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Curriculum in Cardiology
Case Discussion

Constrictive Pericarditis
Faraz Ahmed Farooqui
Department of Cardiology, Cardiothoracic and Neurosciences Centre, All India Institute of Medical Sciences, New Delhi, India

Abstract
A patient presented with Ascites for 3 months. Clinical examination showed ascites , presence of atrial fibrillation and a raised JVP. Further
evaluation helped make a diagnosis of constrictive pericarditis. The questions that arise at each step of evaluation of such a patient starting
from the history to examination and investigation is discussed in this bedside case discussion.

Keywords: Ascites, bedside case discussion, constrictive Pericarditis, restrictive cardiomyopathy

Clinical Presentation Examiners: Please summarize the case history.

A 20‑year‑old male patient, resident of Bihar, presented with
the following:
• Abdominal distension and facial puffiness for 3 months
• Bilateral lower limb swelling for 1 month
• Exertional dyspnea for 1 month.
The patient was apparently asymptomatic 3 months ago when
he began to develop abdominal distension, which was insidious
in onset and gradually progressive. There was associated
facial puffiness. Two months later, he developed swelling of
bilateral lower limbs. He also gives a history of the New York
Heart Association (NYHA) Class II exertional dyspnea which
was insidious in onset. However, dyspnea has not shown any
progressive worsening. There was no associated orthopnea
or paroxysmal nocturnal dyspnea (PND). The patient gives a
history of easy fatigability. The patient would have intermittent
relief in his symptoms with diuretics. However, there would
be a recurrence of symptoms on stopping medications. There
is a history of early satiety; however, appetite has remained
normal. There was no history of palpitations, syncope, or
cyanosis. There was no history of jaundice, hematemesis, or
melena. The patient does not give any history of previous blood
transfusions or high‑risk behavior.
Past history was significant for intake of antitubercular
therapy (ATT) 1 year ago for pleural effusion. He had taken
a complete course of ATT. There was no history of trauma to
the chest, prior chest surgeries, or radiation therapy.
Examinee: A 20‑year‑old male patient has presented with a
3‑month history of progressive abdominal distension followed
by pedal edema and NYHA II dyspnea without associated
orthopnea/PND. There is a history of past extrapulmonary
tuberculosis with a history of ATT intake.
Examiners: What are your differential diagnoses based on
your history?
Examinee: I would like to consider the following differentials
based on the history.
• Constrictive pericarditis  –  In the context of systemic
venous congestion, a history of abdominal distension
preceding pedal edema (ascites precox) and a past
history of extrapulmonary tuberculosis make constrictive
pericarditis the most likely diagnosis
• Restrictive cardiomyopathy  (RCM)  –  Prominent
systemic venous congestion associated with dyspnea
may be a presenting feature of RCM. RCM is more
likely in a patient with a predisposing disease such as
Address for correspondence: Dr. Faraz Ahmed Farooqui,
Department of Cardiology, Cardiothoracic and Neurosciences Centre, All
India Institute of Medical Sciences, New Delhi, India.
E‑mail: farazahmedfarooqui@yahoo.co.in
Date of Submission: 23‑Jul‑2019 Date of Acceptance: 02-Aug-2019
Date of Revision: 28-Jul-2019 Date of Web Publication: 19-Aug-2019

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DOI:
10.4103/jpcs.jpcs_45_19 How to cite this article: Farooqui FA. Constrictive pericarditis. J Pract
Cardiovasc Sci 2019;5:111-5.

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Farooqui: Constrictive pericarditis
diabetes mellitus or amyloidosis. Uncommon causes of
RCM include endomyocardial fibrosis, iron‑overload
cardiomyopathy, and storage disorders such as Fabry
disease, radiation heart disease, and idiopathic RCM[1,2]
• Severe tricuspid regurgitation  (TR)  –  Organic TR is
an important differential diagnosis that closely mimics
pericardial constriction in its clinical presentation. Organic
TR may be congenital or acquired. Ebstein’s anomaly is
an important cause of organic TR. Other causes of organic
TR include tricuspid valve (TV) prolapse, carcinoid
syndrome, trauma, dilated cardiomyopathy, infective
endocarditis (IE), or following surgical excision of the
TV in patients with IE unresponsive to the medical
management.
Examiners: What is the normal thickness of the pericardium?
Examinee: The normal thickness of the pericardium is ≤2 mm.
A thickened pericardium on computed tomography is defined
a pericardial thickness >4 mm.[3,4]
Examiners: What is the most common cause of constrictive
pericarditis?
Examinee: The most common cause of constrictive pericarditis
in the developed world is postsurgical followed by idiopathic
constrictive pericarditis.[5] Tuberculosis continues to be the
most common cause of constrictive pericarditis in India.[6]
Examiners: What percentage of patients develop constrictive
pericarditis after cardiac surgery?
Examinee: Overall, 0.2%–2.4% of patients develop
constrictive pericarditis after cardiac surgery.[5]
Examiners: What is the average duration of presentation of
postsurgical constrictive pericarditis?
Examinee: After cardiac surgery, the interval to presentation
with constrictive pericarditis is on average 2 years but can
range from as little as 1 month to >15 years.[5,7,8]
Examiners: Describe the pathophysiology of constrictive
pericarditis?
Examinee: Constrictive pericarditis arises after a cycle of
injury and inflammation, which might be initiated several
days, months, or years before clinical presentation, although
the precise event often remains elusive. Damage to pericardial
mesothelial cells is associated with an acute reduction of
tissue‑type plasminogen activator and reduced fibrinolytic
activity, with fibrinous inflammation and adhesion formation,
which is a possible mechanism for pericardial fibrosis, although
this mechanism is yet to be proven. Most patients (>80%) with
constrictive pericarditis who undergo pericardiectomy have
nonspecific fibrocalcific thickening of the pericardial tissue,
with evidence of ongoing acute or chronic inflammation of the
pericardium. In contrast to late presentations, reduced pericardial
compliance early in the disease course (usually <3 months) is
more often caused by inflammation than by fibrosis.[5,9]
Examiners: What is your understanding of the hemodynamics
of constrictive pericarditis?
Examinee: The principal hemodynamic abnormality in constrictive
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pericarditis is loss of pericardial compliance, with a reliance on
elevated ventricular pressures to maintain cardiac filling and
output, which eventually leads to primary diastolic heart failure.
The constricting pericardium insulates the heart from respiratory
intrathoracic pressure changes, which make the atrioventricular
pressure gradient decrease on the left during inspiration, and increase
during expiration. In healthy individuals, intrathoracic pressure
decreases by approximately 5–10 mmHg during inspiration, and
this pressure change is fully transmitted to the intracardiac cavities.
However, in patients with a noncompliant or thick pericardium,
the intrathoracic pressure change is not entirely transmitted to the
intracardiac cavities. The extrapericardial components of the vena
cavae and pulmonary veins are still subjected to these intrathoracic
pressure changes, leading to enhanced variation between left
and right filling pressures. Consequently, the pressure difference
between the pulmonary veins and the left ventricle during diastole
decreases with inspiration and increases on expiration, with opposing
changes between the vena cavae and right ventricle. In patients with
constrictive pericarditis, increased pericardial constriction leads to a
fairly fixed combined volume of the left and right ventricles, such that
atrioventricular filling of the right ventricle increases with inspiration
and decreases with expiration. Ventricular interdependence is a
characteristic hemodynamic feature of constrictive pericarditis.[10]
Examiners: What is the mechanism of dyspnea in constrictive
pericarditis?
Examinee: Exertional dyspnea in constrictive pericarditis
is the result of limited or no increase in cardiac output with
physical exertion. Orthopnea and PND are exceedingly rare
in cardiac catheterization procedure (CCP). This is because
pulmonary venous pressure rarely rises high enough to cause
interstitial edema of the lung.
Examiners: What is ascites precox and what is the underlying
mechanism?
Examinee: Ascites in constrictive pericarditis is out of
proportion to pedal edema. It usually occurs earlier than
pedal edema. This is referred to as ascites precox. Suggested
mechanisms include:
• Partial constrictor effect on at least one of the hepatic
veins, as it enters the right atrium
• Relatively high atrial pressures compared to other causes
• Hypoalbuminemia resulting from protein‑losing
enteropathy
• Cardiac cirrhosis
• Increased capillary permeability
• Impedance to lymph flow.[6,11]
Examination
On examination, the patient is thin built with a body mass
index of 17. He is afebrile. The pulse rate is 96 beats/min.
The pulse is irregularly irregular with apex pulse deficit of
20 bpm. No pulsus paradoxus is noted. The blood pressure
is 104/70 mmHg in the right upper limb and 106/72 mmHg
in the right lower limb. There is bilateral lower limb pedal
edema. The jugular venous pressure (JVP) is raised (12 cm
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Farooqui: Constrictive pericarditis

Dilated IVC
+
Mitral Inflow E/A > 0.8

Yes No

Ventricular septal shift Constriction unlikely

with respiration?

Present Absent

Medial e’ velocity ≥ 9 cm/s

Medial e’ velocity ≥ 9 cm/s None
Hepatic vein expiratory end-
Hepatic vein expiratory end-
diastolic reversal ratio ≥ 0.79
diastolic reversal ratio ≥ 0.79

Both present either present ≥ 1 present

Constriction Constriction Constriction

confirmed probable possible

Figure 1: The Mayo Clinic Criteria for Echocardiographic Diagnosis of cardiac catheterization procedure.

above the sternal angle at 45°) with absent “a” wave and
prominent “y” descent. There is no pallor, icterus, or
lymphadenopathy. The oxygen saturation by pulse oximetry
is 100%. There are no peripheral signs of chronic liver
disease.
Cardiovascular Examination
The precordium is symmetrical with no deformity or bulge.
The apex is visible in the fifth intercostal space about 1 cm
medial to the midclavicular line. On palpation, the apex is
tapping and is of a left ventricular (LV) type. There is no
parasternal heave. On auscultation, the first and second sounds
are normal. A pericardial knock is present. No murmur is
heard.
Other Systems Examination
The abdomen is soft. The liver is palpable 4 cm below the
costal margin. The liver dullness is in the fifth right intercostal
space, and the liver span is 14 cm. The liver is nontender and
nonpulsatile. Shifting dullness is presented suggestive of
ascites. The spleen is not palpable.
There is a dull percussion note with decreased breath sounds
in the right infrascapular, infra‑axillary, and mammary areas.
Examiners: Enumerate the positive findings in your
examination.
Examinee:
• Irregularly irregular pulse with a variable volume
• B/L lower limb pitting pedal edema
• Elevated JVP with prominent y descent
• The presence of a high‑pitched pericardial knock
• Shifting dullness on abdominal examination
• Dull percussion note with decreased breath sounds in the
right infrascapular, infra‑axillary, and mammary areas.
Examiners: What percentage of patients with constrictive
pericarditis have atrial fibrillation?
Examinee: In a previous study, atrial fibrillation has found to
be present in 22% of patients with constrictive pericarditis.[12]
Presence of pericardial calcification and higher duration of
disease have been shown to be associated with a higher chance
of developing atrial fibrillation.[13]
Examiners: How common is pericardial knock‑in constrictive
pericarditis?
Examinee: Approximately half of the patients with constrictive
pericarditis demonstrate an audible pericardial knock.
Examiners: How frequent is pericardial calcification in
constrictive pericarditis?
Examinee: Nearly 25%–30% of patients with constrictive
pericarditis demonstrate calcification on a chest radiogram.
Patients with tuberculous constrictive pericarditis have a higher
frequency (35%–50%) of radiologic calcification. Calcification
in constrictive pericarditis is best observed in a lateral chest
projection.[5]
Examiners: What is transient constrictive pericarditis?
Examinee: Transient constrictive pericarditis is characterized
by improvement and resolution of clinical features of
constriction spontaneously or with anti‑inflammatory therapy.
Recurrence of constriction is not seen in patients who respond
to anti‑inflammatory therapy.[14]
Examiners: Enumerate the Mayo Clinic Echocardiographic
Criteria for constrictive pericarditis.[15]
Examinee: Shown in Figure 1.

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Farooqui: Constrictive pericarditis

Examiners: Describe the cardiac catheterization findings in
constrictive pericarditis.
Examinee:
• Significantly elevated right atrial pressure
• Prominent “y” descent in the right atrial waveform,
commonly referred to as “Friedrich’s sign” along with
prominent “x” descent
• “M” or “W” pattern of the right atrial waveform due to
the combination of elevated mean pressure, inconspicuous
positive waves, and prominent descents
• Elevation and equalization of diastolic pressures (within
5 mmHg) in all cardiac chambers
• “Dip and plateau” or “square root sign” in the ventricular
pressure waveforms which refer to the pattern of
accentuated early dip in diastolic pressure followed by
plateauing in mid‑late diastole
• Elevation in the right ventricular systolic
pressure (RVSP) (generally limited to <50 mmHg)
• A marked increase in the right ventricular end‑diastolic
pressure to levels more than one‑third RVSP
Figure 2: Chest X‑ray in cardiac catheterization procedure showing
calcification.
• Failure to decline or increase in right atrial pressure with
inspiration (Kussmaul’s sign)
• Decrease in early diastolic gradient between pulmonary
capillary wedge pressure and minimum LV diastolic
pressure is during inspiration
• Ventricular interdependence as demonstrated by systolic
area index (SAI) >1.1 × (SAI is then calculated as the
ratio of RV area [mmHg × s] to the LV area [mmHg × s]
in inspiration versus expiration).[10,16,17]
Examiners: What is the role of contrast‑enhanced magnetic
resonance imaging (MRI) in a patient with suspected
constrictive pericarditis?
Examinee: MRI provides an accurate assessment of
pericardial thickness in patients with constrictive pericarditis.
In addition, delayed gadolinium enhancement of the
pericardium  (especially  ≥3  mm) is suggestive of ongoing
pericardial inflammation and anti‑inflammatory therapy may
reverse the constriction in such patients.[5,18]
Examiners: Describe the outcomes after pericardiectomy
surgery in patients with constrictive pericarditis.
Examinee: Pericardiectomy is associated with a 30‑day
mortality rate ranging from 5% to 10%. The prognosis is
worse in patients with radiation‑associated and postsurgical
constrictive pericarditis.[19] One‑third of patients may develop
recurrent symptoms due to the progression of underlying
myocardial disease or less often due to recurrent constrictive
pericarditis.[5,20]
Chest X‑ray [Figure 2] shows calcification of the
cardiac border. There is no pulmonary hypertension or
cardiomegaly. Echo finds were suggestive of CCP with a
dilated inferior vena cava and Doppler flow patterns suggest
of CCP [Figure 3].

Figure 3: Echocardiogram showing a dilated inferior vena cava, abnormal movement of the interventricular septum, increased respiratory variation of
the mitral and tricuspid Doppler signals, and enhanced hepatic vein flow reversals.

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Farooqui: Constrictive pericarditis
Figure 4: Catheterization of cardiac catheterization procedure.  Sci 2015;1:130-7.
Figure  4 shows the catheterization findings with a rapid Y
descent on the atrial waves, diastolic equalization, and a dip
and plateau wave.
Declaration of patient consent
The authors certify that they have obtained all appropriate
patient consent forms. In the form the patient(s) has/have
given his/her/their consent for his/her/their images and other
clinical information to be reported in the journal. The patients
understand that their names and initials will not be published
and due efforts will be made to conceal their identity, but
anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
References
1. Pereira NL, Grogan M, Dec GW. Spectrum of restrictive and infiltrative
cardiomyopathies: Part 1 of a 2‑part series. J Am Coll Cardiol
2018;71:1130‑48.
2. Pereira NL, Grogan M, Dec GW. Spectrum of restrictive and infiltrative
cardiomyopathies: Part 2 of a 2‑part series. J Am Coll Cardiol
Journal of the Practice of Cardiovascular Sciences  ¦  Volume 5 ¦ Issue 2 ¦ May-August 2019
2018;71:1149‑66.
3. Talreja DR, Edwards WD, Danielson GK, Schaff HV, Tajik AJ,
Tazelaar HD, et al. Constrictive pericarditis in 26 patients
with histologically normal pericardial thickness. Circulation
2003;108:1852‑7.
4. Khandaker MH, Espinosa RE, Nishimura RA, Sinak LJ, Hayes SN,
Melduni RM, et al. Pericardial disease: Diagnosis and management.
Mayo Clin Proc 2010;85:572‑93.
5. Syed FF, Schaff HV, Oh JK. Constrictive pericarditis – A curable
diastolic heart failure. Nat Rev Cardiol 2014;11:530‑44.
6. Kothari SS, Roy A, Bahl VK. Chronic constrictive pericarditis: Pending
issues. Indian Heart J 2003;55:305‑9.
7. Killian DM, Furiasse JG, Scanlon PJ, Loeb HS, Sullivan HJ. Constrictive
pericarditis after cardiac surgery. Am Heart J 1989;118:563‑8.
8. Gaudino M, Anselmi A, Pavone N, Massetti M. Constrictive pericarditis
after cardiac surgery. Ann Thorac Surg 2013;95:731‑6.
9. Welch TD. Constrictive pericarditis: Diagnosis, management and
clinical outcomes. Heart 2018;104:725‑31.
10. Doshi S, Ramakrishnan S, Gupta SK. Invasive hemodynamics of
constrictive pericarditis. Indian Heart J 2015;67:175‑82.
11. Deepti S, Gupta SK. A case of right sided heart failure. J Pract Cardiovasc
12. Ling LH, Oh JK, Schaff HV, Danielson GK, Mahoney DW, Seward JB,
et al. Constrictive pericarditis in the modern era: Evolving clinical
spectrum and impact on outcome after pericardiectomy. Circulation
1999;100:1380‑6.
13. Rezaian GR, Poor‑Moghaddas M, Kojuri J, Rezaian S, Liaghat L,
Zare N. Atrial fibrillation in patients with constrictive pericarditis: The
significance of pericardial calcification. Ann Noninvasive Electrocardiol
2009;14:258‑61.
14. Haley JH, Tajik AJ, Danielson GK, Schaff HV, Mulvagh SL, Oh JK.
Transient constrictive pericarditis: Causes and natural history. J Am Coll
Cardiol 2004;43:271‑5.
15. Welch TD, Ling LH, Espinosa RE, Anavekar NS, Wiste HJ, Lahr BD,
et al. Echocardiographic diagnosis of constrictive pericarditis: Mayo
clinic criteria. Circ Cardiovasc Imaging 2014;7:526‑34.
16. Geske JB, Anavekar NS, Nishimura RA, Oh JK, Gersh BJ. Differentiation
of constriction and restriction: Complex cardiovascular hemodynamics.
J Am Coll Cardiol 2016;68:2329‑47.
17. Sorajja P. Invasive hemodynamics of constrictive pericarditis, restrictive
cardiomyopathy, and cardiac tamponade. Cardiol Clin 2011;29:191‑9.
18. Wang ZJ, Reddy GP, Gotway MB, Yeh BM, Hetts SW, Higgins CB.
CT and MR imaging of pericardial disease. Radiographics
2003;23:S167‑80.
19. Bertog SC, Thambidorai SK, Parakh K, Schoenhagen P, Ozduran V,
Houghtaling PL, et al. Constrictive pericarditis: Etiology and
cause‑specific survival after pericardiectomy. J  Am Coll Cardiol
2004;43:1445‑52.
20. Chowdhury UK, Subramaniam GK, Kumar AS, Airan B, Singh R,
Talwar S, et al. Pericardiectomy for constrictive pericarditis: A clinical,
echocardiographic, and hemodynamic evaluation of two surgical
techniques. Ann Thorac Surg 2006;81:522‑9.
115