Constrictive pericarditis: Clinical features and causes

INTRODUCTION — The clinical presentation and pathophysiology of constrictive pericarditis and effusive-constrictive pericarditis are reviewed here. Related issues are discussed separately.

●(See "Constrictive pericarditis: Diagnostic evaluation".)

●(See "Constrictive pericarditis: Management and prognosis".)

●(See "Pericardial effusion: Approach to diagnosis" and "Cardiac tamponade".)

●(See "Acute pericarditis: Clinical presentation and diagnosis" and "Acute pericarditis: Treatment and prognosis" and "Recurrent pericarditis".)

DEFINITIONS

●Constrictive pericarditis – Constrictive pericarditis is a clinical syndrome in which an inelastic thickened pericardium restricts cardiac filling [1-3]. The course of this condition may be chronic or transient:

•Chronic constrictive pericarditis – Most constrictive pericarditis has a chronic course, with constriction persisting for greater than three to six months. Pericardial effusion is rarely a prominent feature of chronic constrictive pericarditis. (See 'Constrictive pericarditis' below.)

•Transient constrictive pericarditis – In a minority of cases of constrictive pericarditis, the constriction resolves spontaneously or with medical therapy, with most such cases resolving within three to six months. Cases of transient constrictive pericarditis commonly present as effusive-constrictive pericarditis. (See 'Transient constrictive pericarditis' below.)

●Effusive-constrictive pericarditis – Effusive-constrictive pericarditis is a clinical syndrome caused by constrictive pericarditis and coexisting (often hemodynamically significant) pericardial effusion [4-8]. This syndrome overlaps with transient constrictive pericarditis. (See 'Effusive-constrictive pericarditis' below.)

PERICARDIAL PHYSIOLOGY — The 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. In the absence of pericardial disease, the pericardium stretches to accommodate physiologic changes in cardiac volume (but is noncompliant if its limited reserve volume is exceeded). In the absence of pericardial disease, intrathoracic and right heart pressures decrease during inspiration, leading to an increase in venous return to the right heart and a transient increase in right ventricular chamber size. Because the normal pericardium accommodates the increased venous return by expanding, this increase in venous return does not impair left ventricular (LV) filling.

PATHOPHYSIOLOGY

Constrictive pericarditis — With pericardial constriction, 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 accommodate cardiac volume changes.

The key pathophysiologic features of constrictive pericarditis, which are largely responsible for physical examination, hemodynamic, and imaging findings, are elevated venous pressure, greatly enhanced ventricular interdependence, and the dissociation of intracardiac and intrathoracic pressures.

●Limited late diastolic filling – With constrictive pericarditis, early diastolic filling is even more rapid than in the absence of pericardial disease. Compression does not occur until the cardiac volume approaches the limits of the inelastic pericardium, which begins in middiastole. As a result, virtually all ventricular filling occurs in early diastole, with little or no filling subsequently. As constrictive pericarditis becomes more severe, ventricular volumes and stroke volumes are reduced.

●Dissociation of intracardiac and intrathoracic pressures – The thickened, rigid pericardium prevents the normal inspiratory decrease in intrathoracic pressure from being transmitted to the heart chambers, although this is not present in all cases. The rigid pericardium also does not expand to accommodate increased venous return to the right heart during inspiration. Thus, pulmonary venous pressure, but not LV pressure, declines during inspiration, leading to a reduction in LV volume because of a decrease in the transpulmonary gradient.

●Enhanced ventricular interdependence – The right and left ventricles are interdependent, as these chambers share a septum, epicardial circumferential myocytes, and pericardial space [9]. In the setting of constrictive pericarditis, the external constraint of the pericardium enhances ventricular interdependence (ie, the hemodynamics of the left and right ventricles are influenced by each other to a much greater degree than in the nondiseased state). With enhanced ventricular interdependence, the right ventricular volume expands via shifting of the interventricular septum toward the LV. (See "Constrictive pericarditis: Diagnostic evaluation", section on 'Cardiac catheterization' and "Constrictive pericarditis: Diagnostic evaluation", section on 'Doppler findings'.)

The hemodynamic findings and distinction between constrictive pericarditis and restrictive cardiomyopathy (algorithm 1 and table 1) are discussed in more detail elsewhere. (See "Differentiating constrictive pericarditis and restrictive cardiomyopathy".)

Effusive-constrictive pericarditis — In effusive-constrictive pericarditis, the noncompliant pericardium abnormally restrains the cardiac volume and also elevates the pressure in the pericardial fluid, such that tamponade can occur with a relatively small pericardial effusion [4-7,10]. This results in a variable hemodynamic picture with features ranging from predominantly those of cardiac tamponade to those of constrictive pericarditis. Most cases present with mixed features. (See 'Effusive-constrictive pericarditis' below.)

INCIDENCE AND CAUSES

Constrictive pericarditis — Constrictive pericarditis can occur after virtually any pericardial disease process [1,2,11]. The etiology of constrictive pericarditis varies widely depending on the population studied, with idiopathic or posttreatment (eg, postoperative or postradiation) causes being more common in resource-abundant countries and infectious etiologies being more prominent in resource-limited countries. The following causes of constrictive pericarditis were identified in case series from tertiary care centers primarily in resource-abundant countries [5,12-16]:

●Idiopathic or viral – Constrictive pericarditis was attributed to idiopathic or viral causes in 42 to 61 percent of cases.

While constrictive pericarditis and recurrent pericarditis are both commonly attributed to idiopathic or viral causes, constrictive pericarditis is rarely a sequela of recurrent idiopathic pericarditis.

●Post-cardiac surgery – 11 to 37 percent. Cases of constrictive pericarditis following orthotopic heart transplant have also been reported [17].

●Post-radiation therapy – 2 to 31 percent, primarily after Hodgkin disease or breast cancer.

●Systemic rheumatic diseases (also known as autoimmune diseases) – 3 to 7 percent. Constrictive pericarditis is a rare complication of these disorders. (See "Pericardial involvement in systemic autoimmune diseases".)

●Postinfectious (purulent pericarditis including tuberculosis) – 3 to 15 percent.

Tuberculosis is the leading cause of constrictive pericarditis in resource-limited countries; it is now a rare cause in resource-abundant countries [3]. Tuberculosis should be considered as a possible etiology among patients at high risk of developing active tuberculosis, including those in or from endemic areas and those with human immunodeficiency virus (HIV) infection. (See "Tuberculous pericarditis".)

Whipple disease (caused by Tropheryma whipplei infection) is an uncommon cause of constrictive pericarditis [18].

●Miscellaneous causes (malignancy, trauma, drug-induced, asbestosis, sarcoidosis, uremic pericarditis) – 1 to 10 percent. These are all rare causes of constrictive pericarditis.

In addition, patients with a newly recognized systemic multiorgan fibroinflammatory disease associated with elevated serum levels of immunoglobulin G4 (IgG4) may present with constrictive pericarditis; histopathologically, the pericardium is fibrotic with IgG4-positive lymphoplasmacytic infiltration [19].

Another rare cause of constrictive pericarditis is Myhre syndrome [20].

The risk of constrictive pericarditis following a first episode of acute pericarditis was assessed in 500 patients prospectively studied over a mean follow-up of six years (24 to 120 months) [21]. Constrictive pericarditis developed in 2 of 416 patients with idiopathic/viral pericarditis (0.48 percent) and 7 of 84 patients with a nonviral/nonidiopathic etiology (8.3 percent). The incidence rates of constrictive pericarditis were:

●Idiopathic/viral – 0.76 cases per 1000 person-years

●Autoimmune/pericardial injury syndrome – 4.40 cases per 1000 person-years

●Neoplastic pericarditis – 6.33 cases per 1000 person-years

●Tuberculous pericarditis – 31.65 cases per 1000 person-years

●Other purulent pericarditis – 52.75 cases per 1000 person-years

Transient constrictive pericarditis — A subset of patients with constrictive pericarditis undergoes spontaneous resolution of constrictive pericarditis or responds to medical therapy [1,22,23]. As an example, in a series of 212 patients with echocardiographic findings of constrictive pericarditis, 17 percent had follow-up studies showing resolution at an interval ranging from two months to two years [23]. Some degree of effusion is common in patients with transient constrictive pericarditis, so there is some overlap between this syndrome and effusive-constrictive pericarditis.

The causes of transient constrictive pericarditis are similar to those for effusive-constrictive pericarditis (see 'Effusive-constrictive pericarditis' below), as there is overlap between these conditions. In a series of 36 patients with transient constrictive pericarditis, the most common causes were pericardial inflammation after pericardiotomy (25 percent of cases); infection (viral, bacterial, or tuberculous), idiopathic, systemic rheumatic disease, trauma, and malignancy accounted for the remaining cases [23]. An effusive-constrictive process was identified in the majority (67 percent) of these cases.

Effusive-constrictive pericarditis — Effusive-constrictive pericarditis appears to be relatively uncommon but may be increasing in prevalence, although there are only limited published data.

●In a series of 95 patients undergoing surgery for constrictive pericarditis, 24 percent were diagnosed with effusive-constrictive pericarditis [5].

●In series of patients undergoing pericardiocentesis (of which all [6] or many had cardiac tamponade [24]), coexistent effusive-constrictive pericarditis was diagnosed in 8 [6] and 16 percent [24].

The prevalence of effusive-constrictive pericarditis differs among the various etiologies of pericarditis. Effusive-constrictive pericarditis is much more common among cases of tuberculous pericarditis than among cases of idiopathic pericarditis [25,26]. In a series of 68 patients with tuberculous pericardial effusion, effusive-constrictive disease was present in just over half [27].

In studies from resource-abundant countries, the etiology of effusive-constrictive pericarditis has most commonly been idiopathic, reflecting the frequency of idiopathic pericardial disease in these countries [5,6,26]. Other causes include radiation, malignancy, chemotherapy, postsurgical and post-cardiac injury pericardial disease, infection (tuberculosis and other purulent pericarditis), and end-stage kidney disease [26].

However, the relative frequency of causes of effusive-constrictive pericarditis has varied considerably among individual case series. Thus, in one report of 33 cases of effusive-constrictive pericarditis, the most frequent cause was procedure-related hemopericardium (33 percent), followed by idiopathic (27 percent), post-cardiac surgery (18 percent), postviral (9 percent), malignancy (6 percent), and other (6 percent) [24].

CLINICAL MANIFESTATIONS

Constrictive pericarditis

Clinical presentation — Patients with constrictive pericarditis typically present with one or both of the following constellations of symptoms and signs [1]:

●Volume overload – Symptoms related to volume overload range from peripheral edema to anasarca.

●Decreased cardiac output – Symptoms related to diminished cardiac output in response to exertion include fatigability and dyspnea on exertion.

In a series of 135 patients, 67 percent of patients presented with symptoms of heart failure, 8 percent with chest pain, 6 percent with abdominal symptoms, 4 percent with atrial arrhythmia, and 5 percent with symptoms ascribed to decreased cardiac output (eg, dyspnea, fatigue, and reduced exercise capacity) [12]. (See "Cardiac tamponade" and "Heart failure: Clinical manifestations and diagnosis in adults", section on 'Clinical presentation'.)

The history elicited from a patient with suspected constrictive pericarditis should include responses to focused questions directed at potential underlying causes (eg, history of prior malignancy with thoracic radiation treatment, prior cardiothoracic surgery, chest trauma, etc).

Key signs of constrictive pericarditis — The vast majority of patients with constrictive pericarditis display elevated jugular venous pressure (JVP) on physical examination. Other important but less common features on the physical examination include pulsus paradoxus, Kussmaul sign, a pericardial knock, peripheral edema, ascites, and/or cachexia.

●Elevated JVP – Elevated JVP has been reported in as many as 93 percent of patients with surgically confirmed constrictive pericarditis [12]. The characteristics of the jugular venous waveforms in patients with constrictive pericarditis are different from those in healthy patients and in patients with other types of cardiac disease (figure 1). The x and y troughs are more prominent than the a and v peaks, and the inspiratory decline in venous pressure is confined to the depth of the y descent. In patients in normal sinus rhythm, the resulting waveform is typically W- or M-shaped. (See "Examination of the jugular venous pulse" and "Constrictive pericarditis: Diagnostic evaluation", section on 'Cardiac catheterization'.)

JVP may be in the normal range in patients with early or mild constrictive pericarditis. In addition, occult constrictive pericarditis has been described in patients who are volume depleted; in this setting, JVP may rise only after volume expansion [28]. (See 'Occult constrictive pericarditis' below.)

●Pulsus paradoxus – Pulsus paradoxus (an exaggerated drop in systolic blood pressure greater than 10 mmHg during inspiration) occurs in less than 20 percent of patients with constrictive pericarditis (waveform 1) [12,29]. It is more common in patients with effusive-constrictive pericarditis with coexisting pericardial effusion and cardiac tamponade and it may be more common in patients with coexisting pulmonary disease. (See "Pulsus paradoxus in pericardial disease".)

●Kussmaul sign – Kussmaul sign (the lack of an inspiratory decline in JVP) is present in patients with constrictive pericarditis, but does not distinguish constrictive pericarditis from severe tricuspid valve disease or right-sided heart failure. While case series have noted incidence rates of 13 to 21 percent, Kussmaul sign is much more common in our experience [5,12]. (See "Examination of the jugular venous pulse".)

●Pericardial knock – A medium frequency accentuated heart sound occurring slightly earlier than an S3 may be audible and rarely is palpable. A pericardial knock was reported in 47 percent of patients with constrictive pericarditis in one series [12]. Sixteen percent of patients in the same series had a pericardial friction rub.

●Other findings – Profound cachexia, peripheral edema, ascites, pulsatile hepatomegaly (part of the syndrome of congestive hepatopathy), and pleural effusion are common findings with more severe constrictive pericarditis.

Occult constrictive pericarditis — A syndrome of occult constrictive pericarditis has been described, but few reports are available. The largest series, published in 1977, described 19 patients with chest pain, dyspnea, and fatigue presenting with initially normal physical and hemodynamic findings [28]. Most of these patients had prior history and/or clinical findings suggestive of pericardial disease, and subsequent hemodynamic evaluation after saline infusion (waveform 2) was consistent with unmasking of constrictive pericarditis with volume loading.

Effusive-constrictive pericarditis — Patients with effusive-constrictive pericarditis can present with clinical features of pericardial effusion or constrictive pericarditis or both [6,26]. Cardiac tamponade may or may not be present.

Patients with effusive-constrictive pericarditis generally have a subacute presentation with symptoms lasting days or weeks [26], although more acute or chronic presentations have been described [6]. Acute cases may present with symptoms and signs of cardiac tamponade (such as hypotension) while subacute cases may present with fatigue, shortness of breath, and lower extremity edema [26]. Pleuritic chest pain and fever are common [6]. Common physical signs include jugular venous distention and hepatomegaly [6].

A number of clinical clues suggest that a patient with suspected constrictive pericarditis may have effusive-constrictive pericarditis:

●Pulsus paradoxus is often present; this finding is relatively uncommon in classic constrictive pericarditis because the inspiratory decline in intrathoracic pressure is not transmitted to the right heart chambers. (See "Constrictive pericarditis: Diagnostic evaluation", section on 'Echocardiography'.)

●A pericardial knock is absent.

●The y descent is less marked than expected. (See "Constrictive pericarditis: Diagnostic evaluation", section on 'Cardiac catheterization'.)

●Kussmaul sign is frequently absent.

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 5^th to 6^th 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 10^th to 12^th 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

●Pathophysiology

•Constrictive pericarditis – With pericardial constriction, 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). The key pathophysiologic features of constrictive pericarditis, which are largely responsible for physical examination, hemodynamic, and imaging findings, are elevated venous pressure, greatly enhanced ventricular interdependence, and dissociation of intracardiac and intrathoracic pressures. Pericardial effusion is not a prominent feature of constrictive pericarditis. (See 'Constrictive pericarditis' above.)

•Effusive-constrictive pericarditis – In this condition, a variable-size pericardial effusion is present, and the noncompliant pericardium both constricts the cardiac volume and also elevates the pressure of the pericardial fluid, such that tamponade can occur with a relatively small pericardial effusion. This results in a variable hemodynamic picture with most cases presenting with mixed features of cardiac tamponade and constrictive pericarditis. (See 'Effusive-constrictive pericarditis' above.)

●Etiology

•Constrictive pericarditis – This condition can occur after virtually any pericardial disease process, with the etiologies varying widely depending on the population studied, with idiopathic or posttreatment (eg, postoperative or postradiation) causes being more common in resource-abundant countries and infectious etiologies (eg, tuberculosis) being more prominent in resource-limited countries. The risk of constrictive pericarditis is highest after purulent or tuberculous pericarditis. (See 'Constrictive pericarditis' above.)

•Transient constrictive pericarditis – The causes of this condition are similar to those for effusive-constrictive pericarditis, as there is overlap between these conditions. (See 'Transient constrictive pericarditis' above.)

•Effusive-constrictive pericarditis – This condition has a wide variety of causes; the frequency distribution differs between resource-abundant versus resource-limited countries. In resource-abundant countries, idiopathic cases are the most common. In resource-limited countries, tuberculosis is most common.

Effusive-constrictive pericarditis is more common among cases of tuberculous pericarditis than among cases of idiopathic pericarditis. Pericardial inflammation after pericardiotomy is also a common cause of transient constrictive pericarditis and/or effusive-constrictive pericarditis. (See 'Effusive-constrictive pericarditis' above.)

●Clinical presentation

•Constrictive pericarditis – Patients with constrictive pericarditis typically present with symptoms related to fluid overload (eg, peripheral edema, anasarca) and/or diminished cardiac output (eg, fatigability, dyspnea on exertion). Most patients have elevated jugular venous pressure (JVP). Other important but less common features include pulsus paradoxus, Kussmaul sign, a pericardial knock, edema, ascites, and/or cachexia. (See 'Constrictive pericarditis' above.)

•Effusive-constrictive pericarditis – Patients with this condition usually present with clinical features of pericardial effusion (with or without cardiac tamponade) or constrictive pericarditis or both. In a patient with suspected constrictive pericarditis, clinical findings suggestive of effusive-constrictive pericarditis include: presence of pulsus paradoxus, absence of a pericardial knock, a less marked y descent than typical for pericardial constriction, and absence of Kussmaul sign. (See 'Effusive-constrictive pericarditis' above and "Examination of the jugular venous pulse".)