Constrictive Pericarditis- A Reminder of a Not So Rare Disease

7/28/2019 Constrictive Pericarditis- A Reminder of a Not So Rare Disease

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Review article

Constrictive pericarditis: A reminder of a not so rare disease

Michael Bergman a,c,, Janos Vitrai b, c, Hertzel Salmana,c

a Department of Internal Medicine C, Rabin Medical Center, Gold a Campus (Hasharon), Petah-Tiqva, Israelb Cardiology Department, Rabin Medical Center, Golda Campus (Hasharon), Petah-Tiqva, Israel

c The Sackler School of Medicine, Tel-Aviv University, Ramat-Aviv, Israel

Received 21 June 2005; received in revised form 11 May 2006; accepted 3 July 2006

Abstract

Constrictive pericarditis is a rare condition characterized by clinical signs of right heart failure subsequent to loss of pericardial

compliance. The etiology of constrictive pericarditis has changed during the last decades in developed countries. While, in the past,

tuberculosis and idiopathic pericardial constriction were the prevalent causes of the disease, cardiac surgery has become one of the main

reasons for its development in recent years. However, cases defined as idiopathic constrictive pericarditis are still observed. In addition to the

classical chronic and subacute forms, new presentations, such as effusive-constrictive, localized, transient, occult, and constrictive pericarditis

with normal pericardial thickness, have been described. Although conservative treatment may alleviate the patient's symptoms,

pericardiectomy remains the only definitive treatment for the disease. It is worth noting that the sooner the diagnosis of pericardial

constriction is established, the better the outcome is. The pathophysiological features, clinical findings, diagnostic tools, and therapeutic

approach to constrictive pericarditis are detailed in this review.

2006 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved.

Keywords: Constrictive pericarditis; Pericardium; Constriction

Contents

1. Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 458

2. Pathophysiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 458

3. Etiology and pathology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 458

4. Clinical features . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 459

5. Additional examinations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 459

5.1. Chest X-ray . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 459

5.2. Electrocardiography . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 460

5.3. Echocardiography . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 460

5.4. Heart catheterization and angiography . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 461

5.5. Computed tomography (CT) and magnetic resonance imaging (MRI) . . . . . . . . . . . . . . . . . . . . . . . . . . 461

5.6. Differential diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 461

5.7. Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 462

5.8. Surgical treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 462

5.9. Rare forms of constrictive pericarditis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 462

6. Learning points . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 463

European Journal of Internal Medicine 17 (2006) 457464

www.elsevier.com/locate/ejim

Corresponding author. Department of Internal Medicine C, Rabin Medical Center, Golda Campus (Hasharon), 7, Keren Kayemet St., Petah Tiqva, Israel.

Tel.: +972 3 9372598; fax: +972 3 9372604.

E-mail addresses: [email protected], [email protected] (M. Bergman).

0953-6205/$ - see front matter 2006 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved.

doi:10.1016/j.ejim.2006.07.006
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Acknowledgements . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 463

References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 463

1. Introduction

Dyspnea and peripheral edema are common dilemmas,

and detecting the cause of their appearance should not present

difficulties for an experienced physician. The main diagnostic

possibilities to be considered are the frequent causes of right

heart failure. However, the differential diagnosis should

comprise rare conditions accompanied by diseases of the

pericardium, including constrictive pericarditis [1]. The des-

cription of constrictive pericarditis designated as concretio

cordis dates back more than 300 years [2]. It presents mostly

as chronic fibrous pericardial thickening, calcification of the

pericardium, or both. However, in atypical forms, heartconstriction may develop with a pericardium that is normal in

thickness [3]. According to Hancock [4], compressive peri-

cardial disease may be the sequel of tamponade, as well as

either subacute (fibroelastic) or chronic (rigid shell) constric-

tion. We review herein the natural history of this progressive

and debilitating pericardial disease.

2. Pathophysiology

In the classical form of constrictive pericarditis, the rigid,

heavily fibrosed, or even calcified pericardium causes

restriction of the myocardium, preventing adequate ventric-

ular filling after an initial expansion [2,5]. Because ofdecreased pericardial compliance and increased venous

pressure, the early diastolic filling occurs very rapidly and

stops when the intracardiac volume and pressure reach their

maximum limits. Since the myocardium in constrictive

pericarditis is essentially not affected, relaxation of the left

ventricle is usually normal, indicating that the abnormal

compliance of the myocardium that results in elevated

diastolic pressure equilibrium in all four chambers is due to

the rigid pericardium [2,6]. Consequently, the atrial waves on

the jugular veins show a prominent and deep diastolic Y

descent. The systolic X descent that reflects a fall in the right

atrial pressure throughout early ventricular systole maycreate an M or W pattern of the venous wave [6].

The hallmarks of constrictive pericarditis are the hemody-

namic changes observed during respiration. As a consequence

of a diminished gradient between the pressure in the pul-

monary veins and the left atrium during inspiration, the left-

side flow decreases while the right-side flow increases. These

alterations are due to pericardial rigidity that limits transmis-

sionof decreased intrathoracic pressure to the pericardial space

[2,5]. The existence of hemodynamic alterations in this

condition may also be explained by the rigidity of the

pericardium that limits heart relaxation during diastole with a

subsequent increase in the right heart volume, causing higher

pressure on the intraventricular septum. Subsequently, theseptum moves to the left and the left-side volume decreases.

Occasionally, the increased venous return from the overfilled

splanchnic system elevates the abdominal and right atrial

pressures during inspiration, resulting in an inspiratory dis-

tention of the jugular veins, a phenomenon designated as

Kussmaul's sign [7]. Pulsus paradoxus, i.e., disappearance of

the peripheral pulse during inspiration, is another clinical sign

that can be found in patients with constrictive pericarditis. This

interesting phenomenon is explained by impaired filling of the

left ventricle with an enhanceddecreasein the systolic pressure

during inspiration caused either by fibrosed and calcified

pericardium or by the accumulation of pericardial fluid [8].Shabetai et al. [9] have shown that patients with constricted

pericardium are not able to increase the flow velocity in either

the vena cava or the pulmonary artery during inspiration, a

finding that may be one of the mechanisms for the appearance

of pulsus paradoxus. Pooling of blood in the pulmonary bed

and filling competition of the ventricles in the presence of a

fixed pericardial sac may serve as an additional cause for

appearance of pulsus paradoxus [8].

3. Etiology and pathology

The common forms of constrictive pericarditis are the

subacute elastic constriction and the classical, chronic rigid

constrictive pericarditis. The elastic form of constrictive

pericarditis, described by Hancock [4], presents with clinical

features of tamponade, although the actual cause of the disease

is pericardial constriction. While, in the past, the main cause of

constrictive pericarditis was tuberculosis [2,6], the etiology of

the disease has changed during recent decades [10]. Nowadays,

the most frequent causes of the illness in developed countries

are prior cardiac surgery, irradiation therapy, and idiopathic

pericarditis, while tuberculosis remains the main etiology of the

disease in developing countries [11]. Bertog et al. [12]

examined 163 patients who underwent pericardiectomy

because of constrictive pericarditis over a 24-year period.Forty-six percent of the patients suffered from the idiopathic

form, 37% underwent heart surgery, in 9% the disease deve-

loped after mediastinal irradiation, mostly for Hodgkin's

disease, and in 3.5% the causative agent was tuberculosis.

Rheumatoid arthritis,systemic lupuserythematosus, prior chest

trauma, Wegener's granulomatosis, and purulent pericarditis

were the reason for the disease in the remaining patients.

Ling et al. [13] compared two cohorts of patients after

radical pericardiectomy, one who underwent surgery during

the years 19361962 and the second during the years 1985

1995. The results showed that in patients undergoing surgery

in the last 10 years, open-heart surgery and chest irradiation

458 M. Bergman et al. / European Journal of Internal Medicine 17 (2006) 457464


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emerged as the most frequentcauses of heart constriction. It is

worth noting that in 1218% of patients with constrictive

pericarditis undergoing pericardiectomy, the pericardium was

of a histologically proven normal thickness [3,14]. The time

interval between heart surgery and appearance of constrictive

pericarditis ranges from 3 to 24 months, although it may be as

short as 4 weeks [15]. Several factors, such as residual bloodelements within the pericardial sac, trauma during the

surgical procedure, pericardial irrigation with povidone-

iodine solution, and low-grade infection may be implicated in

the link between surgery and the development of heart

constriction. The role of drugs and chemical substances as an

additional cause of constrictive pericarditis should not be

ignored. Hydralazine, procainamide, penicillin, minoxiline,

isoniazide, and pergolide mesylate have been reported to be

causative agents. Rare diseases, such as Mulibrey nanism,

porphyria, asbestosis, and Whipple's disease, have been

reported to be complicated by constrictive pericarditis [2,16].

Before the era of renal dialysis and transplantation, when themortality rate in patients with chronic renal failure was high,

constriction was a rare complication of uremic pericarditis.

However, the incidence of chronic constrictive pericarditis

increased after the introduction of renal dialysis and kidney

transplantation, probably as a result of improved patient

survival. Patients with renal failure who are treated by

dialysis may develop a peculiar form of pericarditis

designated as dialysis pericarditis, which appears approx-

imately 8 weeks or more after the initiation of dialysis and

may progress to constriction [17].

4. Clinical features

The classical presentation of constrictive pericarditis

consists of symptoms and signs of debilitating chronic

right-side heart failure. Lower leg edema and mild hepatic

congestion appear in the early stage of the disease. Later on,

the hepatic congestion becomes aggravated, with subsequent

development of ascites, anasarca, and jaundice. Dyspnea and

orthopnea appear during the advanced stages of the illness.

Muscle wasting, severe fatigue, and cachexia may be

observed in the end stage of constriction [6].

On physical examination, the most important clinical sign

is increased jugular venous pressure, presented as a rapidly

collapsing, negative wave of the diastolic Y descent. An Xdescent wave may appear in addition to the Y descent [6].

One should keep in mind that dyspnea, tachypnea, arrhythmia,

or tachycardia limit one's ability to observe the changes in the

jugular veins. Kussmaul described a peculiar sign representing

increased jugular venous pressure during inspiration [7,18].

However, this sign is difficult to observe at the bedside [6].

Ling et al. [13] detected the presence of Kussmaul's sign in 28

out of 135 patients with constrictive pericarditis referred for

pericardiectomy. The arterial pulse may be normal. Kussmaul

has described an additional sign designated as pulsus para-

doxus, a pulse simultaneously slight and irregular, disappear-

ing during inspiration and palpated upon expiration [7,18]. It

has been named paradoxusbecause of the absence of a radial

pulse in the presence of a corresponding heartbeat [8].

Evaluation of pulsus paradoxus may be achievedby measuring

systolic blood pressure during inspiration. A decrease in blood

pressure by more than 10 mm Hg during inspiration suggests

the existence of pulsus paradoxus. Paradoxical pulse may also

be observed in patients with cardiac tamponade, pericardialeffusion, restrictive cardiomyopathy, acute myocardial infarc-

tion, severe pulmonary embolism, bronchial asthma, and ten-

sion pneumothorax. Extreme obesity, anaphylactic shock,

stomach volvulus, and superior vena cava obstruction may

play a role in its appearance[8]. Linget al. [13] detectedpulsus

paradoxus in 19% of their patients who underwent surgery

because of pericardial constriction. It should be remembered

that finding a pulsus paradoxus that exceeds 10 mm Hg during

inspiration is uncommon in constrictive pericarditis in the

absence of excessive pericardial fluid [6].

Chest examination may reveal systolic retraction of the

apex. Diastolic pericardial knock, i.e., the presence of earlydiastolic sound, is detected 0.060.12 s after the aortic

component of the second heart sound(A2) (4). S3 was found in

46% of the patients described by the Mayo Clinic group [13]

and in 18% of those described by Bertog et al. [12]. Although

the presence of S3 is typical for the rigid form of constrictive

pericarditis, it can occasionally be heard in patients with the

fibroelastic variety of the disease [6,18]. Hepatomegaly has

been described in 63% of the patients, ascites in 45%, and

peripheral edema in 76% [12]. In addition to hepatomegaly,

prominent hepatic pulsations have been noticed [19]. Older

patients may present with huge ascites and massive edema of

the lower extremities with signs of muscle wasting and

cachexia of the upper extremities. Peripheral edema may beabsent in younger patients [20].

5. Additional examinations

5.1. Chest X-ray

Although chest X-rays are not of great help in the diag-

nosis of constrictive pericarditis, certain findings suggest the

existence of heart constriction. In the classical form of the

disease, the cardiac silhouette is not enlarged, whereas in

other forms of constrictive pericarditis an accumulation of

pericardial effusion may contribute to heart-size extension.Occasionally, the left atrium and superior vena cava may be

dilated [21]. Signs of pericardial calcification are suggestive

of constrictive pericarditis but, as stated by Lorell [6],

Calcified pericardium is not necessarily a constricted one. A

lateral chest X-ray may reveal pericardial calcification over

the right atrium and ventricle, as well as atrioventricular

grooves. Ling et al. [22] reported on roentgenological signs of

pericardial calcification in 36 out of 135 patients with

constrictive pericarditis who underwent pericardiectomy. In

97% of them, the calcifications were found on the inferior,

diaphragmatic surface of the heart, in 76% on the anterior

right ventricular area, and in 62% on the left atrioventricular

459M. Bergman et al. / European Journal of Internal Medicine 17 (2006) 457464


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groove. Following X-ray examination, Bertog et al. [12]

detected pericardial calcifications in 55% of patients withidiopathic constrictive pericarditis, in 10% of individuals

who underwent open heart surgery, and in 6.7% of those in

whom constrictive pericarditis appeared post-irradiation.

5.2. Electrocardiography

Typical electrocardiographic changes in patients with peri-

cardial constriction include low QRS voltage, left or right

bundle branch block, atrial arrhythmia or fibrillation, and the

presence of mitral P wave. In single cases, the electrocar-

diogram tracing could be normal [2,6,12]. However, one

should keep in mind that electrocardiographic findings areusually non-specific and are only suggestive for the diagnosis.

5.3. Echocardiography

The echocardiographic findings observed in patients with

heart constriction are summarized in excellent reviews by

Nishimura [5] and Troughton et al. [19]. In short, at two-

dimensional echocardiogram, a thickened pericardium (mostly

on the RVand on the RA-free walls) may be visualized, as well

as an unusual motion of the interventricular septum, desig-

nated as septal bounce, accompanied by an inspiratory septal

shift to the left. The presence of a dilated, non-collapsing

inferior caval vein is suggestive of constrictive pericarditis. On

the other hand, a normal-sized collapsing inferior caval vein atinspiration almost rules out heart constriction (Fig. 1).

The hallmark of constrictive pericarditis on Doppler

echocardiography is the increased respiratory flow variation

through the heart valves, expressed as a decreased flow

through the left-side valves during inspiration and its

increase with expiration, while on the right-side valves the

reverse phenomenon occurs. This sign is significant in cases

in which the decrease in height at the mitral E wave is at least

25%, and it is highly specific for heart constriction. It should

be noted that cardiac tamponade, due to a similar

pathophysiological mechanism, and severe COPD, due to

increased intrathoracic pressure variations, can create thesame echocardiographic changes. The flow alterations

observed during respiration may be explained by two

mechanisms. One, known as enhanced ventricular interde-

pendence, is due to suction of venous blood to the right

heart at inspiration. Since the heart in constrictive pericarditis

is confined in a rigid shell, the only way for the right heart to

react to the increased inspiratory volume is by a shift of the

interventricular septum to the left with a subsequent decrease

in left ventricular volume. The second mechanism proceeds

through dissociation between the intrathoracic and intracar-

diac pressures. Normally, the inspiratory decrease in intra-

thoracic pressure is transmitted to all intrathoracic organs

Fig. 1. Echocardiographic findings in a patient with the classical form of constrictive pericarditis. (A) Parasternal short axis view showing hyper-reflective,

thickenedposterior pericardium. (B) Doppler mitral flow demonstrating increasedrespiratory changes. (C) Doppler flow in the left ventricular outflow tract. Note

the increased respiratory changes presenting the Echo equivalent of pulsus paradoxus. (D) Subcostal view of the inferior caval vein. There is an absence of

normal inspiratory collapse, presenting the Echo equivalent of dilated jugular veins.



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including the pulmonary veins and heart chambers. In

constrictive pericarditis, the transmission of the inspiratory

negative pressure to the pulmonary veins is normal and,

therefore, does not affect the pressure in the left atrium. The

decreased pressure gradient between the pulmonary veins

and the left atrium leads to a lesser flow into the left atrium

and left ventricle through the mitral valve.The differentiation between constrictive pericarditis and

restrictive cardiomyopathy is a common echocardiographic

problem since, in both conditions, right heart failure is the

predominant clinical feature. In the classical forms, the

enlarged left and right atria on 2D echocardiography, with

thickened ventricular walls and normally appearing pericar-

dium, facilitates the diagnosis of restrictive cardiomyopathy.

If the 2D picture is equivocal for the diagnosis of restrictive

cardiomyopathy, two other distinctive signs may be helpful

for the differential diagnosis between restriction and

constriction. In restrictive cardiomyopathy, respiratory flow

changes are absent, whereas in heart constriction they arepresent. In addition, while in restrictive cardiomyopathy the

relaxation of the myocardium is abnormal, it is normal in

constrictive pericarditis since it is not a myocardial disease.

On the other hand, the so-called mixed cases may present a

difficult diagnostic problem. When the chronic inflammation

aggressively involves the visceral pericardium and the

epicardial layer of the myocardium, mixed diagnostic signs

may be observed.

5.4. Heart catheterization and angiography

In cases of constrictive pericarditis, heart catheterization

shows a nearly equal increased end-diastolic pressure in thefour chambers, the difference between the right and left

ventricular end-diastolic pressures being less than 5 mm Hg

[2,25]. Atrial pressure traces show rapid X (systolic) and Y

(diastolic) descents. Ventricular tracing shows a dip-and-

plateau pressure pattern [2]. The discrepancy between the

decreased pulmonary capillary wedge pressure and the end-

diastolic pressure in the left ventricle during inspiration

reflects a dissociation between intrathoracic and intracardial

pressures [19]. The diagnosis of heart constriction is

suggestive in cases with right ventricular pressure less than

50 mm Hg and a ratio between the right ventricular end-

diastolic pressure to the right ventricular systolic pressure

being more than 1:3 [2]. However, some of these findings can

also be observed in patients with restrictive cardiomyopathy.

5.5. Computed tomography (CT) and magnetic resonance

imaging (MRI)

CT and MRI allow direct visualization of the pericardium

and are presently the standard methods for accurate

measurement of pericardial thickness (Fig. 2). On MRI T1

imaging, pericardium with a thickness of more than 23 mm

appears as a thin, low-signal band bordered by a high-

intensity signal produced by the surrounding pericardial and

epicardial fat [23]. Further options, such as measurement of

atrial and ventricular size, the possibility of assessing thediastolic filling pattern, visualization of a dilated vena cava

inferior, the presence of hepatomegaly and ascites, are of

additional diagnostic value. Moreover, cardiac MRI allows

differentiation between transudate and exudate. While

transudate generates a low signal on T1-weighted images

and a high signal on T2-weighted and gradient-echo images,

exudate emits an intermediate signal on both sequences. In

cases of hemorrhagic effusion, different signal intensities on

spin-echo images may be observed, depending on the

effusion duration [23]. It is notable that in chronic

constriction, the thickened pericardium shows lower signal

intensity than in acute cases [24]. It should be mentioned that

pericardial thickening detected on MRI examination is notnecessarily diagnostic for constrictive pericarditis [21]. On

the other hand, normal pericardial thickness does not rule out

the diagnosis of constrictive pericarditis.

5.6. Differential diagnosis

Constrictive pericarditis should be suspected in patients with

clinical features of right-side heart failure, such as systemic

edema, pleural effusion, ascites, and increased jugular venous

pressure without signs of ventricular dysfunction. Other cardiac

diseases, in particular right atrial myxoma, tricuspid valve

dysfunction, and restrictive cardiomyopathy, must be ruled out[6]. Nephrotic syndrome, obstruction of the superior vena cava,

hepatic diseases, and abdominal malignancies should be

considered in the differential diagnosis. A previous medical

history of pericarditis, open-heart surgery, chest trauma, and

radiotherapy suggest the existence of pericardial constriction.

Preceding radiotherapy can be one of the reasons for both

constrictive pericarditis and restrictive cardiomyopathy. The

differentiation between these two conditions may present certain

difficulties, a subject thoroughly reviewed by Hancock [25].

Restrictive cardiomyopathy may develop in patients with

amyloidosis, Gaucher's or other storage diseases, and endo-

myocardial fibrosis. Sarcoidosis may be complicated with heartFig. 2. CT image of the heart of a patient with constrictive pericarditis,

showing a thickened pericardium.



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restriction but rarely with constrictive pericarditis. On auscul-

tation, heart restriction usually presents with sounds compatible

with mitral and tricuspid regurgitation. In addition, low-pitched,

late S3 and occasionally S4 are indicative of restrictive

cardiomyopathy. In cases with heart restriction and impaired

myocardial relaxation, the early mitral annular velocity and

propagation of initial ventricular inflow are decreased [18,25].Since heart catheterization cannot always distinguish between

constrictive pericarditis and restrictive cardiomyopathy, the

differential diagnosis may be problematic [25,26]. In restrictive

cardiomyopathy, the equilibrium of the end-diastolic pressure

usually exceeds 5 mm Hg. Endomyocardial biopsy is indicated

in difficult cases of restrictive cardiomyopathy. On rare

occasions, there is a need for explorative thoracotomy to

establish the correct diagnosis [6]. Additional examinations,

such as X-rays, CT, MRI, and echocardiography, are important

for the definitive diagnosis of constrictive pericarditis [27].

5.7. Treatment

The treatment of constrictive pericarditis consists of the

administration of diuretics, salt restriction, and supportive

therapy for underlying conditions. Since sinus tachycardia

often appears as a compensatory process, careful adminis-

tration of negative chronotropic drugs, such as beta- and

calcium-channel blockers, may be considered. In patients

with atrial fibrillation and rapid ventricular response, the

recommended drug of choice is digoxin, taking care that the

patient's heart rate remains between 80 and 90 beats per

minute [20]. In patients with transient constrictive pericar-

ditis, anti-inflammatory agents or steroids are indicated [28],

given that in 15% of cases tuberculosis might be theunderlying disease [29]. The use of steroids in the prevention

of constriction is one of the controversial issues in the

treatment of tuberculous pericarditis. Chowdhury et al. [30]

recommend prolonged anti-tuberculosis treatment for tuber-

culous pericarditis and stress that steroid administration is

not always able to prevent pericardial constriction.

5.8. Surgical treatment

In advanced cases, pericardiectomy is the definitive

treatment [13,31], and it is recommended for most patients

with a central venous pressure between 12 and 15 mm Hg.Higher pressures and liver dysfunction as a result of passive

congestion are indications for urgent surgery [10]. It has

been reported that patients' survival following pericardiect-

omy is higher than that of individuals with chronic cons-

trictive pericarditis without surgery [29]. However, choosing

the right time for surgical intervention is an important issue:

the presence of early constriction and advanced disease with

myocardial damage, as well as signs of severe heart failure,

limit the benefits of the operation and may present a serious

surgical risk. The clinical course of constrictive pericarditis

is usually progressive, and deciding the degree of pericardial

constriction and myocardial restriction is not an easy task for

the cardiologist. However, this distinction is important

since, if myocardial involvement dominates, pericardiect-

omy is not indicated. In such cases, a useful rule is: the more

significant respiratory flow variations expressed on echo-

cardiography or heart catheterization, the more likely a

constrictive dominance is present, and the better the surgical

prognosis is.

5.9. Rare forms of constrictive pericarditis

In addition to classical chronic (rigid shell, calcific) and

subacute (elastic) forms, new presentations, such as effusive-

constrictive, localized, transient, occult, and constrictive peri-

carditis with normal pericardial thickness, have been described

[3,28,3236]. Effusive-constrictive pericarditis is a variant of

pericardial constriction with a presence of large pericardial

effusion. In 1968, Spodick and Kulmar[2] described a form of

effusive-constrictive pericarditis that presents as coexisting

tamponade and heart constriction. Three years later, Hancockcharacterized the hemodynamic alterations observed in this

entity. According to Sagrista-Sauleda et al. [32], it develops

as cardiac tamponade, evolving into constriction and failure

of the right atrialpressure todecrease by 50% or more toa level

below 10 mm Hg after removal of the pericardial fluid.

According to the authors, the presenting signs are right heart

failure, distention of the jugular veins and liver enlargement,

the presence of pulsus paradoxus in 66% of the patients,

and lack of pericardial calcification. The etiology in this series

was idiopathic, postsurgical, post-irradiation, or the presence

of neoplasms. The pericardial effusion may be treated

conservatively or by applying invasive procedures such as

pericardiocentesis, balloon pericardiostomy, thoracoscopicpericardiostomy, or even pericardiectomy [33]. The peculiarity

of this variant is the involvement of visceral pericardium and

the need for its removal. In another report, Sagrista-Sauleda

et al. [34] described an entity defined as transient constrictive

pericarditis. Their patients showed signs of effusive acute

idiopathic pericarditis with cardiac constriction that disap-

peared spontaneously in less than 3 months after resolution of

the pericardial effusion. This form of constrictive pericarditis

may appear after pericardiotomy, viral and bacterial infections,

collagen vascular diseases, or idiopathic pericarditis [28,34].

Transient constrictive pericarditis occurred in 10 out of 11

Korean patients with tuberculosis in whom clinical improve-ment was observed after 2 months of anti-tuberculosis treat-

ment[29]. Occult constrictive pericarditis [35] is another rare

form of pericardial constriction presenting with non-specific

complaints, such as dyspnea and fatigue. In these patients,

rapid fluid administration may provoke clinical and hemo-

dynamic features of heart constriction. However, this is a

poorly standardized test, difficult to interpret and, therefore, of

limited use [36].

In summary, thediagnosis of constrictive pericarditis should

be seriously considered in patients with signs of chronic right-

side heart failure with exertional dyspnea, lower limb edema,

and mild liver congestion in the presence of normal ventricular



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contraction. The correct diagnosis should be established before

the development of ascites or anasarca which, progressing to

muscle wasting and fatigue, indicate the end stage of cons-

triction. For theexperienced physician, the finding of distended

jugular veins, pulsatile hepatomegaly, pericardial knock, pul-

sus paradoxus,or the presence of Kussmaul sign canbe of great

diagnostic help. However, the use of additional diagnostictools, such as echocardiography and heart visualization, is

important and may save patients anguish and complications. It

should be remembered that the sooner the diagnosis is

established, the better the outcome is. In other words, the

heart, in order to keep its secrets, does not have to be armored.

6. Learning points

1. Constrictive pericarditis should be included in the

differential diagnosis of right-side heart failure.

2. The most frequent causes for the illness in developed

countries are prior cardiac surgery, irradiation therapy,and idiopathic pericarditis, while tuberculosis remains the

main etiology of the disease in developing countries.

3. Diagnostic approach.Major clinical findings: The classical

presentation consists of symptoms and signs of chronic

right-side heart failure. On physical examination, the most

important clinical signs are increased jugular venous

pressure, pulsus paradoxus, and pericardial knock. Addi-

tional examinations: Two-dimensional echocardiography

shows a thickened pericardium, unusual motion of the

interventricular septum (septal bounce), accompanied by an

inspiratory septal shift to the left, and a dilated, non-

collapsing inferior caval vein. Doppler echocardiography

reveals increased respiratory flow variation through theheart valves. On heart catheterization,there is a nearly equal

increase in end-diastolic pressure in the four chambers. CT

and MRI allow visualization of the pericardial thickness.

4. Treatment includes the administration of diuretics, salt

restriction, and supportive therapy for underlying condi-

tions, plus suitable drug administration in cases of

tuberculous pericarditis. In advanced cases, pericardiect-

omy is the definitive treatment and it is recommended for

patients with a central venous pressure between 12 and

15 mm Hg.

Acknowledgements

We would like to thank Prof. M. Djaldetti for his help in

preparing the manuscript.

References

[1] Wang A, Bashore TM. Undercover and overlooked. N Engl J Med

2004;351:10149.

[2] Myers RBH, Spodick DH. Constrictive pericarditis: clinical and

pathophysiologic characteristics. Am Heart J 1999;138:21932.

[3] Talreja DR, Edwards WD, Danielson GK, et al. Constrictive pericarditis

in 26 patients with histologically normal pericardial thickness.

Circulation 2003;108:18527.

[4] Hancock EW. On the elastic and rigid forms of constrictive pericarditis.

Am Heart J 1980;100:91723.

[5] Nishimura RA. Constrictive pericarditis in the modern era: the diag-

nostic dilemma. Heart 2001;86:61923.

[6] Lorell BH. Pericardial diseases. In: Braunwald E, editor. Heart disease.

Philadelphia: W.B. Saunders Company; 1997. p. 1496505.

[7] Bilchick KC, Wise RA. Paradoxical physical findings described by

Kussmaul: pulsus paradoxus and Kussmaul's sign. Lancet 2002;359:19402.

[8] Khasnis A, Lokhandwala Y. Clinical signs in medicine: pulsus

paradoxus. J Postgrad Med 2002;48:469.

[9] Shabetai R, Fowler NO, Fenton JC, Masangkay M. Pulsus

paradoxus. J Clin Invest 1965;44:188298.

[10] Shabetai R. The pericardium. Boston: Kluwer Academic; 2003.

p. 191251.

[11] Oh KY, Shimizu M, Edwards WE, Tazelaar HD, Danielson GK.

Surgical pathology of the parietal pericardium: a study of 344 cases

(19931999). Cardiovasc Pathol 2001;10:15768.

[12] Bertog SC, Thambidorai SK, Parakh K, et al. Constrictive pericarditis:

etiology and cause-specific survival after pericardiectomy. J Am Coll

Cardiol 2004;43:144552.

[13] Ling LH, Oh JK, Schaff HV, et al. Constrictive pericarditis in the

modern era. Evolving clinical spectrum and impact on outcome afterpericardiectomy. Circulation 1999;100:13806.

[14] Seifert F.C., Miller D.C., Oesterle S.N., et al. Surgical treatment of

constrictive pericarditis: analysis of outcome and diagnostic error.

Circulation 1985; 72(3 Pt 2): II 26473.

[15] Bergman M, Sahar G, Vitrai J, Salman H. Early development of severe

constrictive pericarditis after coronary bypass grafting. Eur J Emerg

Med 2005;12:2457.

[16] Lipsanen-Nyman M, Perheentupa J, Rapola J, Sovijarvi A, Kupari M.

Mulibrey heart disease. Clinical manifestations, long-term course and

results of pericardiectomy in a series of 49 patients born before 1985.

Circulation 2003;107:28105.

[17] Alpert MA, Ravenscraft MD. Pericardial involvement in end-stage

renal disease. Am J Med Sci 2003;325:22836.

[18] Kussmaul A, Stern M. Pericarditis and the paradox pulse, vol. 38. Berl

Klin Wochenschr; 1873.[19] Troughton RW, Asher CR, Klein AL. Pericarditis. Lancet 2004;363:

71727.

[20] Kabbani S. Pericardial diseases. In: Zipes D, editor. Braunwald's heart

disease: a textbook of cardiovascular medicine. Elsevier W.B.

Saunders Company; 2004. p. 175779.

[21] Breen JF. Imaging of the pericardium. J Thorac Imaging 2001;16:

4754.

[22] Ling LH, Oh JK, Breen JF, et al. Calcific constrictive pericarditis: is it

still with us? Ann intern Med 2000;132:44450.

[23] Constantine G, Shan K, Flamm SD, Sivananthan MU. Role of MRI in

clinical cardiology. Lancet 2004;363:216271.

[24] Pohost GM, Hung L, Doyle M. Clinical use of cardiovascular magnetic

resonance. Circulation 2003;108:64753.

[25] Hancock EW. Differential diagnosis of restrictive cardiomyopathy and

constrictive pericarditis. Heart 2001;86:3439.[26] Vaitkus PT, Kussmaul WG. Constrictive pericarditis versus restrictive

cardiomyopathy: a reappraisal and update of diagnostic criteria. Am

Heart J 1991;122:143141.

[27] Masui T, Finck S, Higgins CB. Constrictive pericarditis and restrictive

cardiomyopathy: evaluation with MR imaging. Radiology 1992;182:

36973.

[28] Haley JH, Tajik AJ, Danielson GK, Schaff HV, Mulvagh SL, Oh JK.

Transient constrictive pericarditis: causes andnatural history. J Am Coll

Cardiol 2004;43: 2715.

[29] Yang HS, Song J-K, Song J-M, et al. Clinical characteristics of

constrictive pericarditis diagnosed by echo-Doppler technique in

Korea. J Korean Med Sci 2001;16:55866.

[30] Chowdhury UK, Subramaniam GK, Kumar AS, et al. Pericardiectomy

for constrictive pericarditis: a clinical, echocardiographic, and



8/8

hemodynamic evaluation of two surgical techniques. Ann Thorac Surg

2006;81:5229.

[31] Maisch B, Seferovic PM, Ristic AD, et al. 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. Eur Heart J 2004;25:587610.

[32] Sagrista-Sauleda J, Angel J, Sanchez A, Permanyer-Miralda G, Soler-

Soler J. Effusive-constrictive pericarditis. N Engl J Med 2004;350:46975.

[33] Hancock EW. A clearer view of effusive-constrictive pericarditis.

N Engl J Med 2004;350:4357.

[34] 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;59:9616.

[35] Bush CA, Stang JM, Wooley CF, Kilman JW. Occult constrictive

pericardial disease. Diagnosis by rapid volume expansion and correction

by pericardiectomy. Circulation 1977;56:92430.

[36] Sagrista-Sauleda J. Pericardial constriction: uncommon patterns. Heart2004;90:2578.


Constrictive Pericarditis- A Reminder of a Not So Rare Disease

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