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Differentiating constrictive pericarditis and restrictive cardiomyopathy

Authors: Paul Sorajja, MD, Brian D Hoit, MD
Section Editor: Martin M LeWinter, MD
Deputy Editor: Brian C Downey, MD, FACC

All topics are updated as new evidence becomes available and our peer review process is complete.

Literature rev iew current through: Mar 2020. | This topic last updated: Mar 25, 2020.

INTRODUCTION

Constrictive pericarditis is the result of scarring and consequent loss of the normal elasticity of the pericardial sac.
This leads to impairment of ventricular filling in mid and late diastole. As a result, the majority of ventricular filling
occurs rapidly in early diastole and the ventricular volume does not increase after the end of the early filling period.

Restrictive cardiomyopathy is characterized by a nondilated rigid ventricle, resulting in severe diastolic dysfunction
and restrictive filling that produces hemodynamic changes similar to those in constrictive pericarditis.

Constrictive pericarditis and restrictive cardiomyopathy both lead to diastolic heart failure with normal (or near
normal) systolic function, and characteristically abnormal ventricular filling that results in similar clinical and
hemodynamic features. However, because of their markedly different treatments, differentiating between the two
conditions is critical. In some patients, the correct diagnosis may be readily suggested from the history or routine
diagnostic testing. In others, however, this differentiation cannot be diagnosed before biopsy or even surgical
exploration.

The distinction between constrictive pericarditis and restrictive cardiomyopathy will be reviewed here. The basic
aspects of constrictive pericarditis and idiopathic restrictive cardiomyopathy are discussed separately. (See
"Constrictive pericarditis" and "Idiopathic restrictive cardiomyopathy".)

PATHOPHYSIOLOGY OF RESPIRATORY EFFECTS

An understanding of ventricular volume constraints and ventricular interaction is key in any discussion of the
hemodynamic differences between constrictive pericarditis and restrictive cardiomyopathy.

In patients with constrictive pericarditis, total cardiac volume is fixed by the noncompliant pericardium. The septum
is not involved and can therefore bulge toward the left ventricle when left ventricular volume is less than that on the
right. As a result, ventricular interdependence is greatly enhanced. This bulging may be seen on echocardiography,
or in some cases, on cardiac magnetic resonance imaging [1]. In addition, changes in intrathoracic pressure are
not transmitted to the cardiac chambers because of obliteration of the pericardial space. (See "Constrictive
pericarditis".)

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In restrictive cardiomyopathy, on the other hand, pericardial compliance is normal. Left ventricular systolic function
is normal, or in severe cases, may even be reduced. The respiratory variation in intrathoracic pressure is
transmitted normally to the cardiac chambers.

The different effects of respiration on ventricular filling between the two diseases may be explained by the following
mechanisms:

● In patients with constrictive pericarditis, the pulmonary capillary wedge pressure is influenced by the inspiratory
fall in intra-thoracic pressure, while the left ventricular pressure is shielded from respiratory pressure
variations by the pericardial scar. Thus, inspiration lowers the pulmonary capillary wedge pressure, and
presumably left atrial pressure, but not left ventricular diastolic pressure, thereby decreasing the pressure
gradient for ventricular filling. The less favorable filling pressure gradient during inspiration explains the
decline in filling velocity. Reciprocal changes occur in the velocity of right ventricular filling [2,3]. These changes
are mediated by the ventricular septum, not by increased systemic venous return.

● In patients with restrictive cardiomyopathy, inspiration lowers pulmonary wedge and left ventricular diastolic
pressures equally, thereby leaving the pressure gradient for ventricular filling and filling velocity virtually
unchanged.

A lower left ventricular filling pressure gradient with constrictive pericarditis also leads to a delay in mitral valve
opening and therefore, a longer isovolumic relaxation time during inspiration. This inspiration decline in the filling
gradient is seen in constrictive pericarditis but not restrictive cardiomyopathy.

HISTORY AND PHYSICAL EXAMINATION

In the evaluation of a patient with suspected constrictive pericarditis or restrictive cardiomyopathy, the history can
provide important clues to other systemic disorders which could predispose to either condition (algorithm 1). In
addition, while most patients with constrictive pericarditis and restrictive cardiomyopathy present with symptoms of
heart failure, subtle differences in physical findings may suggest a particular diagnosis.

History — In the evaluation of constrictive pericarditis versus restrictive cardiomyopathy, the history is most valuable
in identifying a systemic disorder which can predispose to either constrictive pericarditis or restrictive
cardiomyopathy:

● A prior history of pericarditis, trauma, cardiac surgery, or a systemic disease that affects the pericardium (eg,
tuberculosis, connective tissue disease, malignancy) makes the diagnosis of constrictive pericarditis more
likely. (See "Etiology of pericardial disease" and "Constrictive pericarditis", section on 'History'.)

● A history of an infiltrative disease that may involve the heart muscle (eg, amyloidosis, sarcoidosis) favors the
diagnosis of restrictive cardiomyopathy. (See "Clinical manifestations and diagnosis of cardiac sarcoidosis"
and "Idiopathic restrictive cardiomyopathy", section on 'Clinical presentation' and "Cardiac amyloidosis: Clinical
manifestations and diagnosis".)

However, prior thoracic radiation treatment (and rarely amyloidosis) can result in either constrictive pericarditis,
restrictive cardiomyopathy, or a condition with features of both constrictive pericarditis and restrictive
cardiomyopathy. (See "Cardiotoxicity of radiation therapy for breast cancer and other malignancies" and
"Constrictive pericarditis".)

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Physical examination — The vast majority of patients with both constrictive pericarditis and restrictive
cardiomyopathy display elevated jugular venous pressure (JVP) on physical examination (figure 1). From the JVP
waveform alone, it is not possible to distinguish between constrictive pericarditis, restrictive cardiomyopathy,
tricuspid regurgitation with an enlarged compliant right atrium, or right heart failure (eg, due to right ventricular
infarction or pulmonary hypertension). The contour of the jugular venous pulse in all these conditions is dominated
by a deep, steep Y descent. (See "Examination of the jugular venous pulse".)

Additional physical examination findings in patients with constrictive pericarditis or restrictive cardiomyopathy can
include:

● Kussmaul's sign (the lack of an inspiratory decline in JVP)

● Pulsus paradoxus (uncommon)
● Peripheral edema
● Ascites and hepatomegaly
● Pleural effusions.

Approximately 50 percent of patients with constrictive pericarditis may present with a pericardial knock (an
accentuated heart sound occurring slightly earlier than a third heart sound, which may be audible and rarely is
palpable), which is not expected in restrictive cardiomyopathy [4]. Conversely, an audible S3 is frequently present in
persons with restrictive cardiomyopathy because of the abrupt cessation of the rapid ventricular filling; this is not
usually present in constrictive pericarditis. (See "Constrictive pericarditis", section on 'Physical examination' and
"Idiopathic restrictive cardiomyopathy", section on 'Clinical presentation'.)

NON-INVASIVE TESTING

Patients suspected of having either constrictive pericarditis or restrictive cardiomyopathy, based on history and
physical examination, should undergo initial evaluation with electrocardiography, chest radiography, and
echocardiography (algorithm 1). While a particular diagnosis is often made following echocardiography, patients
commonly undergo cardiac catheterization, during which invasive hemodynamic evaluation can help elucidate the
correct diagnosis [5]. In most patients, particularly those with prior radiation exposure, there is a role for computed
tomography (CT) or cardiac magnetic resonance (CMR) imaging [5]. Both CT and CMR provide additional detailed
anatomic information about adjacent vascular structures and an accurate measurement of pericardial thickness.

Electrocardiogram — The electrocardiogram may be helpful in distinguishing constrictive pericarditis from

restrictive cardiomyopathy. Depolarization abnormalities (such as bundle branch block), ventricular hypertrophy,
pathologic Q waves, or impaired atrioventricular conduction strongly favor restrictive cardiomyopathy. Low voltage
and isolated repolarization abnormalities can occur in both conditions, although the latter are more common in
constrictive pericarditis. Atrial fibrillation is common in the late stages of both diseases. (See "Constrictive
pericarditis", section on 'Electrocardiography' and "Idiopathic restrictive cardiomyopathy", section on
'Electrocardiogram'.)

Plasma BNP — Plasma concentrations of B type natriuretic peptide (BNP) are increased in numerous conditions,
most notably in patients with left ventricular dysfunction. As a result, plasma BNP has been used in the diagnosis
of dyspnea and to assess the efficacy of therapy and estimate prognosis in patients with heart failure. (See
"Natriuretic peptide measurement in heart failure".)

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