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CARDIOLOGY BOARD REVIEW MANUAL

Cardiology Volume 9, Part 2 1

PUBLISHING STAFFPRESIDENT, GROUP PUBLISHER

Bruce M. White

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Jean M. Gaul

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NOTE FROM THE PUBLISHER:This publication has been developed with-out involvement of or review by theAmerican Board of Internal Medicine.

Constrictive Pericarditis:A Case StudySeries Editor: A. Maziar Zafari, MD, PhD, FACCAssistant Professor of Medicine, Division of Cardiology, Department of Medicine, Emory University School of Medicine, Atlanta, GA;Director, CCU, Atlanta Veterans Affairs Medical Center, Decatur, GA

Contributors: William J. Nicholson, MDCardiology Fellow, Division of Cardiology, Department of Medicine, Emory University School of Medicine, Atlanta, GA;Cardiology Fellow, Atlanta Veterans Affairs Medical Center, Decatur, GA

Andro G. Kacharava, MD, PhDAssistant Professor of Medicine, Division of Cardiology, Emory University School of Medicine, Atlanta, GA;Staff Cardiologist, Atlanta Veterans Affairs Medical Center, Decatur, GA

Introduction. . . . . . . . . . . . . . . . . . . . . . . . . . . . 2

Clinical and Physical Findings . . . . . . . . . . . . . . 3

Physiology and Pathology. . . . . . . . . . . . . . . . . . 5

Diagnostic Imaging Modalities . . . . . . . . . . . . . . 5

Hemodynamics . . . . . . . . . . . . . . . . . . . . . . . . . 7

Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 7

Summary Points . . . . . . . . . . . . . . . . . . . . . . . . 10

References . . . . . . . . . . . . . . . . . . . . . . . . . . . . 11

Table of Contents

Cover Illustration by Stacey Caiazzo

2 Hospital Physician Board Review Manual

I. INTRODUCTION

Constrictive pericarditis (CP) is a relatively rare postin-flammatory disorder with different causes. The diagnosisof CP and its distinction from restrictive cardiomyopathy(RCM) remain notoriously difficult. Patients with bothconditions present with increased left-sided and right-sided filling pressures, and their symptoms may resemblecongestive heart failure (CHF). It is paramount to diag-nose and distinguish these 2 entities because surgicalpericardiectomy is potentially curative in CP.

Constrictive pericarditis is a condition in which the peri-cardial sac undergoes a process of progressive thicken-ing and fibrosis; the natural elasticity of the pericardialsac is lost, subsequently placing the heart in a non-pliable and rigid encasement, impairing diastolic fillingof the ventricles. Restrictive cardiomyopathy refers to idio-pathic or systemic disorders involving the myocardiumcharacterized by restrictive diastolic filling of the ventri-cles. Many clinical and hemodynamic findings are com-mon to both CP and RCM, making the 2 disorders dif-ficult to distinguish. The purpose of this review is todiscuss the clinical presentation of CP and to focus onthe hemodynamic, echocardiographic, and radiologicfindings differentiating it from RCM. Although the diag-nosis can usually be achieved via these noninvasive andinvasive studies, surgical exploration or biopsy occasion-ally is needed to make definitive diagnosis. A case pa-tient is presented to illustrate the management of CP.

ANATOMICAL CONSIDERATIONS

The pericardium is an elastic, closed fibroserous sacsurrounding the heart. It consists of 2 layers of tissue: anexternal (fibrous) and an internal (serous) layer. Theinternal or serous layer is made up of both visceral andparietal pericardium. The visceral layer is a thin mono-cellular layer adherent to the heart’s epicardium. Theparietal pericardium is adjacent to the visceral peri-cardium on one side and becomes tightly opposed tothe external fibrous layer on the other side. The spacecreated between the visceral and parietal layers of thepericardium contains approximately 30 to 50 mL ofserous fluid, which acts as a lubricant to minimize fric-

tion between the 2 layers of pericardium during theheart’s movement throughout the cardiac cycle.1 Thisfluid-filled space defines the pericardial cavity. Thefibrous pericardium envelops the entire heart and ex-tends over approximately 3 cm of the great vesselswhere it then attaches. Therefore, much of the ascend-ing aorta, the main pulmonary artery, all 4 pulmonaryveins, and portions of the inferior and superior venaecavae are contained within the pericardium.2

Fibrosis and scarring can affect each of the layers ofthe pericardium either separately or simultaneously.Development of adhesions and calcifications betweenthe layers can lead to obliteration of the pericardial cav-ity, creating a rigid inelastic “shell” around the heartwith resultant pathophysiologic consequences (ie, CP).As the pericardial cavity is obliterated in patients withCP, even the physiologic amount of pericardial fluidmay disappear; however, excessive amounts of effusivefluid may be present in some patients. As this fluid issubjected to the constricting effects of the scarred peri-cardium, increased pressure can result in cardiac com-pression or tamponade with resultant hemodynamicdeterioration; this entity is known as effusive CP.3,4 Insuch cases, the constrictive hemodynamics are maskedby tamponade and may only become obvious after thepericardial fluid is drained by pericardiocentesis.

ETIOLOGIES

Many different conditions can produce acute peri-carditis, and nearly all are capable of inducing constric-tion, albeit some more frequently than others (Table 1).5

The notable exception is acute rheumatic fever, whichcan produce extremely dense pericardial adhesions thatrarely lead to constriction.6 As with many other diseases,the dominant causes of CP have changed over the years.Tuberculosis was historically the leading cause of CP andremains a dominant cause in developing countries. Neo-plasm, collagen vascular disease, infectious etiologies,radiation therapy, and previous cardiac surgery are someof the more common causes of constriction in moderndeveloped countries.7 However, in numerous cases (evenafter microscopic and culture examination of pericardialscar tissue), an inciting etiology is not found. In fact, idio-pathic or presumed viral etiologies are now the leading

CARDIOLOGY BOARD REVIEW MANUAL

Constrictive Pericarditis: A Case Study

William J. Nicholson, MD, and Andro G. Kacharava, MD, PhD

C o n s t r i c t i v e P e r i c a r d i t i s : A C a s e S t u d y


cause of CP in developed countries, composing up to42% of cases in some series.8 Nevertheless, a thoroughsearch for tuberculosis (ie, evidence of granulomatouslesions) must be made before concluding that the peri-cardial disease is of unknown origin.9

RCM is the least common of the cardiomyopathicdisorders.10 RCM may result from various primary local-ized cardiac processes or may be secondary to systemicinfiltrative or storage diseases.11 Cardiac amyloidosis isthe most common cause of RCM in developed coun-tries, whereas endomyocardial fibrosis is endemic inparts of Africa, India, Asia as well as South and CentralAmerica.12 Although restrictive disease can arise fromsystemic disorders that are rarely encountered in clini-cal practice, conditions such as amyloidosis, sarcoidosis,and hemochromatosis are more common. IdiopathicRCM is an uncommon disorder manifested by the char-acteristic hemodynamic abnormalities of restriction inthe absence of any identifiable cause. Because restric-tive physiology is seen in patients with various systemicdisorders, idiopathic RCM is a diagnosis of exclusion.Although RCM can occur in elderly persons, it shouldbe differentiated from the age-related changes in dias-tolic compliance.13

II. CLINICAL AND PHYSICAL FINDINGS

CASE PATIENT 1 PRESENTATION

Patient 1 is a 52-year-old woman who presents to thegeneral medical clinic complaining of 6 months of dys-pnea. The patient has gained approximately 25 lb dur-ing the preceding several months, with increased ab-dominal girth and discomfort as well as marked lowerextremity swelling. Before her present complaints, shehad been in good health and was taking no medica-tions. Her last medical visit was 15 years earlier whenshe had a cholecystectomy. Initial physical examinationreveals a frail cachectic woman with pitting lower ex-tremity edema, hepatomegaly, and tense ascites. Breathsounds are diminished at the bases bilaterally. Cardio-vascular examination is remarkable for a resting tachy-cardia, jugular venous distention (JVD), and an earlydiastolic sound thought to be an S3. Liver transaminaselevels are elevated, and the patient’s albumin is slightlyreduced. A paracentesis is performed, which reveals atransudate effusion.

Radiologic evaluation of the chest and abdomen aswell as colonoscopy for potentially malignant etiologiesof the prominent ascites and liver disease are both unre-vealing. However, a computed tomography (CT) scan

of the chest shows mild thickening of the pericardium.Liver biopsy shows congestion and nonspecific cirrhosiswithout evidence of infiltrative disease. Subsequentechocardiographic evaluation and cardiac catheteriza-tion are consistent with the diagnosis of CP and help indifferentiating it from RCM.

• What symptoms and signs are frequently associatedwith CP?

• Can these findings reliably differentiate CP fromRCM?

DISCUSSION

The clinical presentation and physical findings aresimilar in CP and RCM.5,14 Symptoms are typically insidi-ous in onset and are closely related to the degree of sys-temic and central venous congestion as well as the degreeof fluid retention. Poor appetite, cachexia, abdominaldiscomfort resulting from splanchnic engorgement,

Table 1. Causes of Constrictive Pericarditis

Idiopathic

Infectious*

Drugs†

Radiation

Chest trauma

Cardiovascular surgery

Heart transplantation

Epicardial defibrillator patches

Connective tissue disease‡

Renal failure (on chronic dialysis)

Myocardial infarction

Neoplasms

Sarcoidosis

Mulibrey nanism (Perheentupa syndrome)

Porphyria cutanea tarda

Asbestosis

Whipple’s disease

Chylopericardium

*Tuberculosis, viral, bacterial, or histoplasmosis.†Hydralazine, cromolyn sodium, procainamide, penicillins, isoniazid,

minoxidil, phenylbutazone, or methysergide.‡Systemic lupus erythematosus, rheumatoid arthritis, or dermato-

myositis.

Adapted from Myers RB, Spodick DH. Constrictive pericarditis: clini-cal and pathophysiologic characteristics. Am Heart J 1999;138(2 Pt 1):219–3, with permission from Elsevier Science.



ascites, and peripheral edema with general fatigue andweakness can cause the clinician to inappropriatelywork-up a diagnosis of liver disease. As central diastolicpressures increase and symptoms of dyspnea, orthop-nea, and occasionally platypnea become more promi-nent, clinicians can erroneously diagnose CHF. As seenwith patient 1, failure to recognize the significance ofelevations in jugular venous pressure (JVP) in a patientpresenting with clear lungs, unremarkable chest radio-graph, hepatomegaly, and ascites may lead the clinicianto pursue liver biopsy before the correct diagnosis is rec-ognized.15

CP and RCM may present predominantly with symp-toms of systemic or pulmonary venous congestion de-pending on the extent and area of pericardial scarringor on the restrictive involvement of the myocardium.Thus, it is difficult to establish the diagnosis on clinicalgrounds alone. Symptoms can develop from weeks todecades after an inciting event (which the patient oftendoes not recall), making the diagnosis less obvious. Sev-eral studies have shown that the average duration ofsymptoms before diagnosis was nearly 2 years.16,17 Forthis reason, constriction and restriction should be con-sidered in any patient with unexplained elevation in

central venous pressure, particularly if there is a historyof a predisposing condition. A clinical history of peri-carditis, tuberculosis, trauma, or radiation therapymakes a diagnosis of CP more likely, whereas a historyof a predisposing systemic disease (such as amyloidosisor sarcoidosis) would favor RCM.

Neck Vein Examination

Examination of the neck veins is essential when con-sidering the diagnosis of CP. Increased JVP has beenreported in 93% of patients with CP18 and is frequentlyencountered in RCM. Kussmaul’s sign, or inspiratoryJVD, replaces the normal inspiratory venous collapse ofthe jugular veins in patients with CP. Although usuallypresent in CP, Kussmaul’s sign is not specific; it is fre-quently present in RCM and can be observed in tricuspidstenosis, right heart failure, or systemic venous conges-tion of any cause. Figure 1 demonstrates the normaljugular venous pulsation contrasted with that seen in pa-tients with CP.19

The normal jugular venous pulsation consists of 3 positive waves (A, C, and V) and 3 negative waves (X,X′, and Y). The positive A-wave is caused by the rightatrial pressure transmitted to the jugular veins duringright atrial systole, and atrial relaxation results in thedescent of the A-wave. The C-wave occurs during thebeginning of right ventricular systole, resulting fromthe bulging of the tricuspid valve into the right atrium.The X-wave descent, representing the atrial relaxation,is a negative wave preceding the C-wave that occurs insystole and followed by X′-wave descent, which repre-sents systolic down movement of the floor of the rightatrium caused by the right ventricular contraction. TheV-wave occurs after the X′-wave descent as a result of anincrease in right atrial pressure and JVP caused by con-tinued inflow of blood to the venous system during lateventricular systole when the tricuspid valve is still closed.The Y-wave descent, or diastolic collapse, is produced byopening of the tricuspid valve and the rapid inflow ofblood into the right ventricle. A sharp Y-wave descentand a rapid ascent to the baseline (referred to as theFriedreich’s sign) are seen in CP (Figure 1B). Thevenous pressure is elevated in these conditions with asharp Y-wave dip in the jugular venous pulsation form-ing a characteristic “W” shape known as the W-wave ofBloomfield.

Cardiac Examination

Cardiac examination can be helpful in diagnosingCP, differentiating it from RCM, and excluding otherpotential etiologies. In RCM, the heart size is preservedand the ventricular systolic impulse is usually normal or

Normal

A

C

X′

X

X

V

Y

Y

V

X′

A

Constrictive pericarditis

Figure 1. Jugular venous pulsation. (A) Normal jugular venouspulsation. (B) Jugular venous pulsation in patients with CP. Thesharp Y-wave dip forms the characteristic “W” shape known asthe W-wave of Bloomfield. CP = constrictive pericarditis.(Adapted from Fuster V, Alexander RW, O’Rourke RA, et al,editors. Hurst’s the heart. 10th ed. New York: McGraw-HillMedical Publishing Division; 2001:228–9. Copyright 2001, withpermission of the McGraw-Hill Companies.)

A

B



enhanced; heart size is either normal or smaller in pa-tients with CP. The non-pliable and rigid encasement ofthe heart in CP results in precordial palpation that isoften quiet without an appreciable ventricular impulse.An S3 is frequently heard in RCM. The abruptly ampu-tated ventricular filling during diastole is caused by con-striction from the rigid pericardium and results in a peri-cardial knock in CP. The knock usually occurs 0.06 to0.12 seconds after the S2 sound and may be made moreprominent by having the patient squat, which increasesafterload.20 In patient 1, pericardial knock was interpret-ed as an S3, evidence of certain degree of CHF, whichtogether with liver cirrhosis were considered the mostlikely initial diagnoses. Significant pulsus paradoxus ofgreater than 10 mm Hg is infrequently encountered inpure CP or in RCM. The constricting pericardium doesnot transmit changes in pleural pressures (see next sec-tion); therefore, the presence of pulsus paradoxus sug-gests coexistent pulmonary disease or effusive CP.21

III. PHYSIOLOGY AND PATHOLOGY

The rigid, non-pliable shell that encases the heart inCP sharply accentuates the ventricular pressure-volumerelationship through several mechanisms. First, intratho-racic and intracardiac pressures are dissociated during res-piration. The effect of the respiratory cycle on intra-thoracic blood flow in CP is critical to understanding thepathophysiologic features.22,23 Normally, inspiration de-creases intrathoracic pressure, which is transmitted to allintrathoracic structures including the pulmonary veinsand cardiac chambers. However, the encased pericardi-um of CP isolates the cardiac chambers from changes inintrathoracic pressure. Therefore, as intrathoracic pres-sures decrease during inspiration, cardiac pressures re-main high. Less of a pressure gradient is created betweenthe pulmonary veins and the left-sided chambers of theheart, resulting in less total pulmonary venous return anddecreased flow velocity during inspiration. This results ina decrease in left ventricular filling with inspiration. Theopposite effect is seen with expiration in CP.

The second physiologic hallmark of CP results frommarked ventricular interdependence. The total cardiacvolume is fixed by the non-pliable pericardium. There-fore, the total amount of blood entering the heart doesnot vary significantly during the respiratory cycle. Duringexpiration, the increase in the intrathoracic pressuredoes not affect the cardiac chambers. However, the pul-monary veins and venae cavae are not shielded by therigid pericardium, and flow velocities in these vessels areaffected. Consequently, flow into the left ventricle with

expiration is increased. The ventricular septum is notdirectly affected by the pericardium and is free to bulgeinto the right ventricle causing reduction in flow velocityin the venae cavae and decreased trans-tricuspid flowvelocity.24 Echocardiography demonstrates these changesin flow velocities, as discussed in the next section.

The third major effect of the inflexible pericardium ofCP on cardiac hemodynamics is impairment of diastolicfilling. The non-pliable pericardium of CP limits diastolicfilling in all cardiac chambers, resulting in elevated end-diastolic pressures. Normally, approximately 75% of ven-tricular filling occurs during phase 2 (rapid filling) ofdiastole, and 10% to 20% occurs during phase 4 (atrialcontraction).15 Because of the elevated atrial pressures inCP, up to 75% to 80% of ventricular filling occurs in thefirst 25% to 30% of diastole.25 Filling rapidly declines bymid-diastole and filling is severely limited in late diastole.Tachycardia would adversely affect diastolic ventricularfilling in a normal patient. In patients with CP, wherenearly all ventricular filling occurs by mid-diastole, tachy-cardia becomes an important means of maintaining car-diac output. Consequently, tachycardia and atrial fibrilla-tion are relatively less important in CP.26

The hallmark of chronic CP is a thickened adherentpericardial sac that restricts filling of the cardiac cham-bers. Pathologically, pericardial inflammation leads tothe deposition of fibrous strands and proliferation of cel-lular infiltrates.27 Eventually, calcification occurs withtotal or near total obliteration of the pericardial space aswell as the remaining fluid, pus, or blood. In some in-stances, loculated large collections of fluid may compressindividual cardiac chambers. Additionally, bandlike con-strictions can develop and compromise any portion ofthe heart, including the valve rings and great vessels,mimicking intrinsic disease of the affected structures.28,29

IV. DIAGNOSTIC IMAGING MODALITIES

ELECTROCARDIOGRAPHY (ECG) FINDINGS

• Can the 12-lead ECG and routine chest radiographyassist diagnosis of CP?

• What additional studies are necessary to accuratelydiagnose CP?

No specific ECG abnormalities are characteristic foreither CP or RCM. Low QRS voltage and nonspecific T-wave changes are common in both conditions. Acompensatory sinus tachycardia is frequently present inRCM and CP, as seen in patient 1. Atrial fibrillation maybe present in CP (up to 10% of patients) and RCM;



however, ventricular arrhythmias and abnormalities ofconduction, including atrioventricular block, are seenmore frequently in RCM. Because these findings arenonspecific, it is not surprising that an ECG tracingrarely can help to differentiate between RCM and CP.However, the presence of a completely normal tracingshould lead the clinician to reconsider the diagnosis ofCP or RCM.

RADIOLOGIC IMAGINGChest Radiography

Chest radiography as a single noninvasive imagingmodality can occasionally help to distinguish CP fromRCM. Pericardial calcification is best seen in the lateralview. “Eggshell” calcifications or amorphous calcifica-tions in the atrioventricular grooves may be seen. Al-though calcification may support the clinical suspicionof CP, its presence is not always specific because it mayoccur without cardiac compression.30

Blood Pool Radionuclide Ventriculography

Blood pool radionuclide ventriculography usuallydemonstrates that the elapsed time to peak filling is de-creased during inspiration, which may be a findingunique to CP.31,32

Computed Tomography and Magnetic ResonanceImaging (MRI)

CT and MRI are the best radiologic modalities todemonstrate pericardial thickening. When imaging withmultislice spin-echo (gated) MRI, the normal pericardi-um appears as a thin (< 3 mm), low-intensity signalbetween the high-intensity signal of the epicardial fat

and the medium-intensity signal of the myocardium.33

Although pericardial fluid also has a low-signal intensity,fluid appears bright on ciné MRI images, allowing one todistinguish fluid from pericardium and to accuratelyassess the pericardial thickness. Pericardial thickening of4 mm or more favors a diagnosis of CP, whereas thicken-ing more than 6 mm is highly specific for CP (Figure 2).34

It should be mentioned that the pericardium can be con-stricting even when it is so thin that it is not recognized asabnormal by any contemporary imaging techniques.Nevertheless, pericardial thickening of more than 5 mm,an enlarged right atrium, and a distorted tubular rightventricle are all highly predictive of pericardial constrict-ing disease.34 In some cases of heavy pericardial calcifica-tion, distinguishing between calcification and fibrous tis-sue may be difficult with MRI. In these cases, CT imagingof the pericardium may be better to assess pericardialthickness.22,35,36 Besides assisting in the diagnosis of CP,CT and MRI permit detailed planning of the surgical ap-proach in patients by revealing the distribution and vari-ations of pericardial thickness, calcification, and possiblemyocardial invasion.

ECHOCARDIOGRAPHY

No single pathognomic echocardiographic findingexists for CP. To distinguish CP from RCM, a combina-tion of findings need to be used. Both M-mode and 2-dimensional echocardiography are not very helpfulin making the distinction between these 2 conditions.In severe cases, the increased thickness and calcifica-tion of the pericardium can be seen with M-mode and 2-dimensional evaluation. Although evaluation of peri-cardial thickness with transthoracic echocardiography

Figure 2. MRI of the pericardium in a patient with constrictive pericarditis. Progressive cardiac failure developed during a viral illness in a76-year-old woman. Gated, T1-weighted, spin–echocardiogram MRI of the heart in the transverse axial view (A) and oblique sagittal view(B) showed diffuse thickening of the entire pericardium, calcifications (arrows in A), RA enlargement, a moderate amount of fluid betweenthe 2 pericardial layers, and increased signal in the cavity, indicative of stasis of blood. LA = left atrium; LV = left ventricle; MRI = magneticresonance imaging; RA = right atrial; RV = right ventricle. (Reprinted with permission from De Benedetti E, Didier D. Images in clinical med-icine. Constrictive pericarditis. N Engl J Med 2000;343:107. Copyright © 2000 Massachusetts Medical Society. All rights reserved.)

RA

RV

RVLV LV

LA

A B



is insensitive, measurement with a transesophagealechocardiogram (TEE) correlates strongly with mea-surements obtained by CT. One study demonstratedthat the superior resolution achieved with TEE allowedbetter pericardial definition; a thickness of 3 mm ormore on TEE was 95% sensitive and 86% specific forthe detection of thickened pericardium.37

Doppler echocardiography and the relatively newermethods of color M-mode and tissue Doppler studiescan differentiate CP from RCM. As previously men-tioned, an important pathophysiologic consequence ofCP is the greatly enhanced interventricular depen-dence resulting from a limited total cardiac volume withan uninvolved, freely moving septum. Thus, ventricularfilling and emptying velocities assessed by mitral and tri-cuspid inflow velocities have much greater respiratoryvariation in CP in contrast to RCM where the interven-tricular septum is involved by the pathological process.Patients with CP have greatly exaggerated reciprocalvariations in the rates of left and right ventricular fillingrelated to the respiratory cycle when compared withnormal subjects.38,39 In most patients with CP, mitralinflow velocity decreases as much as 25% and tricuspidvelocity greatly increases with the first beat after inspira-tion. In contrast, respiratory variation in patients withRCM does not significantly differ from the normal.Doppler interrogation of the pulmonary and hepaticvenous flow can further assist in the differentiation ofthese 2 conditions.40 These respiratory variations are il-lustrated in Figure 3.

New Doppler methods have revealed that color Dop-pler M-mode evaluation of mitral inflow can help distin-guish between RCM and CP. The flow propagation slopeis steep in CP, whereas the same parameter is significant-ly lower in RCM (Figure 4).41 Additionally, tissue Dopplerimaging has shown that patients with CP have a normalor a high normal early diastolic velocity (> 8 cm/sec),whereas patients with RCM have low early diastolic veloc-ities (< 8 cm/sec; Figure 5).42 Doppler echocardiographyhas proven to be one of the most reliable noninvasivemethods to help distinguish CP from RCM.

V. HEMODYNAMICS

Despite the availability of several noninvasive meth-ods of assessment, cardiac catheterization is valuablewhen evaluating a patient with suspected RCM or CP.Catheterization permits quantification of left and rightheart pressures. Additionally, endomyocardial biopsycan be performed and may identify coexisting cardiacdisease or may confirm RCM resulting from an infiltra-

tive process. Finally, catheterization provides informa-tion regarding the coronary anatomy in a patient whowill undergo pericardiectomy and may need concomi-tant coronary artery bypass graft surgery.

Although cardiac catheterization does not necessari-ly confirm the diagnosis of CP, Vaitkus and Kussmaul’sshowed that the overall predictive accuracy of 3 majorhemodynamic criteria can be very specific.43 A differ-ence between right ventricular end diastolic (RVEDP)pressure and left ventricular end diastolic pressure(LVEDP) of 5 mm Hg or less, a right ventricular systolicpressure (RVSP) of 50 mm Hg or less, and a ratio ofRVEDP to RVSP of 1:3 or more are 85%, 70%, and 76%sensitive, respectively, for correctly diagnosing CP. If all3 of these criteria are met, a diagnosis of CP is correctin greater than 90% of patients.

As previously discussed, the tight ventricular inter-action in conjunction with insulation of the cardiacchambers from variations of intrathoracic pressuresduring the respiratory cycle are the 2 key mechanismsunderlying the pathophysiology of CP. These funda-mental principles explain why measuring respiratoryvariation in ventricular hemodynamics during catheter-ization is paramount in the diagnosis of CP.44

As a manifestation of ventricular interdependence,respiratory discordance occurs between the peak RVSPand left ventricular systolic pressure (LVSP) in CP. RVSPincreases with inspiration as LVSP simultaneously de-creases. The pulmonary capillary wedge pressure de-clines more than the LVEDP during inspiration, and thereduction in mitral valve inflow translates into the ob-served reduction in LVSP. In RCM, both the RVSP andLVSP decrease concordantly with inspiration.44

Several other findings on cardiac catheterization canassist in the diagnosis of CP such as increased distancebetween coronary arteries and the cardiac silhouette,which suggest the presence of thickened pericardium, aswell as tethering of coronary arteries to the pericardium.

VI. TREATMENT

• What is the definite therapy for CP?• What are the possible complications of complete

pericardiectomy?

DISCUSSION

The main reason that it is essential to distinguishRCM from CP is that the 2 disorders have distinctlydifferent treatments. CP can be cured surgically withpericardiectomy. Although constrictive physiology



occurring in acute pericarditis may revert sponta-neously,45 surgical removal of the pericardium is nec-essary in most cases. Complete pericardiectomy is theoperative procedure of choice; however, it is surgicallychallenging.46,47 Removal should be as extensive aspossible; however, sometimes calcified islands must beleft or a palliative meshing of the pericardium may berequired to minimize damage to the coronary arteriesand myocardium. Removal of adherent pericardium

may require a prolonged procedure complicated byhypotension and arrhythmias. Most patients respondwell to surgery, but the reported operative mortality of12% is significant.46 Some patients have immediateand often profuse postoperative diuresis, althoughothers may take weeks to months to recover aftersurgery. Poorer results are seen in patients with inade-quate resection, uncorrected coronary artery disease,higher New York Heart Association classifications, and

Normal

Normal

Constrictivepericarditis

Constrictivepericarditis

Restrictivemyocardial

disease


disease

ECG

Apnea

Inspiration

ExpirationNormal Constrictive

pericarditisRestrictivemyocardial

disease

ECG

Apnea

Inspiration

Expiration

Normal Constrictivepericarditis


disease

ECG

Apnea

Inspiration

Expiration

EA E

AE

A

ECG

Apnea

Inspiration

Expiration

E A EA

E

A

A

B D

C

S SSD D

D

S SSD D

Figure 3. Schematic representations of flow patterns (using Doppler echocardiography) in normal subjects, patients with constrictivepericarditis, and patients with restrictive cardiomyopathy. A simultaneous ECG tracing is shown across the top. (A) Respiratory varia-tion across the mitral valve. (B) Respiratory variation in flow across the tricuspid valve. Changes in flow are opposite of those seenacross the mitral valve. (C) Respiratory variation in pulmonary venous flow. (D) Respiratory variation in hepatic venous flow. A-wave = late diastolic filling from atrial contraction; D-wave = peak diastolic flow velocity; ECG = electrocardiographic; E-wave = earlydiastolic filling; S-wave = peak systolic flow velocity. (Adapted from Leung DY, Klein AL. Restrictive cardiomyopathy: diagnosis and prog-nostic implications. In: Otto CM, editor. Practice of clinical echocardiography. Philadelphia: WB Saunders; 1997:474, with permissionfrom Elsevier Science.)



older age. Patients with radiation-induced CP andthose with peripheral organ failure involving the liveror kidneys with ascites and edema also have poorerpostoperative outcomes.46

Occasionally, the chronic external support of thetight pericardium can lead to myocardial atrophy,which can sometimes be detected preoperatively bydecreased myocardial signals on MRI or CT. Re-leasing the heart surgically may allow rapid dilation ofthe ventricle, leading to systolic dysfunction and heartfailure.47,48 Patients dying with low cardiac outputafter complete pericardiectomy have histologic find-ings consistent with myocardial atrophy. When thiscondition is advanced, the heart may continue todilate as it fails, resulting in what has been called theexploding heart syndrome. Fortunately, the condi-tion is reversible in some patients if they can be assist-ed for several days with maximal medical and circula-tory support.49

On the other hand, treatment of RCM is often symp-tomatic. Diuretics are frequently used to relieve systemicand pulmonary venous congestion, which must be donewith caution because high filling pressures are needed tomaintain ventricular filling in RCM. Excessive use of di-uretics may reduce filling and lead to decreased cardiacoutput with resultant systemic hypoperfusion manifested

by fatigue, lightheadedness, and prerenal azotemia.Careful monitoring for these findings should alert theclinician that further diuresis should be avoided. As dis-cussed earlier, efficient diastolic filling is compromised inRCM, leading to the understanding that rate-loweringdrugs (such as β-blockers and calcium channel an-tagonists) may have a beneficial role. Slowing the heartthus allows for more diastolic time in each cardiac cycle and for improved ventricular relaxation and filling.Angiotensin–converting enzyme inhibitors have a prov-en beneficial role in systolic dysfunction; evidence sug-gests they may be beneficial in diastolic dysfunction andthus have a role in treating RCM.50

Rhythm disturbances can be detrimental in patientswith RCM. Atrial fibrillation worsens diastolic dysfunc-tion because of the loss of the atrial contribution to ven-tricular filling. Additionally, a rapid ventricular re-sponse can further compromise patients with RCM.Attempts should be made to maintain sinus rhythm viacardioversion or antiarrhythmic drugs such as amio-darone. If sinus rhythm cannot be achieved or main-tained, then adequate rate control is necessary, andanticoagulation with warfarin is recommended. In fact,the hypercoagulable propensity of particular etiologiesof RCM warrant anticoagulation to prevent thrombusformation regardless of the patient’s rhythm.51

Figure 4. Color Doppler M-mode of dias-tolic flow from the left atrium (LA) towardthe ventricular apex as imaged from the 4-chamber view in patients with constric-tive pericarditis (A) or restrictive cardio-myopathy (B). The flow propagation slopeof the first aliasing contour (white line) issteep for constrictive pericarditis, but theslope is significantly slower in restrictivecardiomyopathy. Respiratory monitoring isshown with inspiration (up arrow) and expi-ration (down arrow). (Reprinted fromRajagopalan N, Garcia MJ, Rodriguez L, etal. Comparison of new Doppler echocar-diographic methods to differentiate con-strictive pericardial heart disease andrestrictive cardiomyopathy. Am J Cardiol2001;87:93, with permission from ExcerptaMedica, Inc.)

APEX

APEX

LA

LA

A

B



Infiltrative disorders may result in advanced conductionsystem disease. Dual chamber pacing should be used tomaintain properly timed atrial contraction to improveventricular filling.

Advanced diastolic dysfunction secondary to RCM isassociated with a poor prognosis in patients with sec-ondary causes such as amyloidosis or hemochromatosis.Idiopathic RCM also has a poor outcome, with 64% and37% survival at 5 and 10 years, respectively.52 Cardiactransplantation can be performed in patients withrefractory symptoms in idiopathic or familial RCM.Combined heart and liver transplantation in patientswith hemachromatosis has yielded good results.53 Al-though transplantation has been a treatment option forsarcoidosis and amyloidosis, recurrence can occur inthe transplanted heart.54–56

DIAGNOSIS AND TREATMENT OF PATIENT 1

Thickening of the pericardium observed on CT scanin patient 1 assisted in establishing the diagnosis of CP,which was further supported by Doppler echocardio-graphic and hemodynamic data obtained during car-diac catheterization. Pericardial biopsy confirmed thediagnosis of CP, and subsequent pericardiectomy is suc-cessful.

VII. SUMMARY POINTS

• The fibrous pericardium envelops the entire heart andmuch of the ascending aorta, the main pulmonary

artery, all 4 pulmonary veins, and portions of the infe-rior and superior venae cavae in patients with CP.

• Tuberculosis has historically been the leading causeof CP; however, neoplasm, collagen vascular disease,infectious etiologies, radiation therapy, and previouscardiovascular surgery are some of the more com-mon causes of constriction in modern developedcountries.

• Symptoms are typically insidious in onset and consistof poor appetite, cachexia, abdominal discomfort(because of splanchnic engorgement, ascites), andperipheral edema along with general fatigue andweakness. However, it is difficult to establish the diag-nosis of CP or RCM on clinical grounds alone.

• A sharp Y-wave descent and a rapid ascent to thebaseline in jugular venous pulsations are seen in CP,RCM, or severe right-sided heart failure.

• In patients with constrictive pericarditis, the rigidpericardium causes an abruptly amputated ventricu-lar filling during diastole, resulting in a pericardialknock that may be made more prominent by havingthe patient squat.

• The rigid, inelastic “shell” that encases the heart in CPsharply accentuates the ventricular pressure-volumerelationship through several mechanisms.

• Pericardial calcification is often seen on the lateralradiograph in CP.

• MRI is very accurate in assessing pericardial thick-ness; thickening of 4 mm or more favors a diagnosisof CP, whereas thickening greater than 6 mm is veryspecific for CP.

Figure 5. A normal subject, a patient withrestrictive cardiomyopathy, and a patientwith constrictive pericarditis: representa-tive examples of the (A) mitral annular M-mode, (B) transmitral Doppler flowvelocities, and (C) tissue Doppler axial ve-locities. A marked difference in early dias-tolic velocity (arrows) despite similar earlytransmitral flow velocities is observed.(Reprinted from Garcia MJ, Thomas JD,Klein AL. New Doppler echocardiograph-ic applications for the study of diastolicfunction. J Am Coll Cardiol 1998;32:871,with permission from the American Col-lege of Cardiology Foundation.)

50 cm/s

Normal Restriction Constriction

50 cm/s

20 cm/s

5 cm/s 5 cm/s 5 cm/s

A

C

B



• Ventricular filling and emptying velocities as assessedby mitral and tricuspid inflow velocities have muchgreater respiratory variation in CP when comparedwith RCM.

• New Doppler methods have revealed that color Dop-pler M-mode evaluation of mitral inflow can helpdistinguish between RCM and CP.

• Cardiac catheterization can be used to diagnose CP.A difference between right ventricular end diastolicpressure (RVEDP) and left ventricular end diastolicpressure of 5 mm Hg or less, a right ventricular sys-tolic pressure (RVSP) of 50 mm Hg or less, and aRVEDP to RVSP ratio of 1:3 or more are consistentwith CP.

• CP can be cured surgically with pericardiectomy;however, the reported operative mortality is 12%.

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