INTRODUCTION — The normal pericardium consists of a visceral monolayer adherent to the epicardial surface of the heart, and the parietal pericardium, a fibroelastic layer that is continuous with the visceral pericardium and forms a sac surrounding the heart that contains a thin layer of fluid. When larger amounts of fluid accumulate (pericardial effusion) or when the pericardium becomes scarred and inelastic, one of three pericardial compressive syndromes may occur:
●Cardiac tamponade – Cardiac tamponade, which may be acute or subacute, is characterized by the accumulation of pericardial fluid under pressure. Variants include low pressure (occult) and regional tamponade.
●Constrictive pericarditis – Constrictive pericarditis is the result of scarring and consequent loss of the normal elasticity of the pericardial sac. Pericardial constriction is typically chronic, but variants include subacute, transient, and occult constrictive pericarditis.
●Effusive-constrictive pericarditis – Effusive-constrictive pericarditis is characterized by underlying constrictive physiology with a coexisting pericardial effusion, often with cardiac tamponade. This usually results in a mixed hemodynamic picture with features of both constrictive pericarditis and cardiac tamponade. The course is generally subacute. Such patients may be mistakenly thought to have only cardiac tamponade; however, elevation of the right atrial and pulmonary wedge pressures after drainage of the pericardial fluid points to the underlying constrictive process. (See "Variants of constrictive pericarditis", section on 'Effusive-constrictive pericarditis'.)
In each of these compressive syndromes, cardiac filling is impeded by an external force. The normal pericardium can stretch to accommodate physiologic changes in cardiac volume. However, after its reserve volume is exceeded, the pericardium markedly stiffens. In both typical constrictive pericarditis and effusive-constrictive pericarditis, cardiac filling is impeded by an external force (ie, the virtually inelastic parietal and/or visceral pericardial tissue, which is thickened, fibrotic, and sometimes calcified). This results in a markedly impaired ability to adapt to volume changes. As a result, an important pathophysiologic feature of constrictive pericarditis is greatly enhanced ventricular interdependence, in which the hemodynamics of the left and right heart chambers are directly influenced by each other to a much greater degree than normal. (See 'Hemodynamic evaluation' below.)
The diagnosis and treatment of constrictive pericarditis are reviewed here. Issues related to variants of constrictive pericarditis, cardiac tamponade, and the evaluation and management of pericardial diseases that do not compromise hemodynamics, are discussed separately. (See "Constrictive pericarditis: Clinical features and causes" and "Variants of constrictive pericarditis" and "Cardiac tamponade" and "Diagnosis and treatment of pericardial effusion" and "Acute pericarditis: Clinical presentation and diagnosis".)
EVALUATION — All patients with suspected constrictive pericarditis, based on history and physical examination, should undergo initial evaluation with electrocardiography (ECG), chest radiography, and echocardiography [1,2]. Subsequent evaluation may include one or more of the following, depending upon the diagnostic quality of the initial echocardiogram, relevant history (eg, prior radiation therapy), and the potential for surgical intervention:
●Cardiac computed tomography (CT).
●Cardiovascular magnetic resonance (CMR) imaging.
●Invasive hemodynamic evaluation during cardiac catheterization (often with concurrent coronary angiography to define the patient's coronary anatomy prior to possible surgical intervention).
Initial evaluation in all patients
Electrocardiography — There are no pathognomonic ECG findings in constrictive pericarditis. Nonspecific ST and T wave changes and tachycardia are common, and low voltage may sometimes be present. In advanced cases, atrial fibrillation is common due to increased atrial pressures. In a series of 143 patients with surgically confirmed constrictive pericarditis, 22 percent had atrial fibrillation and 27 percent had low voltage [3].
Chest radiograph — The presence of pericardial calcification by chest radiograph, especially in conjunction with the appropriate clinical presentation, is highly consistent with constrictive pericarditis. However, the majority of patients with constrictive pericarditis will not have pericardial calcification, so its absence does not exclude constrictive pericarditis.
Chest radiographs that demonstrate a ring of calcification around the heart, best seen on lateral or anterior oblique projections, strongly suggest constrictive pericarditis in patients with symptoms of right-sided HF (image 1) [1]. Pericardial calcification can occur in the absence of constrictive pericarditis, but is usually less dense and has a patchy distribution. Tuberculous constrictive pericarditis, which is very uncommon in the developed world, has an especially high incidence of calcification. A retrospective review of 135 patients with constrictive pericarditis confirmed surgically or at autopsy reported that 36 patients (27 percent) had pericardial calcification [4]. Compared with those without calcification, patients with calcification had the following:
●A greater likelihood of having idiopathic pericardial disease (67 versus 21 percent)
●A longer duration of symptoms
●A greater likelihood of having a pericardial knock, larger atria, and/or atrial arrhythmias
●A higher perioperative mortality, but the same long-term survival
Echocardiography — Transthoracic echocardiography (TTE) is an essential diagnostic test in patients being evaluated for constrictive pericarditis. The American College of Cardiology (ACC)/American Heart Association (AHA)/American Society of Echocardiography (ASE) guidelines and the European Society of Cardiology (ESC) guidelines recommend the use of echocardiography for the evaluation of all patients with suspected pericardial disease [1,5]. Two-dimensional and M-mode echocardiography allow structural visualization while Doppler echocardiography provides hemodynamic information.
Two-dimensional and M-mode — One or more of the following findings may be seen by two-dimensional or M-mode echocardiography in patients with constrictive pericarditis [3,6-8] (see "Echocardiographic evaluation of the pericardium"):
●Increased pericardial thickness – TTE demonstrates increased pericardial thickness with or without calcification in approximately 40 percent of patients. Mildly increased pericardial thickening is often missed, and false positive results suggesting calcification can be obtained if the gain setting is too high. In comparison, measurements of pericardial thickness by transesophageal echocardiography (TEE) correlate strongly with that obtained by CT [9].
●Two-dimensional echocardiography – Two-dimensional echocardiography may reveal [10,11]:
•Dilatation of the inferior vena cava and hepatic veins (plethora) with absent or diminished inspiratory collapse.
•Moderate biatrial enlargement (severe enlargement is more compatible with restrictive cardiomyopathy).
•A sharp halt in ventricular diastolic filling (corresponding to the end of early rapid diastolic filling as noted on Doppler).
•Septal bounce with abrupt transient rightward movement of the interventricular septum.
•Hypermobile atrioventricular valves.
•An abnormal contour between the posterior LV and left atrial walls.
●M-mode – No sign or a combination of signs on M-mode is diagnostic of constrictive pericarditis. However, a normal M-mode study with none of the following findings virtually rules out the diagnosis of constrictive pericarditis [12]:
•Abrupt posterior motion of the ventricular septum in early diastole with inspiration (septal shudder and bounce) caused by underfilling of the LV, due to the decreased pulmonary vein-left atrial gradient with inspiration.
•Systemic venous return is not increased with inspiration due to volume constraints imposed by the abnormal pericardium [13].
•Notching of the ventricular septal tracing in early diastole or with atrial systole due to a transient reversal of ventricular septal transmural pressure at these times in the cardiac cycle [14].
•Rapid posterior motion of the LV posterior wall in early diastole followed by a flattening of the wall in mid-diastole (also a feature of restrictive cardiomyopathy).
•Premature opening of the pulmonic valve as RV diastolic pressure rises above pulmonary arterial pressure.
In a reported series of 143 patients with surgically confirmed constrictive pericarditis, 138 underwent TTE [3]. Increased pericardial thickness >2 mm was seen in 82 percent, abnormal septal motion in 49 percent, and atrial enlargement in 61 percent.
Doppler echocardiography — Doppler echocardiography is critical for the diagnosis of constrictive pericarditis. The following findings on Doppler echocardiography are suggestive of constrictive pericarditis:
●Abnormal filling of the ventricles during early diastole. An increased E velocity of RV and LV inflow is seen due to the abnormally rapid early diastolic filling associated with the combination of a small ventricular volume and rapid recoil. The early diastolic Doppler tissue velocity at the mitral annulus (E') is prominent (unless slowed by mitral annular calcification). The usually positive linear relation between E/E' and left atrial pressure, which is useful for assessing left atrial pressure in cardiomyopathy, is reversed (annular paradox) in constrictive pericarditis [15]. (See "Differentiating constrictive pericarditis and restrictive cardiomyopathy".)
●Annular early diastolic (E') velocities are lower in constrictive pericarditis secondary to surgery or radiation than in other etiologies. The mitral lateral/medial E' ratio is reversed in the majority of patients with constrictive pericarditis. After pericardiectomy, annular velocities are reduced, and the lateral/medial E' ratio normalizes [16].
●The propagation velocity of early diastolic transmitral flow on color M-mode is normal or increased [17].
●Pronounced respiratory variation in ventricular filling – Mitral inflow velocity falls as much as 25 to 40 percent and tricuspid velocity greatly increases (>40 to 60 percent) in the first cardiac cycle following inspiration. The respiratory variation in pulmonary venous flow is even more pronounced [18]. These phenomena, which are manifestations of ventricular interdependence, are not present in either normal subjects or patients with restrictive cardiomyopathy [19]. Increased respiratory variation of mitral inflow may be missing in patients with markedly elevated left atrial pressure, but can sometimes be elicited in such patients by preload reduction with semi-recumbent (rather than supine) positioning or diuretic administration [20].
In an ASE consensus statement, calculation of percentage respiratory variation for mitral and tricuspid inflow was standardized as:
[(inflow velocity expiration minus inflow velocity inspiration) / inflow velocity expiration] X 100
Changes of 25 and 40 percent (mitral and tricuspid inflows, respectively) or greater are considered significant [21].
●Hepatic venous flow reversal increases with expiration, reflecting the ventricular interdependence and the dissociation of intracardiac and intrathoracic pressures [6].
In a study of 130 patients with surgically confirmed constrictive pericarditis who were compared with 36 patients with restrictive myocardial disease or severe tricuspid regurgitation, respiration-related ventricular septal shift, preserved or increased medial annular E', and hepatic vein expiratory diastolic reversals (which collectively are sometimes referred to as the Mayo criteria) were independently associated with constrictive pericarditis [22]. The finding of ventricular septal shift in combination with either a medial E' ≥9 cm/second or an expiratory diastolic reversal ratio ≥0.79 was 87 and 91 percent sensitive and specific for constrictive pericarditis, respectively [22].
Myocardial strain imaging — 2D speckle tracking echocardiography has emerged as a valuable method that helps distinguish constrictive pericarditis from restrictive cardiomyopathy. In constrictive pericarditis, global longitudinal strain is generally preserved (although tethering of the pericardium may result in reduced lateral wall but preserved septal longitudinal strain), whereas circumferential strain and torsion are reduced [23]. A normalization of the lateral to septal longitudinal strain ratio following antiinflammatory treatment may identify patients with transient constrictive pericarditis [24]. (See "Differentiating constrictive pericarditis and restrictive cardiomyopathy", section on 'Speckle tracking echocardiography (STE)'.)
Plasma BNP — Plasma brain natriuretic peptide (BNP) is frequently obtained in the evaluation of patients with dyspnea. While we do not recommend obtaining a BNP specifically to diagnosis constrictive pericarditis, many patients will have had this test performed. There is no threshold for BNP which confirms or excludes the diagnosis of constrictive pericarditis. (See "Natriuretic peptide measurement in heart failure".)
Among patients with cardiomyopathy, BNP is released in response to LV dysfunction and wall stretch. However, wall stretch is limited in constrictive pericarditis by the thickened stiff pericardium. These physiologic differences suggest that the elevation in plasma BNP in constrictive pericarditis should be much less than in other types of cardiomyopathy (eg, restrictive cardiomyopathy), which is consistent with findings of small studies showing BNP values to be lower in patients with constrictive pericarditis. (See "Differentiating constrictive pericarditis and restrictive cardiomyopathy", section on 'Plasma BNP'.)
Additional testing in select patients
Cross-sectional imaging — Patients with a suspected but unconfirmed diagnosis of constrictive pericarditis should undergo cross-sectional imaging of the chest with either cardiac CT or CMR imaging [25]. Most commonly, cross-sectional imaging is performed when the initial echocardiographic examination yields indeterminate (or non-diagnostic) findings, or when technical limitations result in inadequate information from the echocardiogram. However, cross-sectional imaging may also be performed in some patients in whom the diagnosis is confirmed during initial testing, in particular to further assess the anatomy for planned surgical intervention or to assess the presence of concurrent coronary artery disease. The decision to pursue cross-sectional imaging with cardiac CT or CMR should be made on a case-by-case basis, taking into account individual patient characteristics (eg, prior radiation exposure, presence of calcium, desire to image the coronary arteries, etc) as well as institutional availability and/or expertise with a particular imaging technique.
CT scan — Computed tomographic (CT) scanning of the heart, obtained by rapid scanning gated to the cardiac cycle, can provide additional detailed anatomic information about adjacent vascular structures and the extent of pericardial thickening, calcification, and scarring. CT scanning is useful in the diagnosis of constrictive pericarditis and can provide additional data to guide perioperative management decisions. CT may assist in the preoperative planning of pericardiectomy given its ability to identify critical vascular structures [26]. Additionally, in selected patients, CT offers the ability to:
●Assess the extent of lung injury in patients with previous radiation exposure
●Evaluate the location and extent of pericardial calcification
●Avoid the need for invasive coronary angiography in those with normal CT coronary angiography
Findings on CT include increased pericardial thickness and calcification [1]. In a review of 143 patients with surgically confirmed constrictive pericarditis, 97 underwent preoperative CT imaging [3]. A pathologically thickened pericardium (>4 mm) was seen in 72 percent and pericardial calcification in 25 percent. A normal appearance, or nonvisualization, of the pericardium does not rule out constrictive pericarditis. Other findings on CT scanning which suggest constrictive pericarditis include dilatation of the inferior vena cava, deformed ventricular contours, and angulation of the ventricular septum. Nonvisualization of the posterolateral LV wall on dynamic CT may indicate myocardial fibrosis or atrophy and is associated with a poor surgical outcome [27].
CT imaging may also be used to examine the effect of cardiac motion transmitted to the surrounding pulmonary parenchyma. Failure of the immediately adjacent pulmonary structures to pulsate during the cardiac cycle, in the presence of a regionally or globally thickening pericardium, is virtually diagnostic of constrictive pericarditis. PET/CT imaging using 18F-labelled fluorodeoxyglucose can effectively identify pericardial inflammation and predict the response to antiinflammatory therapy in transient constrictive pericarditis [28]. (See "Variants of constrictive pericarditis", section on 'Transient constrictive pericarditis'.)
Magnetic resonance imaging — As with cardiac CT, CMR imaging can provide additional detailed anatomic information and the extent of pericardial thickening, calcification, and scarring that might alter the decision to proceed with surgery based on the likelihood of resolution with medical therapy alone. (See "Variants of constrictive pericarditis", section on 'Transient constrictive pericarditis'.)
Gated CMR imaging provides direct visualization of the normal pericardium, which is composed of fibrous tissue and has a low MRI signal intensity [29]. CMR is advocated by some as the diagnostic procedure of choice for the detection of certain pericardial diseases, including constrictive pericarditis [30-32]. Characteristic CMR features in patients with constrictive pericarditis include increased pericardial thickening, ventricular interdependence, and dilatation of the inferior vena cava, an indirect sign of impaired RV diastolic filling [1]. (See "Clinical utility of cardiovascular magnetic resonance imaging".)
While CT is superior to CMR in detecting calcification, CMR better differentiates small effusions from pericardial thickening. CMR also has the potential to resolve hemodynamic events such as septal bounce and to better identify pericardial inflammation and pericardial-myocardial adherence [26]. CMR with late gadolinium enhancement (LGE) of the pericardium is fairly common but not universal in patients with constrictive pericarditis [33,34]. Persons with constrictive pericarditis and pericardial LGE had greater fibroblast proliferation, chronic inflammation, and pericardial thickening compared with those without LGE [34]. Increased T2 STIR pericardial signal suggests pericardial edema and provides evidence of acute pericardial inflammation [35]. Pericardial LGE and prominent T2 STIR might also be predictors of reversibility of constrictive pericarditis following treatment with antiinflammatory agents [33,36]. (See 'Treatment' below.)
Real-time, free-breathing phase contrast flow can detect the characteristic respirophasic changes in mitral and tricuspid flow; in a study of 16 patients, the sensitivity and specificity of respiratory variation of transmitral flow >25 percent and transtricuspid flow >45 percent were 100 and 100 percent and 90 and 88 percent, respectively [37].
Hemodynamic evaluation — Invasive hemodynamic evaluation is occasionally needed to confirm the diagnosis of constrictive pericarditis, particularly in patients with suboptimal or nondiagnostic echocardiographic findings in whom cross-sectional imaging with cardiac CT or CMR is either unavailable or non-diagnostic [6]. Moreover, patients with diagnostic-quality echocardiographic findings may require coronary angiography for evaluation of coronary anatomy prior to pericardiectomy. In this setting, an invasive hemodynamic evaluation may also be performed. (See "Differentiating constrictive pericarditis and restrictive cardiomyopathy".)
The major hemodynamic findings in patients with constrictive pericarditis include:
●Increased right atrial pressure.
●Prominent x and y descents of venous and atrial pressure tracings (figure 1). In contrast, the y descent of diastolic ventricular filling is absent in tamponade. (See "Cardiac tamponade", section on 'Physical findings'.)
●Kussmaul sign (the lack of an inspiratory decline or an inspiratory increase in central venous pressure).
●Increased RV end-diastolic pressure, usually to a level one-third or more of RV systolic pressure.
●"Square root" signs in the RV and LV diastolic pressure tracings (an early diastolic dip followed by a plateau of diastasis; the last stage of diastole just before contraction), often with an absent a wave [1]. This finding, also called dip and plateau, reflects rapid early diastolic filling of the ventricles, followed by lack of additional filling due to compression in mid and late diastole (figure 2). The plateau configuration (of the dip and plateau pattern) may be diminished or absent in patients who are tachycardic due to the shortening of diastole.
●A greater inspiratory fall in pulmonary capillary wedge pressure compared with LV diastolic pressure.
●Equalization of LV and RV diastolic plateau pressure tracings, with little separation with exercise, since filling, and therefore diastolic pressure, in both ventricles is constrained by the inelastic pericardium [1]. In some patients, this finding is seen only during inspiration (waveform 1).
●Mirror-image discordance between RV and peak LV systolic pressures during inspiration, another sign of increased ventricular interdependence. During peak inspiration, an increase in RV pressure occurs when LV pressure is lowest [38]. These changes can be detected by both invasive hemodynamic monitoring and Doppler echocardiography.
In a series of 143 patients with surgically confirmed constrictive pericarditis, 78 underwent cardiac catheterization [3]. The mean right atrial pressure was 21 mmHg. A dip and plateau pattern was seen in 77 percent, diastolic equalization of pressures in 81 percent, and respiratory variation in the RV-LV pressure relationship in 44 percent.
Right atrial pressure (RAP) fairly closely approximates pericardial pressure, while pulmonary artery wedge pressure (PAWP) approximates LVEDP (in the absence of mitral stenosis). It has been hypothesized that a larger contribution of pericardial restraint, as shown by a greater RAP/PAWP ratio, could help identify patients with "pure" constriction (ie, minimal contribution of myocardial disease to symptoms) in whom pericardiectomy is more likely to be beneficial. In a single-center retrospective study of 113 patients with surgically confirmed constrictive pericarditis, those with values above the median (mean RAP/PAWP ratio 0.86) had greater pericardial thickness (6.6 versus 4.5 mm) and greater postoperative survival [19,39]. While potentially useful, the RAP/PCWP ratio should not be used in isolation and requires further validation. (See "Differentiating constrictive pericarditis and restrictive cardiomyopathy".)
In contrast, mirror image discordance, measured as the inspiratory increase in the ratio of areas under the RV and LV systolic pressure curves (systolic area index), was 97 and 100 percent accurate, respectively, for differentiating 59 cases of surgically proven constrictive pericarditis from 41 cases of restrictive cardiomyopathy [40].
DIAGNOSIS — The diagnosis of constrictive pericarditis is usually made using echocardiography in patients with history and physical findings resulting in a high clinical suspicion. While there is no single diagnostic echocardiographic parameter, one or more of the following findings are commonly seen:
●Increased pericardial thickness
●Abnormal septal motion (ie, "bounce")
●Pronounced respiratory variation in ventricular filling and mitral/tricuspid valve inflow
●Bi-atrial enlargement
●Dilated inferior vena cava and hepatic veins
In patients with poor quality echocardiographic images, or in patients whose echocardiogram is non-diagnostic, the diagnosis is most commonly made by invasive hemodynamic assessment during cardiac catheterization. While there is no single diagnostic hemodynamic parameter, one or more of the following findings are commonly seen:
●Prominent x and y descents of venous and atrial pressure tracings (figure 1)
●Kussmaul sign (the lack of an inspiratory decline or an inspiratory increase in central venous pressure)
●"Square root" signs in the RV and LV diastolic pressure tracings (an early diastolic dip followed by a plateau of diastasis; the last stage of diastole just before contraction), also called dip and plateau
●Mirror-image discordance between peak or mean RV and LV systolic pressures during inspiration, another sign of increased ventricular interdependence
●Increased RV end-diastolic pressure, usually to a level one-third or more of RV systolic pressure
●Increased RA pressure
In patients who undergo CT or CMR, failure of the immediately adjacent pulmonary structures to pulsate during the cardiac cycle, in the presence of a regionally or globally thickening pericardium, is virtually diagnostic of constrictive pericarditis, but is not commonly seen. Additionally, one or more of the following findings are commonly present:
●Increased pericardial thickness and calcification
●Dilatation of the inferior vena cava
●Deformed ventricular contours with or without angulation of the ventricular septum
●Late gadolinium enhancement (LGE) of the pericardium (CMR only)
●Respirophasic changes in mitral and tricuspid flow (seen on real-time free-breathing phase contrast CMR)
DIFFERENTIAL DIAGNOSIS — The symptoms and physical findings in patients with constrictive pericarditis are similar to those of several other disorders. Most importantly, the clinician must distinguish between constrictive pericarditis, which is treated by pericardiectomy; cardiac tamponade, which is treated by drainage of the pericardial effusion; and disorders such as restrictive cardiomyopathy and cirrhosis, which require markedly different treatment [6].
Comparison with restrictive cardiomyopathy — Patients with constrictive pericarditis and restrictive cardiomyopathy have elevated left and right sided filling pressures, often of equal magnitude, and usually have normal systolic ventricular function. While the history, physical examination, and imaging findings may suggest a particular diagnosis (algorithm 1), constrictive pericarditis and restrictive cardiomyopathy are usually distinguished by hemodynamic findings from Doppler echocardiography and/or cardiac catheterization [1]. This is discussed in more detail separately. (See 'Doppler echocardiography' above and "Differentiating constrictive pericarditis and restrictive cardiomyopathy".)
The history and physical examination may provide helpful clues to the diagnosis. Constrictive pericarditis is suggested in a patient with prior pericarditis or a systemic disease predisposing to constrictive pericarditis (eg, tuberculosis). Restrictive cardiomyopathy is more likely in a patient with a predisposing systemic disease such as diabetes mellitus or amyloidosis. A pericardial knock favors constrictive pericarditis, but is difficult to distinguish from the third heart sound of HF. (See "Differentiating constrictive pericarditis and restrictive cardiomyopathy", section on 'History and physical examination'.)
Imaging findings may also provide clues to the diagnosis. Increased thickness or calcification of the pericardium favors the diagnosis of constrictive pericarditis, while myocardial thickening and abnormal myocardial texture suggest restrictive cardiomyopathy. Although increased pericardial thickness is a highly specific finding for the diagnosis of constrictive pericarditis, it is not present in all patients. In one series of 143 patients with surgically confirmed constrictive pericarditis, 26 patients (18 percent) had normal pericardial thickness on histopathologic examination of pericardiectomy specimens [3].
Hemodynamic findings that distinguish the two conditions include:
●Respiratory variation in ventricular inflow velocities – Doppler echocardiography reveals an abnormal increase in respiratory variation of the ventricular inflow velocities in patients with constrictive pericarditis compared with a normal pattern in patients with restrictive cardiomyopathy. (See 'Echocardiography' above.)
●Hepatic venous flow reversal – Hepatic venous flow usually reverses during expiration in constrictive pericarditis but reverses during inspiration in restrictive cardiomyopathy.
●Doppler tissue velocity – The early diastolic Doppler tissue velocity at the mitral annulus (E') is decreased (<8 cm/second) in restrictive cardiomyopathy, due to an intrinsic impairment in myocardial contraction and relaxation. In contrast, the transmitral E' is frequently increased (>12 cm/second) in constrictive pericarditis, since the longitudinal movement of the myocardium is enhanced because of constricted radial motion [15,17]. (See 'Echocardiography' above.)
While E' values <8 or >12 cm/second are highly specific for restrictive cardiomyopathy or constrictive pericarditis, respectively, many patients have an E' velocity between 8 and 12 cm/second, which is nondiagnostic. Despite excellent specificity of E' for differentiating restrictive cardiomyopathy from constrictive pericarditis, its sensitivity is more modest.
●2D speckle tracking echocardiography – In constrictive pericarditis, global longitudinal strain (GLS) is generally preserved, and the ratio of LV free wall to septal longitudinal strain and circumferential strain is reduced; by contrast, in restrictive cardiomyopathy, GLS is reduced, and circumferential strain is preserved [23]. (See "Differentiating constrictive pericarditis and restrictive cardiomyopathy", section on 'Speckle tracking echocardiography (STE)'.)
●Ventricular end-diastolic pressures – Right and left ventricular end-diastolic pressures (RVEDP and LVEDP) are equal or nearly equal in constrictive pericarditis, while LVEDP is usually higher than RVEDP in restrictive cardiomyopathy. However, in many cases of restrictive cardiomyopathy the plateau of diastolic pressure is elevated to a similar extent in both ventricles as more typically occurs in constrictive pericarditis. (See "Differentiating constrictive pericarditis and restrictive cardiomyopathy", section on 'Cardiac catheterization'.)
In the rare patient in whom the hemodynamic and imaging studies fail to establish the diagnosis, endomyocardial or pericardial biopsy may be helpful [41,42].
Comparison with cardiac tamponade — Several similarities exist in the clinical presentation of constrictive pericarditis and cardiac tamponade. However, these entities differ in their effects on diastolic ventricular filling, which can lead to different findings on physical examination, echocardiography, and invasive hemodynamic assessment. (See "Cardiac tamponade", section on 'Comparison with constrictive pericarditis'.)
Comparison with chronic liver disease — Patients with constrictive pericarditis presenting with ascites as the main manifestation are often thought to have cirrhosis, sometimes considered cryptogenic because the history does not reveal an obvious cause. Common physical examination findings in both constrictive pericarditis and chronic liver disease include peripheral edema, ascites, hepatomegaly (part of the syndrome of congestive hepatopathy), and pleural effusion. These findings may lead to the misdiagnosis of chronic liver disease.
The evaluation of jugular venous pressure (JVP) can help distinguish constrictive pericarditis from chronic liver disease. In constrictive pericarditis, there is marked elevation in JVP with characteristic changes in the morphology of the jugular venous pulsations (figure 1). In patients with cirrhosis from chronic liver disease, unless there is tense ascites, JVP is normal or only slightly elevated. JVP in patients with tense ascites should rapidly normalize following removal of ascitic fluid [43]. (See "Congestive hepatopathy" and "Clinical manifestations and evaluation of edema in adults".)
TREATMENT — The signs and symptoms of late (chronic) constrictive pericarditis (eg, anasarca, cachexia, hepatic dysfunction, atrial fibrillation, pericardial calcification, etc) are progressive and often permanent in the majority of patients unless the constrictive pericarditis is surgically treated with pericardiectomy. However, in a minority of patients with early stage (subacute) disease, symptoms and pericardial inflammation may be reversible with antiinflammatory therapy (algorithm 2).
Treatment of early (subacute) disease — For patients with early stage (subacute) constrictive pericarditis who are hemodynamically stable and without evidence of late (chronic) constrictive pericarditis, we initiate a trial of medical therapy rather than immediate pericardiectomy (algorithm 2). Detection of evidence of pericardial inflammation on CMR also supports this approach [36]. Our approach to treatment is as follows:
●Initial conservative management is similar to the treatment of acute pericarditis, typically utilizing a combination of antiinflammatory agents (colchicine and a nonsteroidal antiinflammatory drug [NSAID]) for two to three months before reassessing. (See "Acute pericarditis: Treatment and prognosis".)
●Patients with marked improvement in signs and symptoms of constrictive pericarditis and resolution of inflammation on CMR following initial treatment can typically be weaned from therapy and avoid surgical intervention.
●Patients with ongoing signs and symptoms of early stage (subacute) constrictive pericarditis or ongoing evidence of late gadolinium enhancement on CMR are treated similarly to patients with recurrent pericarditis. Such patients should receive corticosteroids or immune modulating therapy (in addition to NSAIDs and colchicine) for an additional two to three months prior to reassessing. Management of recurrent pericarditis is discussed separately. (See "Recurrent pericarditis", section on 'Treatment'.)
●Patients with refractory signs and symptoms, or those who progress and develop evidence of late (chronic) constrictive pericarditis (eg, anasarca, cachexia, atrial fibrillation, hepatic dysfunction, or pericardial calcification), should be evaluated by cardiothoracic surgery for pericardiectomy. (See 'Treatment of late (chronic) disease' below.)
Treatment of late (chronic) disease — Pericardiectomy is the only definitive treatment option for patients with late (chronic) constrictive pericarditis who have persistent and prominent symptoms [2]. Medical therapy (ie, diuretics) may be used as a temporizing measure and for patients who are not candidates for surgery [1]. While the majority of patients have a significant improvement in symptoms following pericardiectomy, there is a significant perioperative morbidity and mortality. Outcomes are best at high-volume surgical centers with greater experience performing pericardiectomy.
Removal of the thickened and inflamed pericardium can be technically challenging. Efforts should be made to remove as much of the pericardium as is technically feasible [1,3,44,45]. Most patients have relief of symptoms after pericardiectomy, with some evidence that earlier surgical intervention is associated with better outcomes [46]. In one series, New York Heart Association (NYHA) functional class improved markedly among long-term survivors (mean follow-up four years), with 69 percent free of clinical symptoms [47]. Surgical removal of the pericardium has a significant operative mortality. In one series of patients who underwent surgery between 1970 and 1985, the operative mortality was 12 percent [48]. A lower mortality rate of between 4 and 8 percent has been noted in patients who underwent pericardiectomy between 1977 and 2012 [44-47,49-53].
Due to the complex nature of the surgery and the associated operative mortality, surgery should be considered cautiously in patients with either mild or very advanced disease and in those with radiation-induced constrictive pericarditis, myocardial dysfunction, significant renal dysfunction, or mixed constrictive-restrictive disease. Such patients may not benefit from pericardiectomy:
●In patients with constrictive pericarditis, mild symptoms, and a mild to moderate increase in central venous pressure with little or no edema, the surgical risk of pericardiectomy likely outweighs any potential benefit. A trial of medical management with anti-inflammatory agents can be considered in such patients, with reassessment for pericardiectomy should symptoms progress [54]. (See 'Treatment of early (subacute) disease' above.)
●In our experience, patients with "end-stage" constrictive pericarditis derive little or no benefit from pericardiectomy and the operative risk is markedly elevated. Manifestations of end-stage disease can include cachexia, reduced resting cardiac output (cardiac index ≤1.2 L/m2 per min), hypoalbuminemia due to protein losing enteropathy, and/or liver dysfunction due to chronic congestion or cardiogenic cirrhosis.
●Symptoms may persist after successful pericardiectomy in patients with mixed constrictive-restrictive disease (eg, radiation-induced disease) because of abnormalities in intrinsic myocardial compliance. In these patients it is important to assess the extent of myocardial damage with tissue Doppler and/or endomyocardial biopsy.
Diuretics should be used sparingly with the goal of reducing elevated venous pressure, ascites, and edema while awaiting surgical intervention. This approach can help to optimize the patient's hemodynamics prior to surgery and may improve their functional status. Diuretics can also be used for the palliative control of symptoms in patients who are not candidates for surgery.
PREVENTION — Early intervention and appropriate treatment of pericardial effusions and acute pericarditis, along with prophylactic treatment in patients undergoing pericardiotomy, play a large role in preventing constrictive pericarditis. When idiopathic/viral acute pericarditis is appropriately and effectively treated, constrictive pericarditis rarely develops [55]. Similarly, the draining of pericardial effusions, in particular postoperative pericardial effusions, seemingly should reduce the likelihood of chronic constrictive pericarditis [56]. (See "Acute pericarditis: Treatment and prognosis" and "Diagnosis and treatment of pericardial effusion", section on 'Pericardial fluid drainage'.)
Additionally, in contemporary practice, prophylactic treatment to prevent postpericardiotomy syndrome has significantly reduced its occurrence, which will translate to fewer patients with late development of constrictive pericarditis. A full discussion of the approach to prevention of postpericardiotomy syndrome is presented separately. (See "Post-cardiac injury syndromes", section on 'Prevention'.)
PROGNOSIS — Long-term survival after pericardiectomy is inferior to that of an age- and sex-matched population, although in many patients this is likely related to comorbid conditions [44,46,47]. In one series, the 5- and 10-year survival rates were 78 and 57 percent, respectively [47]. Independent adverse predictors of long-term outcome included older age and worse NYHA class. In other series, independent adverse predictors have included older age, renal dysfunction, pulmonary hypertension, RV dysfunction, LV dysfunction, concomitant coronary heart disease, chronic obstructive pulmonary disease, and hyponatremia [44,57].
The etiology of the pericardial disease is also an important determinant of survival [44,47,49,50]. Prior ionizing radiation, because it may induce myocardial injury as well as pericardial disease, is associated with poorer long-term outcomes following surgery [53]. In one series, the seven-year survival rates after surgery for patients with idiopathic, postsurgical, and radiation-induced constrictive pericarditis were 88, 66, and 27 percent [44]. In two subsequent case series, the five-year survival rates after surgery for patients with idiopathic, postsurgical, and postradiation constrictive pericarditis were 80, 56, and 11 percent, and 81, 50, and 0 percent, respectively [49,50].
Preoperative indices of contraction and relaxation also predict postoperative prognosis. An observational study of 40 patients undergoing preoperative cardiac catheterization reported that patients with both an abnormal rate of LV pressure decline (- LV dP/dt <1200 mmHg/s) and an abnormal time constant of LV isovolumic relaxation (tau >50 milliseconds) required more frequent postoperative inotropic support, had higher immediate postoperative mortality, and significantly lower long-term survival (median follow-up 2.4 years) than patients with either two normal values or one abnormality [58]. Preoperative RV systolic dysfunction also appears associated with postoperative morbidity and mortality [59].
SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Pericardial disease".)
INFORMATION FOR PATIENTS — UpToDate offers two types of patient education materials, "The Basics" and "Beyond the Basics." The Basics patient education pieces are written in plain language, at the 5th to 6th grade reading level, and they answer the four or five key questions a patient might have about a given condition. These articles are best for patients who want a general overview and who prefer short, easy-to-read materials. Beyond the Basics patient education pieces are longer, more sophisticated, and more detailed. These articles are written at the 10th to 12th grade reading level and are best for patients who want in-depth information and are comfortable with some medical jargon.
Here are the patient education articles that are relevant to this topic. We encourage you to print or e-mail these topics to your patients. (You can also locate patient education articles on a variety of subjects by searching on "patient info" and the keyword(s) of interest.)
●Beyond the Basics topic (see "Patient education: Pericarditis (Beyond the Basics)")
SUMMARY AND RECOMMENDATIONS
●Diagnostic evaluation (See 'Evaluation' above.)
•Initial evaluation – All patients with suspected constrictive pericarditis should undergo initial evaluation with electrocardiography, chest radiography, and echocardiography. (See 'Initial evaluation in all patients' above.)
•Additional evaluation – Subsequent evaluation may include one or more type of cross-sectional imaging (cardiac computed tomography [CT] or cardiac magnetic resonance [CMR] imaging) or invasive hemodynamic evaluation, depending upon the diagnostic quality of the initial echocardiogram, relevant history (eg, prior radiation therapy), and the potential for surgical intervention. (See 'Additional testing in select patients' above.)
●Diagnosis – The diagnosis of constrictive pericarditis is usually made using echocardiography in patients with history and physical findings resulting in a high clinical suspicion. While there is no single diagnostic echocardiographic parameter, the presence of increased pericardial thickness, abnormal septal motion, pronounced respiratory variation in ventricular filling, biatrial enlargement, and dilated inferior vena cava and hepatic vein, typically in combination, are highly suggestive of constrictive pericarditis. In patients with poor quality echocardiographic images, or in patients whose echocardiogram is nondiagnostic, the diagnosis is most commonly made by invasive hemodynamic assessment during cardiac catheterization. (See 'Diagnosis' above.)
●Differential diagnosis – The symptoms and physical findings in patients with constrictive pericarditis are similar to those of several other disorders. Most importantly, the clinician must distinguish between constrictive pericarditis, which is treated by pericardiectomy; cardiac tamponade, which is treated by drainage of the pericardial effusion; and disorders such as restrictive cardiomyopathy and cirrhosis, which require markedly different treatment. (See 'Differential diagnosis' above.)
●Management
•Early (subacute) disease – For patients with early stage (subacute) constrictive pericarditis who are hemodynamically stable and without evidence of late (chronic) constrictive pericarditis, we initiate a trial of medical therapy rather than immediate pericardiectomy (algorithm 2). (See 'Treatment of early (subacute) disease' above.)
-Initial treatment – Initial conservative management is similar to the treatment of acute pericarditis, typically utilizing a combination of antiinflammatory agents (colchicine and a nonsteroidal antiinflammatory drug [NSAID]) for two to three months.
-Continuing evidence of disease – Patients with ongoing signs and symptoms of early stage (subacute) constrictive pericarditis or ongoing evidence of late gadolinium enhancement on CMR are treated similarly to patients with recurrent pericarditis with the addition of corticosteroids and/or immune modulating therapy. Patients with marked improvement in signs and symptoms of constrictive pericarditis and resolution of inflammation on CMR following initial treatment can typically be weaned from therapy and avoid surgical intervention.
•Late (chronic) disease – Pericardiectomy is the only definitive treatment option for patients with chronic constrictive pericarditis who have persistent and prominent symptoms (algorithm 2). Patients with markers of chronic constrictive pericarditis (eg, anasarca, cachexia, atrial fibrillation, hepatic dysfunction, or pericardial calcification) or signs of progressive systemic congestion (eg, dyspnea, unexplained weight gain, and/or a new or increased pleural effusion or ascites) should undergo earlier surgical intervention. Diuretic therapy is used only as a temporizing measure and for patients who are not candidates for surgery. (See 'Treatment of late (chronic) disease' above.)
