We describe a patient who suffered progressive right heart failure of unknown aetiology, despite a lengthy series of hospital investigations. Constrictive pericarditis had not been suspected during life, and was ultimately diagnosed as an autopsy finding. The salient clinical features and confirmatory investigations for this unusual disorder are reviewed. The case reminds us to consider the possibility of constrictive pericarditis in patients with unexplained chronic right heart failure, so that prompt investigation and treatment can be instigated

Aleksandra M Malkowska

CR Asher

DK Cooper

KM Chinnaiyan

LH Ling

MW Akhter

RB Himmelman

SC Bertog

T Masui

T Tabata

UK Chowdhury

W Stephen Waring

English

PubMed

Springer - Publisher Connector

BioMed CentralCases Journal
ssOpen AcceCase Report
Constrictive pericarditis is an easily overlooked cause of right heart 
failure: a case report
Aleksandra M Malkowska and W Stephen Waring*
Address: Scottish Poisons Information Bureau, Royal Infirmary of Edinburgh, Edinburgh, UK
Email: Aleksandra M Malkowska - aleksandra001@interia.pl; W Stephen Waring* - s.waring@ed.ac.uk
* Corresponding author    
Abstract
We describe a patient who suffered progressive right heart failure of unknown aetiology, despite
a lengthy series of hospital investigations. Constrictive pericarditis had not been suspected during
life, and was ultimately diagnosed as an autopsy finding. The salient clinical features and
confirmatory investigations for this unusual disorder are reviewed. The case reminds us to consider
the possibility of constrictive pericarditis in patients with unexplained chronic right heart failure, so
that prompt investigation and treatment can be instigated.
Case Report
A 76-year-old woman attended a gastroenterology outpa-
tient clinic for investigation of unexplained weight loss
and diffuse abdominal distension. Investigations showed
deranged liver biochemistry tests, and computed tomog-
raphy showed widespread ascites and a small pleural effu-
sion but no focal organ pathology. Analysis of ascitic fluid
showed total protein 34 g/L, and 3 white cells per mm3.
The patient was subsequently referred to a cardiologist,
who noted the presence of a soft systolic murmur. An
echocardiogram showed normal left ventricular dimen-
sions and function, but the right heart chambers were
dilated and there was moderate to severe tricuspid regur-
gitation. Cor pulmonale was suspected and, therefore, the
patient was referred to a respiratory clinic for investigation
of possible underlying lung disease. The patient had been
a non-smoker, with no past history of occupational expo-
sure or tuberculosis. Spirometry was normal and com-
puted tomography pulmonary angiography showed
patent pulmonary arteries, and small bilateral pleural
effusions. Cardiac catheterisation studies were proposed,
but patient defaulted from clinic.
Eighteen months later, the patient was admitted to hospi-
tal in extremis. Initial examination found temperature
34.6°C, blood pressure 65/30 mmHg, and heart rate 85
min-1. The patient had reduced conscious level, cool
peripheries, and elevated jugular venous pressure. An elec-
trocardiogram showed atrial fibrillation and right bundle
branch block. She was diagnosed with sepsis and dehydra-
tion, and treated with intravenous colloid administration,
gradual warming, and systemic antibiotics. Despite these
measures, hypotension and tissue perfusion progressively
worsened and the patient died shortly after arrival in hos-
pital.
Autopsy examination found extensive constrictive pericar-
ditis and chronic hepatic congestion. Additional findings
were colonic diverticular disease and gastrointestinal
ischaemia. Death was attributed to heart failure secondary
to constrictive pericarditis, exacerbated by sepsis and tis-
sue ischaemia. The earlier investigative findings, which
were performed over a 12-month duration, were attribut-
able, in retrospect, to constrictive pericarditis. There had
been no specific clinical signs suggestive of this diagnosis.
Published: 11 July 2008
Cases Journal 2008, 1:27 doi:10.1186/1757-1626-1-27
Received: 16 June 2008
Accepted: 11 July 2008
This article is available from: http://www.casesjournal.com/content/1/1/27
© 2008 Malkowska and Waring; licensee BioMed Central Ltd. 
This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), 
which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.Page 1 of 2
(page number not for citation purposes)
Cases Journal 2008, 1:27 http://www.casesjournal.com/content/1/1/27The initial echocardiograph did not comment specifically
on the pericardium, but is unlikely to have been suffi-
ciently sensitive to detect pericardial thickening.
Discussion
Constrictive pericarditis is a rare disorder that manifests
predominantly as right heart failure and, in severe cases,
systemic hypotension and circulatory collapse [1]. The
diagnosis may be difficult to establish [2]. Histopatholog-
ical features include localised fibrosis and calcification
within the pericardium. All four cardiac chambers become
encased so that diastolic pressure tend to equalise
between them, which results in systemic venous engorge-
ment and diminished ejection volume [3]. Ventricular fill-
ing during early diastole is rapid, due to high systemic
venous pressure, whereas late diastolic filling is impaired
by the rigid pericardium. Cardiac output may be pre-
served, at least in part, due to high diastolic filling pres-
sure. A comparatively small reduction in circulating
volume and central venous pressure can cause a dramatic
fall in ejection volume and cardiac output.
Constrictive pericarditis may arise as a complication of
previous cardiac surgery, pericarditis, and mediastinal
irradiation [4]. Other recognised but rare causes include
connective tissue disorders, malignancy, and local
trauma. Tuberculosis is uncommon in Western nations,
although occurs more commonly in developing countries.
In around one third, no underlying cause is identified,
although adenovirus or echovirus infection has been
implicated [5]. Clinical signs include oedema, ascites,
raised jugular venous pressure, pleural effusion, and
hepatomegaly. Around one third of patients with constric-
tive pericarditis have co-existent atrial fibrillation [6].
Plain chest radiography may show pericardial calcifica-
tion and unexplained pleural effusions [2]. Echocardiog-
raphy may confirm the presence of small ventricular
dimensions with preserved systolic function, and dilated
atria. Abrupt termination of diastolic filling may cause a
characteristic 'septal bounce' [7]. Cardiac catheterisation
studies allow definitive confirmation. Constrictive peri-
carditis may be distinguished from restrictive cardiomy-
opathy by demonstration of thickened pericardium on
computed tomography and magnetic resonance imaging
[8].
Surgical intervention may be appropriate, depending on
the severity of haemodynamic impairment. Total rather
than partial pericardiectomy yields better outcome, and
should be undertaken early, where possible [9]. Survival is
dependent on the underlying aetiology, and patients with
idiopathic constrictive pericarditis tend to have best out-
come after pericardiectomy [10].
Conclusion
Right heart failure may be caused by a variety of disorders,
including cor pulmonale, right ventricular infarction, mitral
stenosis, pulmonary embolism, and primary pulmonary
hypertension. Constrictive pericarditis is a comparatively
rare cause, and the diagnosis may be difficult to establish.
This case reminds us to consider constrictive pericarditis
in patients with unexplained right heart failure, so that
timely investigation and treatment can be initiated.
Consent
Written informed consent was obtained from the patient's
next of kin for publication of this case report. A copy of
the written consent is available for review by the Editor-in-
Chief of this journal.
Competing interests
The authors declare that they have no competing interests.
Authors' contributions
AMM performed a literature review and prepared a draft
manuscript; WSW contributed to manuscript writing and
editing. Both authors read and approved the final manu-
script.
References
1. Asher CR, Klein AL: Diastolic heart failure: restrictive cardio-
myopathy, constrictive pericarditis, and cardiac tamponade:
clinical and echocardiographic evaluation.  Cardiol Rev 2002,
10:214-229.
2. Akhter MW, Nuno IN, Rahimtoola SH: Constrictive pericarditis
masquerading as chronic idiopathic pleural effusion: impor-
tance of physical examination.  Am J Med 2006, 119:e1-e4.
3. Chinnaiyan KM, Leff CB, Marsalese DL: Constrictive pericarditis
versus restrictive cardiomyopathy: challenges in diagnosis
and management.  Cardiol Rev 2004, 12:314-320.
4. Ling LH, Oh JK, Schaff HV, Danielson GK, Mahoney DW, Seward JB,
Tajik AJ: Constrictive pericarditis in the modern era: evolving
clinical spectrum and impact on outcome after pericardiec-
tomy.  Circulation 1999, 100:1380-1386.
5. Cooper DK, Sturridge MF: Constrictive epicarditis following
Coxsackie virus infection.  Thorax 1976, 31:472-4.
6. Tabata T, Kabbani SS, Murray RD, Thomas JD, Abdalla I, Klein AL:
Difference in the respiratory variation between pulmonary
venous and mitral inflow Doppler velocities in patients with
constrictive pericarditis with and without atrial fibrillation.  J
Am Coll Cardiol 2001, 37:1936-1942.
7. Himmelman RB, Lee E, Schiller NB: Septal bounce, vena cava
plethora, and pericardial adhesion: informative two-dimen-
sional echocardiographic signs in the diagnosis of pericardial
constriction.  J Am Soc Echocardiogr 1988, 1:333-340.
8. Masui T, Finck S, Higgins CB: Constrictive pericarditis and
restrictive cardiomyopathy: evaluation with MR imaging.
Radiology 1992, 182:369-373.
9. Chowdhury UK, Subramaniam GK, Kumar AS, Airan B, Singh R, Tal-
war S, Seth S, Mishra PK, Pradeep KK, Sathia S, Venugopal P: Peri-
cardiectomy for constrictive pericarditis: a clinical,
echocardiographic, and hemodynamic evaluation of two sur-
gical techniques.  Ann Thorac Surg 2006, 81:522-529.
10. Bertog SC, Thambidorai SK, Parakh K, Schoenhagen P, Ozduran V,
Houghtaling PL, Lytle BW, Blackstone EH, Lauer MS, Klein AL: Con-
strictive pericarditis: etiology and cause-specific survival
after pericardiectomy.  J Am Coll Cardiol 2004, 43:1445-1452.Page 2 of 2
(page number not for citation purposes)


Constrictive pericarditis is an easily overlooked cause of right heart failure: a case report

Crossref

CB: Constrictive pericarditis and restrictive cardiomyopathy: evaluation with MR imaging. Radiology

Constrictive pericarditis: etiology and cause-specific survival after pericardiectomy.

Diastolic heart failure: restrictive cardiomyopathy, constrictive pericarditis, and cardiac tamponade: clinical and echocardiographic evaluation. Cardiol Rev

Difference in the respiratory variation between pulmonary venous and mitral inflow Doppler velocities in patients with constrictive pericarditis with and without atrial fibrillation.

Marsalese DL: Constrictive pericarditis versus restrictive cardiomyopathy: challenges in diagnosis and management. Cardiol Rev

NB: Septal bounce, vena cava plethora, and pericardial adhesion: informative two-dimensional echocardiographic signs in the diagnosis of pericardial constriction.

SH: Constrictive pericarditis masquerading as chronic idiopathic pleural effusion: importance of physical examination.

Sturridge MF: Constrictive epicarditis following Coxsackie virus infection. Thorax

Tajik AJ: Constrictive pericarditis in the modern era: evolving clinical spectrum and impact on outcome after pericardiectomy. Circulation

Venugopal P: Pericardiectomy for constrictive pericarditis: a clinical, echocardiographic, and hemodynamic evaluation of two surgical techniques. Ann Thorac Surg

file:///data/core-remote/dit/data/Springer-OA/pdf/9c9/aHR0cDovL2xpbmsuc3ByaW5nZXIuY29tLzEwLjExODYvMTc1Ny0xNjI2LTEtMjcucGRm.pdf

Constrictive pericarditis is an easily overlooked cause of right heart failure: a case report

Abstract

Similar works

Full text

Available Versions

Springer - Publisher Connector

Crossref