in all chambers We present a patient with unexplained dyspnea recurrent right-sidepleural effusion and ascites Analysis of the ascitic fluid revealed a high protein content and an elevated serum-ascites gradientEchocardiography computed tomography and cardiac catheterization revealed the diagnosis of CP He underwent completepericardiectomy and to date has made a good recoveryThe diagnosis of CP is often neglected by admitting physicians who usuallyattribute the symptoms to another disease process This case exemplifies the difficulty in diagnosing this condition as well as theinvestigation required and provides a discussion of the benefit and outcomes of prompt treatment

1 Introduction

Constrictive pericarditis (CP) is a disease characterized by theencasement of the heart by a rigid nonpliable pericardiumdue to dense fibrosis and adhesions This causes impaireddiastolic cardiac function [1] Patients with pericardial con-striction may present with two types of complaints thoserelated to fluid overload ranging from peripheral edema toanasarca and those related to diminished cardiac outputresponse to exertion such as fatigability and dyspnea onexertion Pericardial constriction should be considered inany patient with an unexplained elevation in jugular venouspressure particularly if there is a history of a predisposingcondition [2] The common cause of this disease is idio-pathic or viral pericarditis Other causes include tuberculosistrauma cardiac surgery irradiationwithmediastinum septicinfections histoplasmosis systemic lupus erythematosusrheumatoid arthritis malignancies and chronic kidney dis-ease along with chronic dialysis [2ndash5] Pericardial diseaserarely presents as the initial manifestation tuberculosis [6ndash9] Cardiac CT and MRI can detect pericardial thickeningand calcification with high accuracy [10] Echocardiography

is very useful for differential diagnosis between CP andrestrictive cardiomyopathy [11 12] The gold standard fordiagnosis is cardiac catheterization Pericardiectomy is theonly definitive treatment of CP and should be as complete aspossible [4 13 14]

2 Case Report

Thepatient is a 52-year-old man who gradually suffered sinceabout 5 years from exertional dyspnea weakness and lackof energy fatigue feeling pleuritic chest pain distension ofabdomen and peripheral edema Patient has a past history ofhospitalization one year ago due to chest pain and receivedcoronary angiography and it was normal The patient alsoreceived diagnostic thoracentesis 6 months before due todyspnea and the presence of right-side pleural effusionand he had exudative pleural effusion with lymphocyte-dominant and nondiagnostic cytology and for this reasonhe received thoracoscopy and pleural biopsy which werenondiagnostic The patient referred to our hospital due topain and progressive abdominal distention in the past 10 dayswas hospitalized On physical examination the patient was

2 Case Reports in Cardiology

hemodynamically stable (blood pressure was 11080mmHgand pulse was 78 beats per minute) JVP was very elevatedHeart sounds were muffle and reduction of sound was foundat the base of the right lung In the examination mildhepatomegaly with ascites and peripheral edema was seenPrimary laboratory evaluations were normal Analysis of theascitic fluid revealed a high protein content (41 gdL) andan elevated serum-ascites gradient (16 gdL) In abdominalsonography congestive hepatomegaly mild splenomegalyascites and evidence of portal hypertension were seen Inupper endoscopy esophageal varices were not seen and viralhepatitis serology was negative In chest and abdominal CTright pleural effusion pericardial thickness and calcificationascites and inferior vena cava dilation were seen (Figure 1)To study abdominal vascular thrombosis MRV (MagneticResonance Venography) was performed and the results werenormal In the conducted echocardiography enlargementof right atrium (44mm) right ventricle (46mm) and leftatrium (42mm) along with mild pericardial effusion peri-cardial calcification inferior vena cava dilation (28mm) andseptal bouncing was found (Figure 2)

Right and left cardiac catheterization were performed forthe patient in which elevation and equalization of right atrialpressure (29mmHg) pulmonary capillary wedge pressure(30mmHg) mean pulmonary arterial pressure (33mmHg)right ventricular diastolic pressure (30mmHg) and leftventricular diastolic pressure (30mmHg) were found Curvesrecorded in right heart catheterization indicate increase ofsuperior vena cava pressure at time of breath and descendingand then horizontal curve of right ventricular pressure(square root sign) was evident (Figure 3) Coronary angiog-raphy was normal All of the findings were consistent withCP

The patient underwent cardiac surgery during whichpericardium was fully thick and calcified (Figure 4(a)) andreceived pericardiectomy Pathological study of pericardialsample indicated fibrous pericarditis without granuloma(Figure 4(b)) Gramrsquos staining staining for acid-fast baciliiand culture of the pericardium for bacteria fungus and acid-fast bacilli were negative

One year after the operation the patient reported adramatic improvement in his exertion tolerance along withdecrease of dyspnea and distension of abdomen

3 Discussion and Conclusion

This case illustrates an unusual cause of ascites The mostcommon cause of ascites in the United States is cirrhosisfollowed distantly by cancer right-sided heart failure tuber-culosis pancreatic disease and various rare infection andhematologic diseases [15] A serum-ascites albumin gradientge11 gdL and an ascites fluid total protein gt25 gdL aretypical of CP and other postsinusoidal causes of ascitesSinusoidal diseases such as liver cirrhosis exhibit a serum-ascites albumin gradient gt11 gdL but an ascites fluid totalprotein lt25 gdL [16] When ascites is present estimation ofthe jugular venous pressure is critical since it can frequentlyseparate cardiac from noncardiac causes Elevated jugular

venous pressure can be challenging to detect even when theassessment is made by experienced clinicians The overallcorrelation between clinical assessment of the jugular venouspressure direct measurement of central venous pressure bycentral venous catheterization is poor an overall accuracyof 56 has been reported in classifying the central venouspressure as low normal or high with a sensitivity fordetection of a high central venous pressure (gt10 cm ofwater) of less than 60 [17ndash19] In this case the failure torecognize the elevated jugular venous pressure led to a delayin diagnosis and extensive diagnostic testing Symptoms ofCP are typically related to systemic venous congestion andlow cardiac outputWhereas elevated jugular venous pressurewas present in nearly all patients with CP in a large caseseries peripheral edema was absent in approximately 25of patients particularly early in the disease process and lessthan 6of patients presentedwith predominantly abdominalsymptoms [2]Therefore a high index of suspicion is requiredto diagnose this entity especially in patients with elevatedprotein-count ascites jugular venous distention and nocardiopulmonary symptoms Pleural effusion occurs in 44ndash50 of patients with CP [4 20] Tomaselli and coworkersretrospectively analyzed 30 patients who presented with CPand found that 60 (18 patients) had pleural effusion [21]Bilateral and symmetrical effusions were found in 12 patientsand the remaining 6 had unilateral pleural fluid (3 had right-side effusion and 3 had left-side effusion) Our patient hadleft side pleural effusion Pericardial thickening detected onCT or MRI is absent in up to 28 of patients with surgicallyproven CP [13] Our patient had right side heart failure anda typical cardiac CT calcification Typical echocardiographicfindings such as normal systolic function a plethoric inferiorvena cava a restrictive mitral inflow pattern with respiratoryvariation reversal of expiratory hepatic vein flow a septalmotion suggestive of enhanced ventricular interaction or anelevated early diastolic mitral annular velocity (1198641015840) detectedby tissue Doppler imaging may not be observed if images arepoor or if CP is not explicitly noted as a potential diagnosis[22 23] Elevated and equalized diastolic pressures on cardiaccatheterization are the rule for CP Ventricular filling is rapidearly and blunted late by the stiffened pericardial sac leadingto the characteristic steep y descent of right atrial pressureand the dip and plateau of ventricular pressure [24 25]Although these hemodynamic patterns can be observed inother causes of heart failure such as restrictive cardiomyopa-thy discordance between changes in right and left ventricularsystolic pressures during respiration known as ventricularinterdependence reliably distinguishes CP from these otherconditions [13 26] Most patients with CP required surgicalpericardiectomy Removal of densely adherent pericardiumis usually successful but can be extremely challenging [2]Moreover recovery can be delayed for several weeks andpatients in whom the constriction has progressed to the pointof abnormal ventricular function severely reduced cardiacoutput cachexia or end-organ dysfunction derive the leastbenefit from the procedure [4 27] an observation that under-scores the importance of prompt diagnosis and treatment

The diagnosis of CP in our patient was probably delayedfrom two reasons the rarity of the diagnosis and the failure

Case Reports in Cardiology 3

(a) (b) (c)

Figure 1 Chest CT showing pleural effusion cardiac calcification ascites and IVC dilation

(a)

RAIVC

(b) (c)

Figure 2 Echocardiography showing septal bouncing (a) dilation of IVC (b) pericardial effusion and calcification (c)

RV pressure

LV pressure

Figure 3 The equalization of diastolic pressures and ldquosquare root signrdquo or ldquodip and plateau signrdquo of the left ventricular waveforms

The arrows indicate thickened pericardium

(a)

Paucicellular fibrosis

(b)

Figure 4 Surgical and pathological findings


to recognize the elevated jugular venous pressure on initialexamination This case reminds us that reconsideration ofclinical information from a different angel can facilitate thediagnostic process in patients with complex conditions Inconclusion in case there is any calcification in a cardiac CTwith right-sided heart failure symptoms we should considerthe diagnosis of constrictive pericarditis and performingfurther cardiac investigations

References

[1] R B H Myers and D H Spodick ldquoConstrictive pericarditisclinical and pathophysiologic characteristicsrdquo American HeartJournal vol 138 no 2 pp 219ndash232 1999

[2] L H Ling J K Oh H V Schaff et al ldquoConstrictive pericarditisin the modern era evolving clinical spectrum and impact onoutcome after pericardiectomyrdquo Circulation vol 100 no 13 pp1380ndash1386 1999

[3] J Cameron S N Oesterle J C Baldwin and E W HancockldquoThe etiologic spectrum of constrictive pericarditisrdquo AmericanHeart Journal vol 113 no 2 pp 354ndash360 1987

[4] S C Bertog S K Thambidorai K Parakh et al ldquoConstrictivepericarditis etiology and cause-specific survival after peri-cardiectomyrdquo Journal of the American College of Cardiology vol43 no 8 pp 1445ndash1452 2004

[5] P P Sengupta M F Eleid and B K Khandheria ldquoConstrictivepericarditisrdquo Circulation Journal vol 72 no 10 pp 1555ndash15622008

[6] V V Bashi S John E Ravikumar P S Jairaj K Shyamsunderand S Krishnaswami ldquoEarly and late results of pericardiectomyin 118 cases of constrictuve pericarditisrdquo Thorax vol 43 no 8pp 637ndash641 1988

[7] M Pedreira Perez A Virgos Lamela F J CrespoMancebo J LCervantes G Fernandez de la Reguera and R BarraganGarcıaldquo40 yearsrsquo experience in the surgical treatment of constrictivepericarditisrdquoArchivos del Instituto de Cardiologia deMexico vol57 no 5 pp 363ndash373 1987

[8] H Raffa and J Mosieri ldquoConstrictive pericarditis in SaudiArabiardquo East AfricanMedical Journal vol 67 no 9 pp 609ndash6131990

[9] S Arsan S Mercan A Sarigul et al ldquoLong-term experiencewith pericardiectomy analysis of 105 consecutive patientsrdquoThoracic and Cardiovascular Surgeon vol 42 no 6 pp 340ndash344 1994

[10] R Rienmuller M Gurgan E Erdmann B M Kemkes EKreutzer and C Weinhold ldquoCT and MR evaluation of peri-cardial constriction a new diagnostic and therapeutic conceptrdquoJournal of Thoracic Imaging vol 8 no 2 pp 108ndash121 1993

[11] B Maisch P M Seferovic A D Ristic et al ldquoGuidelines on thediagnosis and management of pericardial diseases executivesummary The Task Force on the Diagnosis and Managementof Pericardial Diseases of the European Society of CardiologyrdquoEuropean Heart Journal vol 25 no 7 pp 587ndash610 2004

[12] J-W Ha S R Ommen A J Tajik et al ldquoDifferentiation ofconstrictive pericarditis from restrictive cardiomyopathy usingmitral annular velocity by tissue Doppler echocardiographyrdquoAmerican Journal of Cardiology vol 94 no 3 pp 316ndash319 2004

[13] D R Talreja W D Edwards G K Danielson et al ldquoCon-strictive pericarditis in 26 patients with histologically normalpericardial thicknessrdquoCirculation vol 108 no 15 pp 1852ndash18572003

[14] U K Chowdhury G K Subramaniam A S Kumar et al ldquoPeri-cardiectomy for constrictive pericarditis a clinical echocar-diographic and hemodynamic evaluation of two surgical tech-niquesrdquo Annals of Thoracic Surgery vol 81 no 2 pp 522ndash5292006

[15] B A Runyon ldquoCurrent concepts care of patients with ascitesrdquoThe New England Journal of Medicine vol 330 no 5 pp 337ndash342 1994

[16] J P Howard D Jones P Mills R Marley and A WraggldquoRecurrent ascites due to constrictive pericarditisrdquo FrontlineGastroenterology vol 3 no 4 pp 233ndash237 2012

[17] D J Cook ldquoClinical assessment of central venous pressure inthe critically illrdquo American Journal of the Medical Sciences vol299 no 3 pp 175ndash178 1990

[18] D J Cook and D L Simel ldquoDoes this patient have abnormalcentral venous pressurerdquo Journal of the American MedicalAssociation vol 275 no 8 pp 630ndash634 1996

[19] A F Connors Jr D R McCaffree and B A Gray ldquoEvaluationof right-heart catheterization in the critically ill patient withoutacute myocardial infarctionrdquo The New England Journal ofMedicine vol 308 no 5 pp 263ndash267 1983

[20] A R Wychulis D C Connolly and D C McGoon ldquoSurgicaltreatment of pericarditisrdquo Journal of Thoracic and Cardiovascu-lar Surgery vol 62 no 4 pp 608ndash617 1971

[21] G Tomaselli G Gamsu and M S Stulbarg ldquoConstrictivepericarditis presenting as pleural effusion of unknown originrdquoArchives of Internal Medicine vol 149 no 1 pp 201ndash203 1989

[22] J K Oh L K Hatle J B Seward et al ldquoDiagnostic role ofDoppler echocardiography in constrictive pericarditisrdquo Journalof the American College of Cardiology vol 23 no 1 pp 154ndash1621994

[23] RW Troughton C R Asher andA L Klein ldquoPericarditisrdquoTheLancet vol 363 no 9410 pp 717ndash727 2004

[24] A T Hansen P Eskildsen and H Gotzsche ldquoPressure curvesfrom the right auricle and the right ventricle in chronicconstrictive pericarditisrdquo Circulation vol 3 no 6 pp 881ndash8881951

[25] EMeaney R Shabetai V Bhargava et al ldquoCardiac amyloidosisconstrictive pericarditis and restrictive cardiomyopathyrdquoAmer-ican Journal of Cardiology vol 38 no 5 pp 547ndash556 1976

[26] D G Hurrell R A Nishimura S T Higano et al ldquoValue ofdynamic respiratory changes in left and right ventricular pres-sures for the diagnosis of constrictive pericarditisrdquo Circulationvol 93 no 11 pp 2007ndash2013 1996

[27] J-W Ha J K Oh H V Schaff et al ldquoImpact of left ven-tricular function on immediate and long-term outcomes afterpericardiectomy in constrictive pericarditisrdquo Journal ofThoracicand Cardiovascular Surgery vol 136 no 5 pp 1136ndash1141 2008

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Volume 2014Hindawi Publishing Corporationhttpwwwhindawicom


hemodynamically stable (blood pressure was 11080mmHgand pulse was 78 beats per minute) JVP was very elevatedHeart sounds were muffle and reduction of sound was foundat the base of the right lung In the examination mildhepatomegaly with ascites and peripheral edema was seenPrimary laboratory evaluations were normal Analysis of theascitic fluid revealed a high protein content (41 gdL) andan elevated serum-ascites gradient (16 gdL) In abdominalsonography congestive hepatomegaly mild splenomegalyascites and evidence of portal hypertension were seen Inupper endoscopy esophageal varices were not seen and viralhepatitis serology was negative In chest and abdominal CTright pleural effusion pericardial thickness and calcificationascites and inferior vena cava dilation were seen (Figure 1)To study abdominal vascular thrombosis MRV (MagneticResonance Venography) was performed and the results werenormal In the conducted echocardiography enlargementof right atrium (44mm) right ventricle (46mm) and leftatrium (42mm) along with mild pericardial effusion peri-cardial calcification inferior vena cava dilation (28mm) andseptal bouncing was found (Figure 2)

Right and left cardiac catheterization were performed forthe patient in which elevation and equalization of right atrialpressure (29mmHg) pulmonary capillary wedge pressure(30mmHg) mean pulmonary arterial pressure (33mmHg)right ventricular diastolic pressure (30mmHg) and leftventricular diastolic pressure (30mmHg) were found Curvesrecorded in right heart catheterization indicate increase ofsuperior vena cava pressure at time of breath and descendingand then horizontal curve of right ventricular pressure(square root sign) was evident (Figure 3) Coronary angiog-raphy was normal All of the findings were consistent withCP

The patient underwent cardiac surgery during whichpericardium was fully thick and calcified (Figure 4(a)) andreceived pericardiectomy Pathological study of pericardialsample indicated fibrous pericarditis without granuloma(Figure 4(b)) Gramrsquos staining staining for acid-fast baciliiand culture of the pericardium for bacteria fungus and acid-fast bacilli were negative

One year after the operation the patient reported adramatic improvement in his exertion tolerance along withdecrease of dyspnea and distension of abdomen

3 Discussion and Conclusion

This case illustrates an unusual cause of ascites The mostcommon cause of ascites in the United States is cirrhosisfollowed distantly by cancer right-sided heart failure tuber-culosis pancreatic disease and various rare infection andhematologic diseases [15] A serum-ascites albumin gradientge11 gdL and an ascites fluid total protein gt25 gdL aretypical of CP and other postsinusoidal causes of ascitesSinusoidal diseases such as liver cirrhosis exhibit a serum-ascites albumin gradient gt11 gdL but an ascites fluid totalprotein lt25 gdL [16] When ascites is present estimation ofthe jugular venous pressure is critical since it can frequentlyseparate cardiac from noncardiac causes Elevated jugular

venous pressure can be challenging to detect even when theassessment is made by experienced clinicians The overallcorrelation between clinical assessment of the jugular venouspressure direct measurement of central venous pressure bycentral venous catheterization is poor an overall accuracyof 56 has been reported in classifying the central venouspressure as low normal or high with a sensitivity fordetection of a high central venous pressure (gt10 cm ofwater) of less than 60 [17ndash19] In this case the failure torecognize the elevated jugular venous pressure led to a delayin diagnosis and extensive diagnostic testing Symptoms ofCP are typically related to systemic venous congestion andlow cardiac outputWhereas elevated jugular venous pressurewas present in nearly all patients with CP in a large caseseries peripheral edema was absent in approximately 25of patients particularly early in the disease process and lessthan 6of patients presentedwith predominantly abdominalsymptoms [2]Therefore a high index of suspicion is requiredto diagnose this entity especially in patients with elevatedprotein-count ascites jugular venous distention and nocardiopulmonary symptoms Pleural effusion occurs in 44ndash50 of patients with CP [4 20] Tomaselli and coworkersretrospectively analyzed 30 patients who presented with CPand found that 60 (18 patients) had pleural effusion [21]Bilateral and symmetrical effusions were found in 12 patientsand the remaining 6 had unilateral pleural fluid (3 had right-side effusion and 3 had left-side effusion) Our patient hadleft side pleural effusion Pericardial thickening detected onCT or MRI is absent in up to 28 of patients with surgicallyproven CP [13] Our patient had right side heart failure anda typical cardiac CT calcification Typical echocardiographicfindings such as normal systolic function a plethoric inferiorvena cava a restrictive mitral inflow pattern with respiratoryvariation reversal of expiratory hepatic vein flow a septalmotion suggestive of enhanced ventricular interaction or anelevated early diastolic mitral annular velocity (1198641015840) detectedby tissue Doppler imaging may not be observed if images arepoor or if CP is not explicitly noted as a potential diagnosis[22 23] Elevated and equalized diastolic pressures on cardiaccatheterization are the rule for CP Ventricular filling is rapidearly and blunted late by the stiffened pericardial sac leadingto the characteristic steep y descent of right atrial pressureand the dip and plateau of ventricular pressure [24 25]Although these hemodynamic patterns can be observed inother causes of heart failure such as restrictive cardiomyopa-thy discordance between changes in right and left ventricularsystolic pressures during respiration known as ventricularinterdependence reliably distinguishes CP from these otherconditions [13 26] Most patients with CP required surgicalpericardiectomy Removal of densely adherent pericardiumis usually successful but can be extremely challenging [2]Moreover recovery can be delayed for several weeks andpatients in whom the constriction has progressed to the pointof abnormal ventricular function severely reduced cardiacoutput cachexia or end-organ dysfunction derive the leastbenefit from the procedure [4 27] an observation that under-scores the importance of prompt diagnosis and treatment

The diagnosis of CP in our patient was probably delayedfrom two reasons the rarity of the diagnosis and the failure


(a) (b) (c)


(a)

RAIVC

(b) (c)


RV pressure

LV pressure



(a)


(b)




References

































of






Disease Markers



OncologyJournal of





PPAR Research



Journal of

ObesityJournal of

















(a) (b) (c)


(a)

RAIVC

(b) (c)


RV pressure

LV pressure



(a)


(b)




References

































of






Disease Markers



OncologyJournal of





PPAR Research



Journal of

ObesityJournal of


















References

































of






Disease Markers



OncologyJournal of





PPAR Research



Journal of

ObesityJournal of





















of






Disease Markers



OncologyJournal of





PPAR Research



Journal of

ObesityJournal of
















new case report chronic constrictive...

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