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CARDIOLOGY BOARD REVIEW MANUAL
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Constrictive Pericarditis:
A Case Study
Debra Dreger
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ASSISTANT EDITOR
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Assistant 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, MD
Cardiology 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, PhD
Assistant Professor of Medicine, Division of Cardiology,
Emory University School of Medicine, Atlanta, GA;
Staff Cardiologist, Atlanta Veterans Affairs Medical Center, Decatur, GA
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Table of Contents
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Introduction. . . . . . . . . . . . . . . . . . . . . . . . . . . . 2
Clinical and Physical Findings . . . . . . . . . . . . . . 3
Physiology and Pathology. . . . . . . . . . . . . . . . . . 5
Diagnostic Imaging Modalities . . . . . . . . . . . . . . 5
Hemodynamics . . . . . . . . . . . . . . . . . . . . . . . . . 7
Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 7
Summary Points . . . . . . . . . . . . . . . . . . . . . . . . 10
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . 11
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Cardiology Volume 9, Part 2 1
CARDIOLOGY BOARD REVIEW MANUAL
Constrictive Pericarditis: A Case Study
William J. Nicholson, MD, and Andro G. Kacharava, MD, PhD
I. INTRODUCTION
Constrictive pericarditis (CP) is a relatively rare postinflammatory disorder with different causes. The diagnosis
of CP and its distinction from restrictive cardiomyopathy
(RCM) remain notoriously difficult. Patients with both
conditions present with increased left-sided and rightsided filling pressures, and their symptoms may resemble
congestive heart failure (CHF). It is paramount to diagnose and distinguish these 2 entities because surgical
pericardiectomy is potentially curative in CP.
Constrictive pericarditis is a condition in which the pericardial sac undergoes a process of progressive thickening and fibrosis; the natural elasticity of the pericardial
sac is lost, subsequently placing the heart in a nonpliable and rigid encasement, impairing diastolic filling
of the ventricles. Restrictive cardiomyopathy refers to idiopathic or systemic disorders involving the myocardium
characterized by restrictive diastolic filling of the ventricles. Many clinical and hemodynamic findings are common to both CP and RCM, making the 2 disorders difficult to distinguish. The purpose of this review is to
discuss the clinical presentation of CP and to focus on
the hemodynamic, echocardiographic, and radiologic
findings differentiating it from RCM. Although the diagnosis can usually be achieved via these noninvasive and
invasive studies, surgical exploration or biopsy occasionally is needed to make definitive diagnosis. A case patient is presented to illustrate the management of CP.
ANATOMICAL CONSIDERATIONS
The pericardium is an elastic, closed fibroserous sac
surrounding the heart. It consists of 2 layers of tissue: an
external (fibrous) and an internal (serous) layer. The
internal or serous layer is made up of both visceral and
parietal pericardium. The visceral layer is a thin monocellular layer adherent to the heart’s epicardium. The
parietal pericardium is adjacent to the visceral pericardium on one side and becomes tightly opposed to
the external fibrous layer on the other side. The space
created between the visceral and parietal layers of the
pericardium contains approximately 30 to 50 mL of
serous fluid, which acts as a lubricant to minimize fric-
2 Hospital Physician Board Review Manual
tion between the 2 layers of pericardium during the
heart’s movement throughout the cardiac cycle.1 This
fluid-filled space defines the pericardial cavity. The
fibrous pericardium envelops the entire heart and extends over approximately 3 cm of the great vessels
where it then attaches. Therefore, much of the ascending aorta, the main pulmonary artery, all 4 pulmonary
veins, and portions of the inferior and superior venae
cavae are contained within the pericardium.2
Fibrosis and scarring can affect each of the layers of
the pericardium either separately or simultaneously.
Development of adhesions and calcifications between
the layers can lead to obliteration of the pericardial cavity, creating a rigid inelastic “shell” around the heart
with resultant pathophysiologic consequences (ie, CP).
As the pericardial cavity is obliterated in patients with
CP, even the physiologic amount of pericardial fluid
may disappear; however, excessive amounts of effusive
fluid may be present in some patients. As this fluid is
subjected to the constricting effects of the scarred pericardium, increased pressure can result in cardiac compression or tamponade with resultant hemodynamic
deterioration; this entity is known as effusive CP.3,4 In
such cases, the constrictive hemodynamics are masked
by tamponade and may only become obvious after the
pericardial fluid is drained by pericardiocentesis.
ETIOLOGIES
Many different conditions can produce acute pericarditis, and nearly all are capable of inducing constriction, albeit some more frequently than others (Table 1).5
The notable exception is acute rheumatic fever, which
can produce extremely dense pericardial adhesions that
rarely 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 and
remains a dominant cause in developing countries. Neoplasm, collagen vascular disease, infectious etiologies,
radiation therapy, and previous cardiac surgery are some
of the more common causes of constriction in modern
developed countries.7 However, in numerous cases (even
after microscopic and culture examination of pericardial
scar tissue), an inciting etiology is not found. In fact, idiopathic or presumed viral etiologies are now the leading
Constrictive Pericarditis: A Case Study
cause of CP in developed countries, composing up to
42% of cases in some series.8 Nevertheless, a thorough
search for tuberculosis (ie, evidence of granulomatous
lesions) must be made before concluding that the pericardial disease is of unknown origin.9
RCM is the least common of the cardiomyopathic
disorders.10 RCM may result from various primary localized cardiac processes or may be secondary to systemic
infiltrative or storage diseases.11 Cardiac amyloidosis is
the most common cause of RCM in developed countries, whereas endomyocardial fibrosis is endemic in
parts of Africa, India, Asia as well as South and Central
America.12 Although restrictive disease can arise from
systemic disorders that are rarely encountered in clinical practice, conditions such as amyloidosis, sarcoidosis,
and hemochromatosis are more common. Idiopathic
RCM is an uncommon disorder manifested by the characteristic hemodynamic abnormalities of restriction in
the absence of any identifiable cause. Because restrictive physiology is seen in patients with various systemic
disorders, idiopathic RCM is a diagnosis of exclusion.
Although RCM can occur in elderly persons, it should
be differentiated from the age-related changes in diastolic compliance.13
II. CLINICAL AND PHYSICAL FINDINGS
CASE PATIENT 1 PRESENTATION
Patient 1 is a 52-year-old woman who presents to the
general medical clinic complaining of 6 months of dyspnea. The patient has gained approximately 25 lb during the preceding several months, with increased abdominal girth and discomfort as well as marked lower
extremity swelling. Before her present complaints, she
had been in good health and was taking no medications. Her last medical visit was 15 years earlier when
she had a cholecystectomy. Initial physical examination
reveals a frail cachectic woman with pitting lower extremity edema, hepatomegaly, and tense ascites. Breath
sounds are diminished at the bases bilaterally. Cardiovascular examination is remarkable for a resting tachycardia, jugular venous distention (JVD), and an early
diastolic sound thought to be an S3. Liver transaminase
levels are elevated, and the patient’s albumin is slightly
reduced. A paracentesis is performed, which reveals a
transudate effusion.
Radiologic evaluation of the chest and abdomen as
well as colonoscopy for potentially malignant etiologies
of the prominent ascites and liver disease are both unrevealing. However, a computed tomography (CT) scan
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 dermatomyositis.
Adapted from Myers RB, Spodick DH. Constrictive pericarditis: clinical and pathophysiologic characteristics. Am Heart J 1999;138(2 Pt 1):
219–3, with permission from Elsevier Science.
of the chest shows mild thickening of the pericardium.
Liver biopsy shows congestion and nonspecific cirrhosis
without evidence of infiltrative disease. Subsequent
echocardiographic evaluation and cardiac catheterization are consistent with the diagnosis of CP and help in
differentiating it from RCM.
• What symptoms and signs are frequently associated
with CP?
• Can these findings reliably differentiate CP from
RCM?
DISCUSSION
The clinical presentation and physical findings are
similar in CP and RCM.5,14 Symptoms are typically insidious in onset and are closely related to the degree of systemic and central venous congestion as well as the degree
of fluid retention. Poor appetite, cachexia, abdominal
discomfort resulting from splanchnic engorgement,
Cardiology Volume 9, Part 2 3
Constrictive Pericarditis: A Case Study
A
C
A
central venous pressure, particularly if there is a history
of a predisposing condition. A clinical history of pericarditis, tuberculosis, trauma, or radiation therapy
makes a diagnosis of CP more likely, whereas a history
of a predisposing systemic disease (such as amyloidosis
or sarcoidosis) would favor RCM.
X
V
Normal
X′
A
Y
V
X
X′
B
Y
Constrictive pericarditis
Figure 1. Jugular venous pulsation. (A) Normal jugular venous
pulsation. (B) Jugular venous pulsation in patients with CP. The
sharp Y-wave dip forms the characteristic “W” shape known as
the 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-Hill
Medical Publishing Division; 2001:228–9. Copyright 2001, with
permission of the McGraw-Hill Companies.)
ascites, and peripheral edema with general fatigue and
weakness can cause the clinician to inappropriately
work-up a diagnosis of liver disease. As central diastolic
pressures increase and symptoms of dyspnea, orthopnea, and occasionally platypnea become more prominent, clinicians can erroneously diagnose CHF. As seen
with patient 1, failure to recognize the significance of
elevations in jugular venous pressure (JVP) in a patient
presenting with clear lungs, unremarkable chest radiograph, hepatomegaly, and ascites may lead the clinician
to pursue liver biopsy before the correct diagnosis is recognized.15
CP and RCM may present predominantly with symptoms of systemic or pulmonary venous congestion depending on the extent and area of pericardial scarring
or on the restrictive involvement of the myocardium.
Thus, it is difficult to establish the diagnosis on clinical
grounds alone. Symptoms can develop from weeks to
decades after an inciting event (which the patient often
does not recall), making the diagnosis less obvious. Several studies have shown that the average duration of
symptoms before diagnosis was nearly 2 years.16,17 For
this reason, constriction and restriction should be considered in any patient with unexplained elevation in
4 Hospital Physician Board Review Manual
Neck Vein Examination
Examination of the neck veins is essential when considering the diagnosis of CP. Increased JVP has been
reported in 93% of patients with CP18 and is frequently
encountered in RCM. Kussmaul’s sign, or inspiratory
JVD, replaces the normal inspiratory venous collapse of
the jugular veins in patients with CP. Although usually
present in CP, Kussmaul’s sign is not specific; it is frequently present in RCM and can be observed in tricuspid
stenosis, right heart failure, or systemic venous congestion of any cause. Figure 1 demonstrates the normal
jugular venous pulsation contrasted with that seen in patients 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 right
atrial pressure transmitted to the jugular veins during
right atrial systole, and atrial relaxation results in the
descent of the A-wave. The C-wave occurs during the
beginning of right ventricular systole, resulting from
the 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 in
systole and followed by X′-wave descent, which represents systolic down movement of the floor of the right
atrium caused by the right ventricular contraction. The
V-wave occurs after the X′-wave descent as a result of an
increase in right atrial pressure and JVP caused by continued inflow of blood to the venous system during late
ventricular systole when the tricuspid valve is still closed.
The Y-wave descent, or diastolic collapse, is produced by
opening of the tricuspid valve and the rapid inflow of
blood into the right ventricle. A sharp Y-wave descent
and a rapid ascent to the baseline (referred to as the
Friedreich’s sign) are seen in CP (Figure 1B). The
venous pressure is elevated in these conditions with a
sharp Y-wave dip in the jugular venous pulsation forming a characteristic “W” shape known as the W-wave of
Bloomfield.
Cardiac Examination
Cardiac examination can be helpful in diagnosing
CP, differentiating it from RCM, and excluding other
potential etiologies. In RCM, the heart size is preserved
and the ventricular systolic impulse is usually normal or
Constrictive Pericarditis: A Case Study
enhanced; heart size is either normal or smaller in patients with CP. The non-pliable and rigid encasement of
the heart in CP results in precordial palpation that is
often quiet without an appreciable ventricular impulse.
An S3 is frequently heard in RCM. The abruptly amputated ventricular filling during diastole is caused by constriction from the rigid pericardium and results in a pericardial knock in CP. The knock usually occurs 0.06 to
0.12 seconds after the S2 sound and may be made more
prominent by having the patient squat, which increases
afterload.20 In patient 1, pericardial knock was interpreted as an S3, evidence of certain degree of CHF, which
together with liver cirrhosis were considered the most
likely initial diagnoses. Significant pulsus paradoxus of
greater than 10 mm Hg is infrequently encountered in
pure CP or in RCM. The constricting pericardium does
not transmit changes in pleural pressures (see next section); therefore, the presence of pulsus paradoxus suggests coexistent pulmonary disease or effusive CP.21
III. PHYSIOLOGY AND PATHOLOGY
The rigid, non-pliable shell that encases the heart in
CP sharply accentuates the ventricular pressure-volume
relationship through several mechanisms. First, intrathoracic and intracardiac pressures are dissociated during respiration. The effect of the respiratory cycle on intrathoracic blood flow in CP is critical to understanding the
pathophysiologic features.22,23 Normally, inspiration decreases intrathoracic pressure, which is transmitted to all
intrathoracic structures including the pulmonary veins
and cardiac chambers. However, the encased pericardium of CP isolates the cardiac chambers from changes in
intrathoracic pressure. Therefore, as intrathoracic pressures decrease during inspiration, cardiac pressures remain high. Less of a pressure gradient is created between
the pulmonary veins and the left-sided chambers of the
heart, resulting in less total pulmonary venous return and
decreased flow velocity during inspiration. This results in
a decrease in left ventricular filling with inspiration. The
opposite effect is seen with expiration in CP.
The second physiologic hallmark of CP results from
marked ventricular interdependence. The total cardiac
volume is fixed by the non-pliable pericardium. Therefore, the total amount of blood entering the heart does
not vary significantly during the respiratory cycle. During
expiration, the increase in the intrathoracic pressure
does not affect the cardiac chambers. However, the pulmonary veins and venae cavae are not shielded by the
rigid pericardium, and flow velocities in these vessels are
affected. Consequently, flow into the left ventricle with
expiration is increased. The ventricular septum is not
directly affected by the pericardium and is free to bulge
into the right ventricle causing reduction in flow velocity
in the venae cavae and decreased trans-tricuspid flow
velocity.24 Echocardiography demonstrates these changes
in flow velocities, as discussed in the next section.
The third major effect of the inflexible pericardium of
CP on cardiac hemodynamics is impairment of diastolic
filling. The non-pliable pericardium of CP limits diastolic
filling in all cardiac chambers, resulting in elevated enddiastolic pressures. Normally, approximately 75% of ventricular filling occurs during phase 2 (rapid filling) of
diastole, and 10% to 20% occurs during phase 4 (atrial
contraction).15 Because of the elevated atrial pressures in
CP, up to 75% to 80% of ventricular filling occurs in the
first 25% to 30% of diastole.25 Filling rapidly declines by
mid-diastole and filling is severely limited in late diastole.
Tachycardia would adversely affect diastolic ventricular
filling in a normal patient. In patients with CP, where
nearly all ventricular filling occurs by mid-diastole, tachycardia becomes an important means of maintaining cardiac output. Consequently, tachycardia and atrial fibrillation are relatively less important in CP.26
The hallmark of chronic CP is a thickened adherent
pericardial sac that restricts filling of the cardiac chambers. Pathologically, pericardial inflammation leads to
the deposition of fibrous strands and proliferation of cellular infiltrates.27 Eventually, calcification occurs with
total or near total obliteration of the pericardial space as
well as the remaining fluid, pus, or blood. In some instances, loculated large collections of fluid may compress
individual cardiac chambers. Additionally, bandlike constrictions can develop and compromise any portion of
the 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 radiography
assist diagnosis of CP?
• What additional studies are necessary to accurately
diagnose CP?
No specific ECG abnormalities are characteristic for
either CP or RCM. Low QRS voltage and nonspecific
T-wave changes are common in both conditions. A
compensatory sinus tachycardia is frequently present in
RCM and CP, as seen in patient 1. Atrial fibrillation may
be present in CP (up to 10% of patients) and RCM;
Cardiology Volume 9, Part 2 5
Constrictive Pericarditis: A Case Study
RV
RA
LV
RV
LV
LA
A
B
Figure 2. MRI of the pericardium in a patient with constrictive pericarditis. Progressive cardiac failure developed during a viral illness in a
76-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 between
the 2 pericardial layers, and increased signal in the cavity, indicative of stasis of blood. LA = left atrium; LV = left ventricle; MRI = magnetic
resonance imaging; RA = right atrial; RV = right ventricle. (Reprinted with permission from De Benedetti E, Didier D. Images in clinical medicine. Constrictive pericarditis. N Engl J Med 2000;343:107. Copyright © 2000 Massachusetts Medical Society. All rights reserved.)
however, ventricular arrhythmias and abnormalities of
conduction, including atrioventricular block, are seen
more frequently in RCM. Because these findings are
nonspecific, it is not surprising that an ECG tracing
rarely can help to differentiate between RCM and CP.
However, the presence of a completely normal tracing
should lead the clinician to reconsider the diagnosis of
CP or RCM.
RADIOLOGIC IMAGING
Chest Radiography
Chest radiography as a single noninvasive imaging
modality can occasionally help to distinguish CP from
RCM. Pericardial calcification is best seen in the lateral
view. “Eggshell” calcifications or amorphous calcifications in the atrioventricular grooves may be seen. Although calcification may support the clinical suspicion
of CP, its presence is not always specific because it may
occur without cardiac compression.30
Blood Pool Radionuclide Ventriculography
Blood pool radionuclide ventriculography usually
demonstrates that the elapsed time to peak filling is decreased during inspiration, which may be a finding
unique to CP.31,32
Computed Tomography and Magnetic Resonance
Imaging (MRI)
CT and MRI are the best radiologic modalities to
demonstrate pericardial thickening. When imaging with
multislice spin-echo (gated) MRI, the normal pericardium appears as a thin (< 3 mm), low-intensity signal
between the high-intensity signal of the epicardial fat
6 Hospital Physician Board Review Manual
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 to
distinguish fluid from pericardium and to accurately
assess the pericardial thickness. Pericardial thickening of
4 mm or more favors a diagnosis of CP, whereas thickening more than 6 mm is highly specific for CP (Figure 2).34
It should be mentioned that the pericardium can be constricting even when it is so thin that it is not recognized as
abnormal by any contemporary imaging techniques.
Nevertheless, pericardial thickening of more than 5 mm,
an enlarged right atrium, and a distorted tubular right
ventricle are all highly predictive of pericardial constricting disease.34 In some cases of heavy pericardial calcification, distinguishing between calcification and fibrous tissue may be difficult with MRI. In these cases, CT imaging
of the pericardium may be better to assess pericardial
thickness.22,35,36 Besides assisting in the diagnosis of CP,
CT and MRI permit detailed planning of the surgical approach in patients by revealing the distribution and variations of pericardial thickness, calcification, and possible
myocardial invasion.
ECHOCARDIOGRAPHY
No single pathognomic echocardiographic finding
exists for CP. To distinguish CP from RCM, a combination of findings need to be used. Both M-mode and
2-dimensional echocardiography are not very helpful
in making the distinction between these 2 conditions.
In severe cases, the increased thickness and calcification of the pericardium can be seen with M-mode and
2-dimensional evaluation. Although evaluation of pericardial thickness with transthoracic echocardiography
Constrictive Pericarditis: A Case Study
is insensitive, measurement with a transesophageal
echocardiogram (TEE) correlates strongly with measurements obtained by CT. One study demonstrated
that the superior resolution achieved with TEE allowed
better pericardial definition; a thickness of 3 mm or
more on TEE was 95% sensitive and 86% specific for
the detection of thickened pericardium.37
Doppler echocardiography and the relatively newer
methods of color M-mode and tissue Doppler studies
can differentiate CP from RCM. As previously mentioned, an important pathophysiologic consequence of
CP is the greatly enhanced interventricular dependence resulting from a limited total cardiac volume with
an uninvolved, freely moving septum. Thus, ventricular
filling and emptying velocities assessed by mitral and tricuspid inflow velocities have much greater respiratory
variation in CP in contrast to RCM where the interventricular septum is involved by the pathological process.
Patients with CP have greatly exaggerated reciprocal
variations in the rates of left and right ventricular filling
related to the respiratory cycle when compared with
normal subjects.38,39 In most patients with CP, mitral
inflow velocity decreases as much as 25% and tricuspid
velocity greatly increases with the first beat after inspiration. In contrast, respiratory variation in patients with
RCM does not significantly differ from the normal.
Doppler interrogation of the pulmonary and hepatic
venous flow can further assist in the differentiation of
these 2 conditions.40 These respiratory variations are illustrated in Figure 3.
New Doppler methods have revealed that color Doppler M-mode evaluation of mitral inflow can help distinguish between RCM and CP. The flow propagation slope
is steep in CP, whereas the same parameter is significantly lower in RCM (Figure 4).41 Additionally, tissue Doppler
imaging has shown that patients with CP have a normal
or a high normal early diastolic velocity (> 8 cm/sec),
whereas patients with RCM have low early diastolic velocities (< 8 cm/sec; Figure 5).42 Doppler echocardiography
has proven to be one of the most reliable noninvasive
methods to help distinguish CP from RCM.
tive process. Finally, catheterization provides information regarding the coronary anatomy in a patient who
will undergo pericardiectomy and may need concomitant coronary artery bypass graft surgery.
Although cardiac catheterization does not necessarily confirm the diagnosis of CP, Vaitkus and Kussmaul’s
showed that the overall predictive accuracy of 3 major
hemodynamic criteria can be very specific.43 A difference between right ventricular end diastolic (RVEDP)
pressure and left ventricular end diastolic pressure
(LVEDP) of 5 mm Hg or less, a right ventricular systolic
pressure (RVSP) of 50 mm Hg or less, and a ratio of
RVEDP to RVSP of 1:3 or more are 85%, 70%, and 76%
sensitive, respectively, for correctly diagnosing CP. If all
3 of these criteria are met, a diagnosis of CP is correct
in greater than 90% of patients.
As previously discussed, the tight ventricular interaction in conjunction with insulation of the cardiac
chambers from variations of intrathoracic pressures
during the respiratory cycle are the 2 key mechanisms
underlying the pathophysiology of CP. These fundamental principles explain why measuring respiratory
variation in ventricular hemodynamics during catheterization is paramount in the diagnosis of CP.44
As a manifestation of ventricular interdependence,
respiratory discordance occurs between the peak RVSP
and left ventricular systolic pressure (LVSP) in CP. RVSP
increases with inspiration as LVSP simultaneously decreases. The pulmonary capillary wedge pressure declines more than the LVEDP during inspiration, and the
reduction in mitral valve inflow translates into the observed reduction in LVSP. In RCM, both the RVSP and
LVSP decrease concordantly with inspiration.44
Several other findings on cardiac catheterization can
assist in the diagnosis of CP such as increased distance
between coronary arteries and the cardiac silhouette,
which suggest the presence of thickened pericardium, as
well as tethering of coronary arteries to the pericardium.
V. HEMODYNAMICS
• What is the definite therapy for CP?
• What are the possible complications of complete
pericardiectomy?
Despite the availability of several noninvasive methods of assessment, cardiac catheterization is valuable
when evaluating a patient with suspected RCM or CP.
Catheterization permits quantification of left and right
heart pressures. Additionally, endomyocardial biopsy
can be performed and may identify coexisting cardiac
disease or may confirm RCM resulting from an infiltra-
VI. TREATMENT
DISCUSSION
The main reason that it is essential to distinguish
RCM from CP is that the 2 disorders have distinctly
different treatments. CP can be cured surgically with
pericardiectomy. Although constrictive physiology
Cardiology Volume 9, Part 2 7
Constrictive Pericarditis: A Case Study
ECG
E
E
E
A
S
A
D
S D
D
ECG
S
A
Apnea
Apnea
Inspiration
Inspiration
Normal
Constrictive
pericarditis
A
Expiration
Restrictive
myocardial
disease
Expiration
Normal
Constrictive
pericarditis
C
Restrictive
myocardial
disease
ECG
E A
E
ECG
E
A
A
Apnea
Apnea
S
D
D
S
S
Inspiration
Inspiration
Expiration
Normal
B
Constrictive
pericarditis
Expiration
Restrictive
myocardial
disease
Normal
D
Constrictive
pericarditis
Restrictive
myocardial
disease
Figure 3. Schematic representations of flow patterns (using Doppler echocardiography) in normal subjects, patients with constrictive
pericarditis, and patients with restrictive cardiomyopathy. A simultaneous ECG tracing is shown across the top. (A) Respiratory variation across the mitral valve. (B) Respiratory variation in flow across the tricuspid valve. Changes in flow are opposite of those seen
across 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 = early
diastolic filling; S-wave = peak systolic flow velocity. (Adapted from Leung DY, Klein AL. Restrictive cardiomyopathy: diagnosis and prognostic implications. In: Otto CM, editor. Practice of clinical echocardiography. Philadelphia: WB Saunders; 1997:474, with permission
from Elsevier Science.)
occurring in acute pericarditis may revert spontaneously,45 surgical removal of the pericardium is necessary in most cases. Complete pericardiectomy is the
operative procedure of choice; however, it is surgically
challenging.46,47 Removal should be as extensive as
possible; however, sometimes calcified islands must be
left or a palliative meshing of the pericardium may be
required to minimize damage to the coronary arteries
and myocardium. Removal of adherent pericardium
8 Hospital Physician Board Review Manual
may require a prolonged procedure complicated by
hypotension and arrhythmias. Most patients respond
well to surgery, but the reported operative mortality of
12% is significant.46 Some patients have immediate
and often profuse postoperative diuresis, although
others may take weeks to months to recover after
surgery. Poorer results are seen in patients with inadequate resection, uncorrected coronary artery disease,
higher New York Heart Association classifications, and
Constrictive Pericarditis: A Case Study
APEX
LA
A
APEX
LA
Figure 4. Color Doppler M-mode of diastolic flow from the left atrium (LA) toward
the ventricular apex as imaged from the
4-chamber view in patients with constrictive pericarditis (A) or restrictive cardiomyopathy (B). The flow propagation slope
of the first aliasing contour (white line) is
steep for constrictive pericarditis, but the
slope is significantly slower in restrictive
cardiomyopathy. Respiratory monitoring is
shown with inspiration (up arrow) and expiration (down arrow). (Reprinted from
Rajagopalan N, Garcia MJ, Rodriguez L, et
al. Comparison of new Doppler echocardiographic methods to differentiate constrictive pericardial heart disease and
restrictive cardiomyopathy. Am J Cardiol
2001;87:93, with permission from Excerpta
Medica, Inc.)
B
older age. Patients with radiation-induced CP and
those with peripheral organ failure involving the liver
or kidneys with ascites and edema also have poorer
postoperative outcomes.46
Occasionally, the chronic external support of the
tight pericardium can lead to myocardial atrophy,
which can sometimes be detected preoperatively by
decreased myocardial signals on MRI or CT. Releasing the heart surgically may allow rapid dilation of
the ventricle, leading to systolic dysfunction and heart
failure.47,48 Patients dying with low cardiac output
after complete pericardiectomy have histologic findings consistent with myocardial atrophy. When this
condition is advanced, the heart may continue to
dilate as it fails, resulting in what has been called the
exploding heart syndrome. Fortunately, the condition is reversible in some patients if they can be assisted for several days with maximal medical and circulatory support.49
On the other hand, treatment of RCM is often symptomatic. Diuretics are frequently used to relieve systemic
and pulmonary venous congestion, which must be done
with caution because high filling pressures are needed to
maintain ventricular filling in RCM. Excessive use of diuretics may reduce filling and lead to decreased cardiac
output with resultant systemic hypoperfusion manifested
by fatigue, lightheadedness, and prerenal azotemia.
Careful monitoring for these findings should alert the
clinician that further diuresis should be avoided. As discussed earlier, efficient diastolic filling is compromised in
RCM, leading to the understanding that rate-lowering
drugs (such as β-blockers and calcium channel antagonists) may have a beneficial role. Slowing the heart
thus allows for more diastolic time in each cardiac cycle
and for improved ventricular relaxation and filling.
Angiotensin–converting enzyme inhibitors have a proven beneficial role in systolic dysfunction; evidence suggests they may be beneficial in diastolic dysfunction and
thus have a role in treating RCM.50
Rhythm disturbances can be detrimental in patients
with RCM. Atrial fibrillation worsens diastolic dysfunction because of the loss of the atrial contribution to ventricular filling. Additionally, a rapid ventricular response can further compromise patients with RCM.
Attempts should be made to maintain sinus rhythm via
cardioversion or antiarrhythmic drugs such as amiodarone. If sinus rhythm cannot be achieved or maintained, then adequate rate control is necessary, and
anticoagulation with warfarin is recommended. In fact,
the hypercoagulable propensity of particular etiologies
of RCM warrant anticoagulation to prevent thrombus
formation regardless of the patient’s rhythm.51
Cardiology Volume 9, Part 2 9
Constrictive Pericarditis: A Case Study
Normal
Restriction
Constriction
A
50 cm/s
50 cm/s
20 cm/s
B
5 cm/s
5 cm/s
5 cm/s
Figure 5. A normal subject, a patient with
restrictive cardiomyopathy, and a patient
with constrictive pericarditis: representative examples of the (A) mitral annular
M-mode, (B) transmitral Doppler flow
velocities, and (C) tissue Doppler axial velocities. A marked difference in early diastolic velocity (arrows) despite similar early
transmitral flow velocities is observed.
(Reprinted from Garcia MJ, Thomas JD,
Klein AL. New Doppler echocardiographic applications for the study of diastolic
function. J Am Coll Cardiol 1998;32:871,
with permission from the American College of Cardiology Foundation.)
C
Infiltrative disorders may result in advanced conduction
system disease. Dual chamber pacing should be used to
maintain properly timed atrial contraction to improve
ventricular filling.
Advanced diastolic dysfunction secondary to RCM is
associated with a poor prognosis in patients with secondary causes such as amyloidosis or hemochromatosis.
Idiopathic RCM also has a poor outcome, with 64% and
37% survival at 5 and 10 years, respectively.52 Cardiac
transplantation can be performed in patients with
refractory symptoms in idiopathic or familial RCM.
Combined heart and liver transplantation in patients
with hemachromatosis has yielded good results.53 Although transplantation has been a treatment option for
sarcoidosis and amyloidosis, recurrence can occur in
the transplanted heart.54 – 56
DIAGNOSIS AND TREATMENT OF PATIENT 1
Thickening of the pericardium observed on CT scan
in patient 1 assisted in establishing the diagnosis of CP,
which was further supported by Doppler echocardiographic and hemodynamic data obtained during cardiac catheterization. Pericardial biopsy confirmed the
diagnosis of CP, and subsequent pericardiectomy is successful.
•
•
•
•
•
•
VII. SUMMARY POINTS
• The fibrous pericardium envelops the entire heart and
much of the ascending aorta, the main pulmonary
10 Hospital Physician Board Review Manual
•
artery, all 4 pulmonary veins, and portions of the inferior and superior venae cavae in patients with CP.
Tuberculosis has historically been the leading cause
of CP; however, neoplasm, collagen vascular disease,
infectious etiologies, radiation therapy, and previous
cardiovascular surgery are some of the more common causes of constriction in modern developed
countries.
Symptoms are typically insidious in onset and consist
of poor appetite, cachexia, abdominal discomfort
(because of splanchnic engorgement, ascites), and
peripheral edema along with general fatigue and
weakness. However, it is difficult to establish the diagnosis of CP or RCM on clinical grounds alone.
A sharp Y-wave descent and a rapid ascent to the
baseline in jugular venous pulsations are seen in CP,
RCM, or severe right-sided heart failure.
In patients with constrictive pericarditis, the rigid
pericardium causes an abruptly amputated ventricular filling during diastole, resulting in a pericardial
knock that may be made more prominent by having
the patient squat.
The rigid, inelastic “shell” that encases the heart in CP
sharply accentuates the ventricular pressure-volume
relationship through several mechanisms.
Pericardial calcification is often seen on the lateral
radiograph in CP.
MRI is very accurate in assessing pericardial thickness; thickening of 4 mm or more favors a diagnosis
of CP, whereas thickening greater than 6 mm is very
specific for CP.
Constrictive Pericarditis: A Case Study
• Ventricular filling and emptying velocities as assessed
by mitral and tricuspid inflow velocities have much
greater respiratory variation in CP when compared
with RCM.
• New Doppler methods have revealed that color Doppler M-mode evaluation of mitral inflow can help
distinguish between RCM and CP.
• Cardiac catheterization can be used to diagnose CP.
A difference between right ventricular end diastolic
pressure (RVEDP) and left ventricular end diastolic
pressure of 5 mm Hg or less, a right ventricular systolic pressure (RVSP) of 50 mm Hg or less, and a
RVEDP to RVSP ratio of 1:3 or more are consistent
with CP.
• CP can be cured surgically with pericardiectomy;
however, the reported operative mortality is 12%.
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12 Hospital Physician Board Review Manual