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Chapter 9
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Diseases Mimicking Constrictive
Pericarditis: Salient Features andNovel
Strategies ofManagement
9.1 Introduction
9.1.1 Clinical Features
The diagnosis of chronic constrictive pericarditis and its individual predictive ranking continues to be a challenge because its physical ndings and haemodynamics
mimic restrictive cardiomyopathy, tropical endomyocardial brosis and a few other
diseases.
About 50% of patients presenting with cardiac failure have normal ejection fraction, an entity known as cardiac failure with preserved ejection fraction. Hypertension
is the commonest cause of cardiac failure with preserved ejection fraction. In about
10–20% of cases, such a clinical presentation could be due to constrictive pericarditis, restrictive cardiomyopathy, cardiac amyloidosis, and Budd-Chiari syndrome.
Other rare causes are cirrhosis of liver with portal hypertension, and chronic pulmonary embolism [1–24, 68–70, 73–124, 135–159, 161–179, 181–198].
Understanding the pathophysiology of chronic constrictive pericarditis and integrating the results of non-invasive and invasive techniques are the Rosetta stone in
the differential diagnosis of constrictive pericarditis and related disorders such as
endomyocardial brosis, restrictive cardiomyopathy, post cardiac transplant
allograft rejection, and Budd-Chiari syndrome [1–71, 73–124, 135–159, 161–179,
181–198]. New echocardiographic techniques such as tissue Doppler imaging, two-
dimensional speckle tracking echocardiography, dual source computed- tomography,
and tagged cine-magnetic resonance imaging with the analysis of phase contrast
angiography sequences are promising novel approaches [72, 199–235, 238–249,
253–288]. In this chapter, we provide step-wise algorithms based on clinical pic-
ture, echocardiography, and multimodality imaging for differentiating them, and
novel management strategies of important disease entities.
Ltd. 2023
U. K. Chowdhury, L. K. Sankhyan, Surgical Treatment of Chronic Constrictive
Pericarditis, https://doi.org/10.1007/978-981-99-5808-5_9
143© The Author(s), under exclusive license to Springer Nature Singapore Pte

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9 Diseases Mimicking Constrictive Pericarditis: Salient Features and Novel Strategies…
Because the management of constrictive pericarditis and restrictive cardiomyopathy are radically different, it is extremely important to recognize the two entities.
Restrictive cardiomyopathy used to be relatively rare and caused largely by amyloidosis. It is becoming more common because of an epidemic of obesity and metabolic syndrome.
The clinical features of constrictive pericarditis and restrictive cardiomyopathy
overlap in many respects. Pulmonary congestion is usually absent in constrictive
pericarditis but is present in restrictive cardiomyopathy. Paradoxical pulse and
Kussmaul’s sign are present in approximately one-third of the cases of constrictive
pericarditis. A pericardial knock points to constriction, but a prominent third heart
sound in restrictive cardiomyopathy can be confusing [25–31, 95–97, 105, 125,
126, 138, 200, 202].
Although ndings on electrocardiogram and chest roentgenogram are nonspecic, a calcied pericardium indicates constriction; patchy endomyocardial calcication and cardiomegaly suggest endomyocardial brosis/restrictive
cardiomyopathy, whereas low voltage QRS complex suggests amyloidosis [5, 25–
31, 52, 81, 95–97, 105, 125, 126, 128, 138, 139, 200–202, 238].
On speckle tracking echocardiography, patients with constrictive pericarditis
have normal longitudinal restoration mechanics but impaired circumferential or
rotational mechanics, which is the opposite of patients with restrictive cardiomyopathy and endomyocardial brosis [176, 212, 258].
Based on the echocardiographic features, an algorithm has been suggested by the
American Society of Echocardiography and European Association of
Echocardiography (now European Association for Cardiovascular Imaging) for the
differentiation of constriction versus restriction [161–166].
The algorithm utilizes the ve features: (i) mitral inow E/A ratio more than 0.8
with dilated inferior caval vein as the rst step, (ii) ventricular septal motion—septal bounce, (iii) medial tissue Doppler e′, (iv) medial e′ versus lateral e′, and (v)
hepatic vein reversal in expiration versus inspiration [36, 65, 82–87, 106, 107, 138,
161–166, 176, 239, 258].
9.1.2 Echo Doppler Signs ofConstriction
• Septal bounce/septal shudder
• Medial mitral inow variation of more than 25%
• Mitral e′>9cm/sec
• e′ ratio of medial to lateral >0.91
• Hepatic vein—expiratory diastolic reversal ratio >0.79
• Pericardial thickness on transesophageal echocardiography >4mm
• Strain: Longitudinal strain is preserved, and circumferential strain is reduced
Medial mitral annular e′>9cm/sec combined with enhanced respiratory variation
represents a robust combination for diagnosis of constrictive pericarditis with

9.1 Introduction
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greater than 90% sensitivity [189]. An e′ velocity greater than 8cm/sec excludes
restrictive cardiomyopathy [36, 65, 106, 107, 139, 161–166, 176, 203–205, 239,
258]. However, respiratory variation is seen in only two-thirds of patients with con-
strictive pericarditis. Patients with restrictive cardiomyopathy usually have thickened ventricles [36, 65, 82–87, 106, 107, 138, 161–166, 176, 239, 258].
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9.1.3 Cardiac Computed Tomography
Cardiac computed tomography is a valuable tool for assessing pericardial thickness
and calcication [2, 259]. A thickened pericardium (more than 4mm) is suggestive
of constriction, although constrictive pericarditis can be present without pericardial
thickening in upto 20% of cases [240].
Computed tomography can give additional information on chamber size, inferior
caval venous size and pericardial effusion. ECG-gated images can identify the septal bounce [75].
9.1.4 Cardiac Magnetic Resonance
The following information can be obtained from cardiac magnetic resonance
imaging:
• Pericardial thickness: A thickness greater than 4mm is abnormal.
• Left atrial enlargement: The left atrium is enlarged more than right atrium (LA:
RA >2.0) in constrictive pericarditis. Biatrial enlargement is common in restric-
tive cardiomyopathy [37, 38, 107].
• Cine magnetic resonance imaging: Increased ventricular coupling with inspira-
tory attening of septum can be quantied in short axis cardiac magnetic reso-
nance. A septal excursion greater than 12% is specic for constrictive
pericarditis.
• Tagged cine MR: Detects pericardial adhesion and immobility of pericardial-
myocardial interface in constrictive pericarditis [86, 285].
• Delayed enhancement of pericardium is seen in constriction indicating pericar-
dial inammation.

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9 Diseases Mimicking Constrictive Pericarditis: Salient Features and Novel Strategies…
9.1.5 Cardiac Catheterization Data
Before the advent of echo Doppler, haemodynamic catheterization data remained
the gold standard for conrming the diagnosis of constriction. Haemodynamic differentiation of constrictive pericarditis from restrictive cardiomyopathy may be difcult (Table9.1).
9.2 Endomyocardial Fibrosis
9.2.1 Denition
Endomyocardial brosis is also known as Davies disease. In this type of restrictive
cardiomyopathy, there occurs scarring and deposition of brous tissue in the endocardium and subendocardial myocardium of right, left, or both ventricles that leads
to restriction of ventricular lling and diastolic dysfunction [1, 9, 25, 141, 217, 241,
254, 255].
9.2.2 Epidemiology
It is common in the poor inhabitants of the tropics with most of the 780 cases
reported in the last 2 decades originating from Uganda, Nigeria, Ivory Coast, India
(Kerala), and Brazil [1, 9, 25, 141, 217, 241, 254, 255].
It accounted for 15% of mortality due to heart failure in Uganda, 22% in Nigeria,
20% of heart failure patients aged below 40years in Ivory Coast, and 2.5% of
patients aged below 40years attending cardiac referral services in Kerala [6, 7, 39,
99, 217, 241].
The average age of newly diagnosed cases among the 123 new cases at Sree
Chitra Thirunal Institute of Medical Sciences and Technology in 1991–2001 was
33years as compared to 25years among the 295 new cases in the 1976–1990 series.
Three percent were aged below 10years, and 12% were between 11 and 20years
[200, 254, 255]. There is scanty evidence to suggest an ethnic or racial predisposition [107, 199, 200, 218].
9.2.3 Causation: Evolving Concepts
Although the aetiopathogenesis of tropical endomyocardial brosis is unknown, the
following hypotheses have been put forward as plausible explanations:
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