1. Clin. Clardiol. 18, 341-350 (1995)
Clinical Pathologic Correlations
This section edited by Bruce Wallel;M.D.
ConstrictivePericarditis:Its History and Current Status
0.
NOBLE FOWLER,
M.D.
Divisioii of Cardiology, Department of Medicine, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA
Summary: The diagnosis of constrictive pericarditis remains strictivecardiomyopathyis presented. The topic of occult con-
a challengebecause it is often mimicked by restrictive cardio- strictive pericardial disease is discussed briefly. A discussion
myopathy. The last few years have seen numerous advances in of the timing of pericardial resection for the treatment of con-
our ability to differentiatebetween these two conditionswhich strictive pericarditis ends the review.
often have similar physical findings and hemodynamics.This
review begins with a brief history of constrictive pericarditis;
this is followed by an extensivediscussion of newer etiologies, Key words: constrictive pericarditis, pericardial disease
and then the classical clinical history and physical examina-
tion findings are described. Radiologic, electrocardiographic,
and angiographic findings are discussed. The hernodynamics Introductionand HistoricEvents
of constrictive pericarditis are reviewed. Recent results of
echocadiographic and echo-Doppler investigations are pre- Constrictive pericarditis has been defined as a chronic fi-
sented. Emphasis is placed upon the limitations of M-mode brous thickening of the wall of the pericardial sac which is so
echocardiographyin the diagnosis of constrictivepericarditis. contracted so that normal diastolic filling of the heart is pre-
The value of echocardiographicDoppler studies of mitral and vented.‘
tricuspid flow velocity patterns, as well as of those in the pul- The existence of constrictive pericarditis has been known
monary veins and hepatic veins, is described. Nuclear ven- for centuries. In 1669, Richard Lower wrote of dyspnea and
triculograms and angiocardiogramstend to show more rapid intermittentpulse in a patient with constrictivepericarditi~.~In
ventricularfilling in constrictivepericarditis than in restrictive 1842, Conigan describedthe pericardial knock sound (bruit de
cardiomyopathy. Although only a small number of patients fra~pement).~ 1873, Kussmaul described the paradoxical
In
has been studied, these evaluations seem to have merit in sep- arterial pulse in mediastinopericarditi~.~ eponym “Pick’s
The
arating restrictive cardiomyopathy from constrictive peri- disease” was given to constrictivepericarditis with ascites and
carditis.The role of computedtomography scanning and mag- hepatomegaly following Pick‘s de~cription.~ first suc-
The
netic resonance imaging studies of pericardial thickness in cessful pericardiectomy in the US. was performed in 1929by
confirming the presence of constrictivepericarditis is discus- ChurchilL6The modem era of diagnosis and treatment of this
sed. Abnormal pericardial thickening (> 3 mm) confirms the disease was signaled by Paul Dudley White’s St. Cyre’s Lec-
diagnosis of constrictivepericarditis, but only if the character- ture in 1935.’ This paper described 15 patients, 7 of whom
istic hernodynamic pattern is present. The usefulness of en- were successfullyoperated upon at the MassachusettsGeneral
domyocardial biopsy in recognizing specific varieties of re- Hospital. Bloomfield7demonstrated elevated right atrial pres-
sure and elevated right ventricular (RV) diastolicpressure with
an early diastolic dip in a patient who had constrictive peri-
carditis. Hansen et aL’s paper in 1951 dealt with the RV dip
Address for reprints: and plateau pressure-pulsepattern.8 A similar pressure-pulse
Noble 0. Fowler, M.D. pattern in restrictive cardiomyopathy was described 2 years
University of Cincinnati College of Medicine lateregHancock popularized the condition known as effusive-
Medical Sciences Building constrictiveperi~arditis.’~ Constrictivepericarditis as a com-
23 1 Bethesda Avenue plication of cardiac surgery was first reported in 1972.’’ The
M.L. #542 value of computed tomography (CT) scanning in constrictive
Cincinnati, OH 45267, USA pericarditiswas discussed by Isner etal. in 1982,’*and Soulen
Received: May 17, 1994 et al. wrote of magnetic resonance imaging (MRI) studies in
Accepted: June 14, 1994 this disease in 1984.13

2. 342 Clin. Cardiol. Vol. 18. June 1995
TABLE Etiologic background of constrictive pericarditis
I be followed by acute pericarditis; clinical evidence of con-
strictive pericarditis may appear years later. Radiation therapy
1. Unknown antecedent
2. Following idiopathic pericarditis
for Hodgkin's disease may be followed by some variety of
3. Specific infections pericarditis in 20% of instances.29 Radiation therapy was a
Bacterial leading cause of constrictive pericarditis in 95 cases reported
Tuberculosis from Stanford University, in which 3 1% of instances followed
Fungal disease-e.g., histoplasmosis,coccidioidomycosis radi0thera~y.I~ the other hand, radiation therapy was re-
On
Vial diseases, especially Coxsackie B sponsible in only 5% of 313 cases studied at the Mayo
Parasitic disease: amebiasis, echinococcosis Clinic.'* Radiation therapy was responsible in 2 of 27 cases in
4. Connective tissue disease: rheumatoid arthritis, lupus erythe- another seriesI9and in none of 26 cases in another.30
matosus, scleroderma Patients with end-stage renal disease who are treated by he-
5. Neoplastic disease modialysis or renal transplantation may develop constrictive
Secondary to breast cancer, lung cancer, lymphoma, pericarditis in addition to acute pericarditis and cardiac tam-
melanoma p ~ n a d e . " ' -We have seen one case recently.
~~
Primary mesothelioma
6. Trauma Connective Tissue Disease
Nonpenetrating
Penetrating Rheumatic fever generally is considered a rare cause of
I. Post cardiac surgical procedures (incidence0.243%) constrictive pericarditis, although Roberts and Spray found it
8. Radiation therapy to be a cause in 2 of 3 14 cases.35A Mayo Clinic study found
9. End-stagerenal disease rheumatic fever to be the cause in 4 of 23 1 cases of constrictive
10 Following cardiac pacemaker insertion
~ e r i c a r d i t i sRheumatoid arthritis is a fairly common cause
.~~
11. Following certain drugs--e.g.,methysergide, procainamide-
of constrictive pericarditis or effusive-constrictive pericardi-
induced lupus syndrome
12. Hereditary: mulibrey nanism tis.37-40 Lupus erythematosus may be followed by constrictive
13. Rare: sarcoidosis, asbestosis, postmyocardial infarction, amy- pencarditis. A total of seven cases, six in men, had been re-
loidosis ported by 1988?l Rarely, constrictive pericarditis may follow
a drug-induced lupus-like syndrome."2,43
Infectious Diseases
Etiology
Many pericardial infections may be followed by constric-
Most causes of acute pericarditis may also cause chronic tive pericarditkU6 Infections caused 3% of constrictive
constrictivepericarditis (Table I). Idiopathic pericarditis head- pericarditis in cases reported in the Mayo Clinic series.'*
ed the list of known anmedents in some seriesl47l5 but the most Tuberculosis was responsible forO-8% of cases in recent stud-
common group is that with no recognized antecedent.I5-l9In ies in Western countries,17~30~36 causes the majority of cas-
but
earlier series, tuberculosis was the most commonly recogniz- es in India22and in certain areas of A f r i ~ a . 4 ~ pericarditis
Viral
able cause20,21 a 1990series fromthe Mayo Clinic, compris-
In has been identified as a cause of constrictive p e r i c a r d i t i ~ ' ~ . ~ ~ - ~ ~
ing 3 13patients operated upon since 1936,6% had tuberculo- or was suspected because the syndrome of constrictive peri-
sis.'* However, in nonindustrialized nations such as India, carditis followed an epidemic of viral disease.I4In endemic ar-
tuberculosis was found to cause 61% of 118 instances of con- eas, histoplasmosis may be a relatively common cause of peri-
strictive pericarditis.22 carditis and may be followed by constrictive p e r i ~ a r d i t i s ; ~ ~
Constrictive pericarditis may follow n~npenetrating~~ or however, a follow-up study of 10 of 16 cases of acute histo-
penetrating trauma, including cardiac surgical procedures or plasma pericarditis found no instances of constrictive peri-
the penetration of pacing catheters into the pericardial space.24 carditis 6 months to 10 years later.51Histoplasmosis may pro-
Cardiac surgical procedures are estimated to be followed by duce fibrosing mediastinitis, accompanied by both constric-
constrictive pericarditis in 0.2 to 0.3%of instances; 158 cases tive pericarditis and superior vena caval o b ~ t r u c t i o nRe- .~~
were found in the world literature in 1989.25Another report in cently reported cases of constrictive pericarditis caused by in-
the same year described personal experience with 45 cases. fections include Legionella p n e u m ~ p h i l ameningococcal
,~~
The interval between the cardiac operation and the appearance i n f e ~ t i o nLassa fever,55Whipple's
,~~ actinomyco-
of symptoms ranged from 1 to 204 months (mean 23.4 s ~ s nocardia asteroides,5*staphylococcal infection after car-
?~
months). In this series, 62% had had the post-pericardiotomy diac surgery,59amnla '
sl oe ,
@ and Streptococcus milleri.61
syndrome.26An unusual instance of traumatic constrictive
pericarditis resulted from self-mutilation by sewing needles Neoplastic Disease
inserted through the chest wall.27
Radiation therapy of mediastinal tumors is an important Metastatic neoplasm, most commonly from the lung, the
cause of pericardial disease, in addition to causing myocardial, breast, or one of the lymphoma group, may be responsible for
valvular, and coronary artery disease?8 Radiation therapy may the syndrome of constrictivepericarditis.62 In one recent series,

3. N. 0. Fowler: Constrictive pericarditis 343
4 of27 cases were caused by neoplastic disease. l 9 Occasional ranging in age from 8 to 70 years.15 The symptoms of con-
instances are caused by primary pericardial me~otheliorna.6~ strictive pericarditis usually develop slowly over a pcriocl of
years, but occasionally, especially when the cause is known,
Rare and Uncommon Causes (Table 11) can be shown to develop within a few months alicr cardiac
surgery or mediastinal irradiation. I n one series of45 patients,
Myocardial infarction may be followed by constrictive symptoms developed between 1 and 204 months following
pericarditis, but only rarely, with only a few instances having cardiac surgery.2hGimlettex6reported that 28 patients devel-
been reported.I9% h4-h7 Some instances are associated with oped constrictive pericarditis within 1 year after acute pcr-
Dressler’s postmyocardial infarction syndrome,67and others carditis. Wise and ContiX7 reported on more than I00 patients,
with hemopericardium complicating anticoagulant therapy.65 among whom dyspnea, present in 78%, was the most coninion
There appears to be an association between congenital atri- symptom; edema was present in 64%, abdominal swelling in
al septnl defect and constrictive peri~arditis.6~-~~ Mat-
Just and 64%, abdominal discomfort in 32%, fatigue in 25%, and or-
tingly’-’ reported 4 cases, and 63 from a literature review, of as- thopneain 22%. Abdominal discomfort, nausea, and voiniting
sociation between atrial septa1defect and pericardial adhesion, may be due to hepatic or bowel congestion. SchiavoneXX re-
effusion, or constriction. The reason for this association is un- ported dyspnea in each of I8 patients, edema i n 13 of 18, and
known. bloating in 12 of 18. Wychulis r t al. Is reported eKort dyspnea
Constrictivepericarditis may have a hereditary background, in 90% of 137 cases. Chest pain, possibly due to active intlani-
for example, mulibrey nanism, reported principally from Fin- ination, was present in 24%. Right upper quadrant or abdom-
land.7JThese patients have skeletal muscle hypotonia, hepatic nal pain was reported in I 1%, and only three patients reported
enlargement (? congestive), dilated cerebral ventricles, and orthopnea or paroxysmal nocturnal dyspnea. Cameron ri (I/. l 7
retinal pigmentation hence mu for muscle, li for liver, br for reported exertional dyspnea in 56% of 95 cases; fatigue was
brain, ey for eyes. Nanism is from the Greek nanos, a dwarf. A present in 55%, increasing abdominal girth in 29%,,and ab-
few instances have been reported from the United dominal pain in 12%.
Other uncommon causes of constrictive pericarditis include
~ a r c o i d o s i s primary chyl~pericardium,~~
,~~ dermatomyosi-
ti^,'^ a u b e s t ~ s i s , ~ ~ ~
systemic amyloidosis,8’and implantation Physical Examination
of a cardioverter defibrillator.82
A few instances have been associated with drugs, such as General examination may show abdominal enlargement
procainamide-induced lupus syndrome?? methy sergide ther- due to ascites. Schiavone reported ascites in 17 01’ I9 pa-
apy,83,KJ hydralazine-induced lupus syndr0me.4~
and Rarely, a tients;x8however, the Stanford group reported ascites i n only
transient hemodynamic pattern of constrictive pericarditis 28% of 95 cases,I7 and Bashi eta/.22 90% oftheir I I8 cases.
in
may be found in acute idiopathic pericarditis.8s Tuna and Danielson found ascites in 60% of the Mayo Clinic
series.I8 Hepatomegaly is common, being found in 73%)of
one series,lX in 100% of another.’? The combination oi‘as-
and
History cites and hepatomegaly may lead to a mistaken diagnosis of
liver disease, as occurred initially in 10of 95 patients reported
Many series have noted a male preponderance. In a series from Stanford.I7 Splenomegaly due to portal hypertension is
described by Wychulis er al., 100 of 137 patients were male, commonl6. However, the almost universal presence of ele-
vated jugular venous pressure, 99% in one study and 100%
in two others,22,x9 should eliminate liver cirrhosis as the cause
of hepatomegaly and ascites. Peripheral edema was found in
64% of one seriesx7and 70% of another.36Schiavone reported
TABLE 11 Rare and uncommon causes of constrictive pericarditis
edema in 13 of 19 cases,88and edema was reported i n 84%’of
(1Y88-1993)
another group.22The cardiac apical impulse is often abscnt;
Amyloldosis this was true in 90% of Wood’s series2‘
Actinoinycosis Many of the physical findings that are thought characteris-
Nocardia asteroides tic of constrictive pericarditis are, in fact, quite variable.
Implantable cardioverter defibrillatorinfection Paradoxical arterial pulse is an inconsistent finding; it was
Myocardial infarction (Dressler’s syndrome) found in 40% in one series,lS 16% in another,I7 14% in anoth-
Asbestosis er,x9but in 84% in one study.22A pericardial knock sound was
Whipplc’sdisease described in 5%,17 in 46% (S3),36in 11 of 19 cases,xxund
Lassa fever 36%.89 Kussmaul’s sign (inspiratory swelling of the neck
Hydralazine-inducedlupus-like syndrome veins) is a rather uncommon finding, appearing in only 13%of
Pericardial mesothelioma 95 patients.I7 This sign is not specific; it also occurs with KV
Dennatumyositis failure, restrictive cardiomyopathy, RV infarction, and in tri-
Self-mutilationwith sewing needles
cuspid s t e n o s i ~The~mechanism of Kussmaul’s sign is un-
.~
Sclerotherapyof esophageal varices
certain.91Kussmaul ascribed this phenomenon to inspiratory

4. 344 Clin. Cardiol. Vol. 18, June 1995
traction on the great veins in the mediastinum. Studies of pa- outflow tract obstruction by a fibrousband.ImWe reported an
tients with constrictivepericarditis show littlerespiratory vari- instance of RV hypertrophy associated with constriction of the
ation in superior vena caval flow velocity?2 One study as- left atrioventriculargroove in a 13-year-oldboy?4 Fukuda et
cribed the inspiratory swelling of the neck veins to transmis- a1.'O1 reported a case with ECG evidence of RV hypertrophy
sion of the normal inspiratory increase of intra-abdominal without outflow tract obstruction. Levine reported changes of
pressure to a tense, overly filled systemic venous system?O left ventricular (LV) hypertrophy in 5 of 67 patients and a sug-
One physical finding is against the diagnosis.Cardiac mur- gested pseudoinfarctionpattern in 6 of the 67?8
murs usually are not found unless there is complicatingvalvu-
lar disease or a fibrous band constricting the RV outflow tract.
Paut et aLZ0 found murmurs in only 3 of 53 patients, one of Echocardiogramsand Echo-DopplerStudies
whom had aortic stenosis.Schrire et ~ 1reported tricuspiddi-
. ~ ~
astolic murmurs in two cases, probably caused by constriction M-Mode Echocardiograms
of the atrioventricularring, producing tricuspid stenosis.
M-mode echocardiograms provide useful information in
constrictivepericarditis,but are not diagnostic of the disease.
Radiologic Studies Ventricular dimensions usually are normal and ventricular
function is preserved. Pericardial thickening was recognized
The typical chest radiogram shows a heart of normal size, in only 38% of one series of 40 patientslo2and in 42% of an-
with clear lung fields. However,the cardiopericardial silhou- other? In the series of Engel et U ~ . , ~ left atrial enlargement
O ~
ette may be enlarged, especially with effusive-constrictive was present in 75% and premature pulmonary valve opening
pericarditis. Left atrial enlargement may occur.94Paul et al. in 14%of cases. This last finding may be explainedby the fact
found moderate or marked increase in heart size in 38% of pa- that the elevated RV diastolic pressure is equal to pulmonary
tients of their series.20
Radiologic evidence of pericardial cal- artery diastolic pressure, or nearly so. Paradoxical septal mo-
cification was found in 40% of 23 l cases in the Mayo Clinic tion is nearly always present. Diastolic flattening of the LV
but in only 5% of the Stanfordseries.17In the study by posterior wall is often present and was found in 85% of this se-
Bashi et al. of 118 patients,pericardial calcificationwas found ries. An atrial systolic septal notch may be seen.Io2
in 21%22 and in another by Oh et aLg9 was found in 1 of 25
it
patients. Pleural effusions are found in 2 60% of patients, and Two Dimensional Echocardiograms
pulmonary edema in 5-10%.95
Angiocardiographicstudies tend to show loss of the normal Two-dimensionalechocardiogramsmay offer some help in
outward convexity of the right atrial border, as well as evi- recognizing Constrictive pericarditis but are more useful when
dence of pericardial thickening at the right atrial level. supplemented by Doppler studies. Characteristically,there is
biatrial enlargement, with normal ventricular dimensions.
Ventricular ejection fraction is preserved. Diastolic septal
Electrocardiographic Studies bounce may be seen. Fast-speed echocardiographic studies
may show evidence of rapid ventricular filling in early dias-
The electrocardiogram(ECG)is seldom normal in constric- tole.lo3D'Cruz etal.'"'' described a decreased angle (450")
tive pericarditis.An intra-atrialconduction defect with "P mi- between the posterior wall of the left atrium and that of the left
wale" pattern is common?6 P-wave changes suggestive of left ventricle in the parastemal long-axis view in five of seven pa-
atrial enlargement were found in 37% of 54 cases in one tients with constrictive pericarditis. This was not seen in other
study?' in 19% of 47 cases in anotherY8an in 3 1% of 122 cas- forms of heart disease with left atrial enlargement, except in 1
es in an0ther.9~ voltage of the QRS complex and atrial ar-
Low of 16 patients with mitral stenosis. Inspiratory movement of
rhythmias are frequentfindings.Paul etal.20reported atrial fib- the interventricularand interatrial septum toward the left has
rillation in 18of 52 patients and atrial flutter in 5. Wood found been described.lo5
atrial fibrillation in 35% and atrial flutterin In his study, Inferior vena caval plethora: The inferior vena cava usual-
atrial fibrillation was more common when the process was of ly is dilated in constrictivepericarditis, and its diameter shows
longer duration. Cameron et al. l7 reported atrial fibrillation in little respiratory variation;'@ however, RV failure or cardiac
13% of 95 patients.The study by McCaughan et al. of 23 1 pa- tamponade may show the same findings.
tients reported low-voltageQRS in 40% and atrial arrhythmias Superior vena cavalflow velocity patterns: Superior vena
in 29%.36Bashi et d Zfound low-voltageQRS in 75% of 118
2 caval Doppler flow velocity patterns were studied in 14 pa-
patients and atrial arrhythmiasin 10%.Atrial flutter was found tients with cardiac tamponade, 7 with constrictive pericarditis,
in 5 of 52 cases in one studyF0in 3 of 78 cases in anotherY7and and 8 normal In six of seven patients with constric-
in 3 of 67 in another?8Patternsof bundle-branchblock or ven- tive pericarditis, diastolicflow velocity exceeded systolicflow
tricular hypertrophy are uncommon. Right ventricular hyper- velocity. There was little respiratory variation in systolic flow
trophy, in some instances, produced by a fibrous band con- velocity in normal subjects or in constrictive pericarditis. In
stricting the RV outflow tract, was reported in 6 of 122 cases.99 tamponade there was little diastolic flow in the first expiratory
Chesler et al. also reported a singlecase due to right ventricular heart beat, corresponding to a loss of the right atrial Ydescent.

5. N. 0. Fowler: Constrictive pericarditis 345
With tamponade, there was marked inspiratory augmentation narrow pulse pressure in the RV pressure pulse. Hansen and
of both systolicand diastolic flow velocity. co-workersxdescribed a diastolic dip and plateau pattern in the
Pulnionaty venousflow: Schiavoneet al. lox in a study of
Io79 right ventricle in six cases of constrictive pencarditis. Wood
four patients with constrictivepericarditis,found that both sys- showed that LV end-diastolic pressure usually did not exceed
tolic and diastolicflow velocity increased during expiration in RV end-diastolic pressure by more than 5 mmHg.21Yu et al.
constrictive pencarditis, but only diastolic flow velocity stated that RV systolic pressure usually did not exceed 50
showed an expiratory increase in four cases of restrictive car- mmHg and that RV diastolic pressure was characteristically
diomyopathydue to amyloidosis.Klein et al. " studied 14pa- more than one-third of RV systolic pressure in constrictive
tients with constrictive pericarditis by Doppler transesoph- peri~arditis."~
ageal echocardiography.In inspiration, the pulmonary venous Wood pointed out that the cardiac output tends to be greater
systoliddiastolic flow velocity ratio fell below 0.65 in con- and the systemic arteriovenous oxygen difference smaller in
strictive pericarditis. Also, peak diastolic flow velocity fell constrictive pericarditis than in cardiomyopathy.21 his se-
In
40% on average during inspiration.These two features sepa- ries, cardiac output averaged 4.7 Vmin in constrictive pen-
rated constrictive pericarditisfrom restrictivecardiomyopathy. carditis, and 3.5 Vmin in cardiomyopathy.Arteriovenous oxy-
Hepatic veinflow velocity patterns: Von Bibra and associ- gen difference averaged 5 1 ml/l in constrictive pericarditis,
atesI'(~studied 13patients with constrictivepericarditis and25 and 75 mv1in cardiomyopathy.In early constrictive pericardi-
with RV pressure overload. Patients with constrictive pericar- tis, the cardiac output tends to be normal.I15 In a study of 10
ditis showed late systolic and late diastolic flow reversal; those patients with constrictive pericarditis,Reddy116 reported mean
with tricuspid regurgitation showed only systolic flow rever- right atrial pressures from 7 to 30 mmHg, and RV end-dias-
sal. Oh and associatesx9 found expiratory augmentation of di- tolic pressures from 9 to 32 mmHg. L f ventricular end-dias-
et
astolic flow reversal (2 25% of forward flow) in a study of 25 tolic pressures were from 9 to 32 mmHg. The cardiac index
patients with constrictive pericarditis. ranged from 1.4to 3.2 Vmin/m2,and the arteriovenousoxygen
Tricuspid and mitral valve flow velocity patterns: Using difference from 4.8 to 9.2 ~ 0 1 %In seven patients, Qberg et
.
Doppler echocardiography,Hatle et al. studied mitral and al. reported right atrial pressures from 1 4 2 4 mmHg and pul-
tricuspid valve flow velocity patterns in 7 patients with con- monary wedge pressures 14-26 mmHg.*17 Cardiac index was
strictivepericarditisand in 12with restrictivecardiomyopathy. 2.0-3.2 Vmin/m2.Left ventricular ejection fractionswere nor-
Patients with constrictive pericarditis had marked inspiratory mal in all. Reddy116 reported that right atrial pressure tracings
decrease in early mitral flow velocity and increase in early tri- showed prominent X and Y descents, with little respiratory
cuspid flow velocity compared with normal controls and with variation. Normally, the right atrial mean pressure falls sever-
patients with restrictive cardiomyopathy. Mancuso et al. al mmHg relative to intrathoracicpressure during inspiration.
studied Seven patients with constrictive pericarditis and six With constrictive pericarditis, because of the fibrotic shell sur-
with restrictive cardiomyopathy. Patients with constrictive rounding the heart, inspiratory fall in intrathoracicpressure is
pericarditis showed higher diastolic mitral flow velocity pat- not reflected in the right atrial pressure tracing, and the right
terns at the onset of expiration,with a decrease at the onset of atrial pressure tends to show no change or may actually rise
inspiration. Reciprocalflow velocity changes with respiration during inspiration.
were found across the tricuspid valve. Patients with restrictive Cameron et al.17 reported on 95 patients with constrictive
cardiomyopathy showed little change in mitral and tricuspid pericarditis, 23 of whom had effusive-constrictive disease.
flow velocity with respiration, but had moderate to severe mi- Mean right atrial pressure was 16 k 5 mmHg; mean RV end-
tral and tricuspid regurgitation. Trivial mitral and tricuspid re- *
diastolic pressure was 18 6 mmHg; mean pulmonary capil-
gurgitation was found in only one patient with constrictive *
lary wedge pressure was 19 5 mmHg; and mean LV end-di-
pericarditis. Oh and associatess9found that there was < 10% astolic pressure was 21 f 5 mmHg. Mean cardiac index was
respiratory variation in mitral valve early diastolic velocity in 2.2 k 0.7 Ymin/m2.
normal subjects and in those with restrictive cardiomyopathy, Occasionalinstances of localized cardiac constriction by fi-
whereas with constrictive pericarditis there was a > 25% expi- brous bands are reported. Vallance et al.llg described an in-
ratory increase in mitral valve early diastolic velocity. stance of constrictionof the RV outtlow tract, with an RV pres-
sure of 1151-1 6 mmHg and a pulmonary arterial pressure of
30/11 mmHg. There was an ECG pattern of RV hypertrophy.
Hernodynamics Pulmonary trunk constriction by a fibrous or a fibro-calcific
band has been reported.lm2 l I 9 Fibrous bands constricting the
The hemodynamics of constrictive pericarditis were re- atrioventricular grooves may produce tricuspid or mitral
viewed by Shabetai et al. Since, by definition,constrictive ~tenosis.~~,~~-94 Some instances of RV outflow tract obstruc-
pericarditisimpairs diastolic filling of the ventricles, elevation tion by fibro-calcificbands have followed previous surgery for
of both RV and LV end diastolic pressures is to be expected. constrictivepericarditis. *O
This finding was reported in 100% of the Mayo Clinic Circulating atrial natriuretic factor tends to be low or nor-
Right atrial pressure elevation with prominent X and Y de- mal in constrictive pericarditis, rising after pericardial resec-
scents ("W' wave form) was first reported by Bloomfield et tion.lz1> This suggests that atrial natriuretic factor release is
lZ2
aL7 These authors also described the early diastolic dip and more likely to be associated with atrial stretch than with in-

6. 346 Clin. Cardiol. Vol. 18, June 1995
creased atrial pressure alone. Anand and associates studied 16 atrial pressure by more than 4-5 mmHg. Similarly, LV and RV
patients with constrictive pericarditis and compared them with diastolic pressures may be within 45 mmHg of each other and
-
ii group with myocardial disease and edema.'*? Right atrial are equal to the right atrial and pulmonary capillary wedge
preswre tended to be higher and pulmonary arterial pressure pressures. Some instances of restrictive cardiomyopathy have
lower in the group with constrictive pericarditis. Total body this same equalization of RV and LV filling pressures. Several
water, extracellular fluid volume, and exchangeable sodium studies have described methods of distinguishing between
were higher in constrictive pericarditis, and circulating atrial these two clinically similar disorders. These include the rate of
natriuretic peptide values were lower. Plasma norepinephrine, LV filling; patterns of diastolic flow across the mitral and tri-
renin activity, and aldosterone were comparably elevated in cuspid valves; superior vena caval, pulmonary venous. and
the two groups. hepatic venous flow velocity patterns; and MRI and CT scan
studies of pericardial thickness. Studies of the relationship be-
tween RV and LV diastolic pressures may be helpful, but are
Effusive-ConstrictivePericarditis seldom definitive. Endomyocardial biopsy may be helpful if a
specific infiltrative cardiomyopathy is found. The following
This condition was mentioned by Wood2I and by Spodick studieshave been made in relatively small numbers ofpatients,
and Kumar123 was popularized by Hancock.'" In this dis-
and especially those with restrictive cardiomyopathy, and need
ease, in addition to pericardial thickening and diastolic cardiac confirmation in larger series.
constriction, there is a collection of fluid between the parietal
and visceral pericardium (epicardium). As a result, the cardi- Left Ventricular Filling Rate
opericardial silhouette may be larger on chest radiogram than
is usually the case with purely constrictive pericarditis. In the Tyberg and associates, using angiocardiography, found LV
Stanford series of 23 cases, 10followed radi0thera~y.l~ Other diastolic filling to be more rapid in constrictive pericarditis
etiologies consisted of seven instances of idiopathic pericardi- (averaging 85% in the first half of diastole) than in normals
tis, three of connective tissue disease, two that followed infec- (averaging 65% in the first half of diastole), or in amyloid re-
tions, and one neoplastic. I have seen instances due to rheuma- strictive cardiomyopathy (averaging 45% in the first half of di-
toid disease, tuberculosis, and penetrating trauma. Some in- astole).I17 More rapid LV filling in constrictive pericarditis
stances are associated with uremia. l o Effusive-constrictive than in restrictive cardiomyopathy, using fast-speed echocar-
pencarditis was reported to follow Lassa fever,55salmonella diography, was found in a study by Janos et (11. Io3 Gerson rt
infection?" and streptococcal infection.6' nl. 125 using nuclear ventriculography, found more rapid LV
The hemodynamic features are characteristic. Right atrial, filling in constrictive pericarditis than in normals or in restric-
pulmonary wedge, and intrapericardial pressures are equally tive cardiomyopathy. h o n e y et al. found that LV diastolic
increased, and there is a prominent X descent and no promi- filling was more rapid in constrictive pericarditis than in re-
nent Y descent in the right atrial pressure trace and no promi- strictive cardiomyopathy throughout the first 1040% of the
nent early diastolic dip in the RV pressure tracing. When all diastolic period.
the pericardial fluid is removed by needle pericardiocentesis,
intrapericardial pressure falls to near zero, but the right atrial,
Mitral and Wcuspid Diastolic Flow Patterns
RV diastolic, and pulmonary wedge pressures remain elevat-
ed. In addition, a prominent Ydescent appears in the right atri-
al pressure trace and a large early diastolic dip appears in the Hatle and associates,lIl using Doppler echocardiography,
RV pressure record. found that patients with constrictive pericarditis had a marked
inspiratory decrease in early mitral flow velocity (2 25%).
whereas this decrease was < 15% in normals and in patients
Distinction between ConstrictivePericarditis and with restrictive cardiomyopathy. Oh and associates8' found
Restrictive Cardiomyopathy similar respiratory variations in early mitral flow velocity i n
patients with constrictive pericarditis. It should be pointed out
Patients with constrictive pericarditis and restrictive car- that similar flow patterns may be found with obstructive air-
diomyopathy may have similar clinical and hemodynamic pat- way disease and cardiac tamponade. Mancuso et al."2 found
terns. Both may have persistent elevation of systemic venous mitral and tricuspid regurgitation common in restrictive car-
pressure, a positive Kussmaul's sign, pulsus paradoxus, and a diomyopathy and uncommon in constrictive pericarditis.
heart that is of normal size or slightly enlarged on chest radio-
gram. Both conditions often have a preserved LV ejection frac- PulmonaryVenous Flow Velocity
tion of 2 0.50and a similar hemodynamic pattern, with an ear-
ly diastolic dip and plateau pattern in pressure records of both In constrictive pericarditis, Doppler transesophageal echo-
right and left ventricles. Right atrial pressure and pulmonary cardiography showed a peak diastolic flow velocity fall of >
capillary wedge pressures usually are increased within the 40% on inspiration. This plus a systolic/diastolic flow ratio <
range of 12-32 mmHg. In constrictive pencarditis, the pul- 0.65 in inspiration demarcated constrictive pericarditis from
monary wedge pressure typically does not exceed the right restrictive cardiomyopathy.109

7. N. 0. Fowler: Constrictive pericarditis 347
Hepatic Vein Flow Velocity Patterns ocardial and pericardial disease may be present, in particular
with sarcoid~sis,~~ radiation therapy,126
~ancarditis,’~~
or con-
Patients with constrictivepericarditis tend to show late sys- nective tissue disease.
tolic and diastolic flow reversal by Doppler echocardiogra-
phy.Il0Patients with constrictive pericarditis were found to
have expiratory augmentation of diastolic flow reversaleg9 Wtment
With restrictivecardiomyopathy,there is inspiratory augmen-
tation of flow reversal.89 Although some improvement in pulmonary and systemic
congestion can often be obtained by the use of diuretics, this is
MRI and CT Scan Studies achieved at the expense of a reduction in cardiac output. Oc-
casional cases of subacute constrictive pericarditis will re-
This subject was reviewed by Hoit.12’ The normal peri- spond to medical management, including adrenal steroids, if
cardiuni is < 3 mm in thickness.If a patient has a hemodynam- treated before the stage of pericardial fibrosis.30 Thus, in
ic pattern consistent with constrictivepericarditisor restrictive symptomatic patients, the treatment is ordinarily that of peri-
cardiomyopathy,the diagnosis of constrictivepericarditis can cardial resection. Because the mortality rate of this operation
be made when pericardial thickness is found to be > 3 mm by tends to be higher in patients with advanced symptoms, one
CT scanningor MRI study. However,not all cases of constric- should not wait until the patient is totally incapacitated. On the
tive pericarditishave such evidence of pericardial thickening. other hand, patients with few or no symptoms may remain sta-
McCaughan et al. describedpencardial thickeningby CT scan ble for years, and one can safely defer operation in those who
in 13of 16cases (8 1%).36 Masui et al. found pericardial thick- are in functional class I or early class 1 of the NYHA. Patients
1
ening (1 mm) by MRI study in 15 of 17 patients with con-
4 who are in late and progressive class 1 should be recommend-
1
strictive pericarditi~.~~~ et ~ 1found the CT scan to
Killian . ~ ~ ed for pericardial resection. Tuna and Danielsonlgreported an
show increasedpericardial thickening in 23 of 29 post cardiac operative mortality rate of 1% for patients in functional class-
surgical cases of constrictive pericarditis. Cacoub et a1.19 es I and 11, 10%for those in class 1 1 and 46% for those in class
1,
found that only 6 of 16 patients with constrictivepericarditis IV. The overall mortality rate for 3 13patients operated upon at
had pericardialthickening on CT scan, and two were negative Mayo Clinic since 1936was 14%.
on MRI study. Oren et al. 129found increasedpericardial thick- The surgical mortality rate was 16% in 118 cases reported
ness in each of five cases of constrictivepericarditis,using cine by Bashi et al. ,22 and 11% in 52 patients operated upon in the
computed tomography.Oh et aLg9found increased pericardial last 12years of this series. The operativemortality was 12% in
thickening by CT scan in each of 2 1patients with constrictive Cameron et al. 5. report of 95 patients undergoing surgery at
pericarditis. Stanford University.17In areview by Siefert et u1.,132 80-90%
of hospital survivors achieved NYHA class I or 1 functional
1
Hernodynamic Investigations status followingpericardial resection.
Both restrictive cardiomyopathyand constrictivepericardi- Occult ConstrictivePericardial Disease
tis may produce equal elevations of RV and LV end-diastolic
pressuresto between 12and 30 mmHg. Pressurevalues favor- Bush and associates133 described 19 patients with occult
ing constrictivepericarditis include an RV systolic pressure < constrictivepericarditis. These patients had normal or nearly
50 d g ; LV diastolic pressure not exceeding RV diastolic normal right atrial pressures (1-8 d g ) , which rose abnor-
pressure by more than 5 mmHg; RV diastolicpressure exceed- mally and equilibratedwith pulmonary wedge or LV end-dias-
ing one-third of RV systolic pressure.13o Vaitkus and Kuss- tolic pressures after infusion of one liter of normal saline with-
maul’snview found that of 70 patients meeting all three crite- in 6-8 min. A dip and plateau pattern in the RV pressure trace
ria, 9 1% had constrictivepericarditis. Of 18 satisfying one cri- and a lack of respiratory variation in right atrial pressure also
terion or none, 17 (94%) had restrictive cardi~myopathy.’~~ appeared after infusion. Eleven patients were operated upon
and had improvementin fatigue and dyspnea; all had pericar-
dial adhesions at operation. The place of this test in diagnosing
EndomyocardialBiopsy and treating pericardial disease is uncertain. It is difficultto ex-
plain all the symptoms in these patients entirely on the basis of
Endomyocardial biopsy may be helpful in distinguishing abnormal hemodynamics. Also, there may be some risk in
between constrictivepericarditisand restrictivecardiomyopa- saline infusion at this rate, and pulmonary wedge pressure
thy, especially when a specific diagnosis of restrictive disease should be carefully monitored if this test is carried out.
can be made, for example, cardiac amy10idosis.l~~ When the
myocardial biopsy shows a normal pattern or a nonspecific
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