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Chapter 9
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Diseases Mimicking Constrictive Pericarditis: Salient Features andNovel Strategies ofManagement
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 [124, 6870, 73124, 135159, 161179, 181198].
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 [171, 73124, 135159, 161179,
181198]. 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, 199235, 238249,
253288]. 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
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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 [2531, 9597, 105, 125,
126, 138, 200, 202].
Although ndings on electrocardiogram and chest roentgenogram are non­specic, a calcied pericardium indicates constriction; patchy endomyocardial cal­cication and cardiomegaly suggest endomyocardial brosis/restrictive cardiomyopathy, whereas low voltage QRS complex suggests amyloidosis [5, 25
31, 52, 81, 9597, 105, 125, 126, 128, 138, 139, 200202, 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 [161166].
The algorithm utilizes the ve features: (i) mitral inow 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, 8287, 106, 107, 138,
161166, 176, 239, 258].
9.1.2 Echo Doppler Signs ofConstriction
• Septal bounce/septal shudder
• Medial mitral inow variation of more than 25%
• Mitral e>9cm/sec
• e ratio of medial to lateral >0.91
• Hepatic vein—expiratory diastolic reversal ratio >0.79
• Pericardial thickness on transesophageal echocardiography >4mm
• Strain: Longitudinal strain is preserved, and circumferential strain is reduced
Medial mitral annular e>9cm/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 8cm/sec excludes restrictive cardiomyopathy [36, 65, 106, 107, 139, 161166, 176, 203205, 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, 8287, 106, 107, 138, 161166, 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 calcication [2, 259]. A thickened pericardium (more than 4mm) 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 4mm 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 quantied in short axis cardiac magnetic reso-
nance. A septal excursion greater than 12% is specic 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 inammation.
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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 conrming the diagnosis of constriction. Haemodynamic dif­ferentiation of constrictive pericarditis from restrictive cardiomyopathy may be dif­cult (Table9.1).
9.2 Endomyocardial Fibrosis
9.2.1 Denition
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 40years in Ivory Coast, and 2.5% of patients aged below 40years 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 33years as compared to 25years among the 295 new cases in the 1976–1990 series. Three percent were aged below 10years, and 12% were between 11 and 20years [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: