Introduction
Background
Constrictive pericarditis (CP) is a reduction in the elasticity, or stiffening, of the pericardium, a sack-like covering that surrounds the heart, resulting in impaired filling of the heart with blood. The symptoms of CP, which may include exercise intolerance, liver failure, dyspnea, and renal failure, appear insidiously (see Clinical Details) and may be misleading.
Patients respond dramatically to a complete surgical pericardiectomy when it is performed early in the disease process; therefore, it is important to consider CP when making the diagnosis. Anatomic imaging findings, such as calcifications and thickening of the pericardium, may be present, but the most reliable and most important findings are related to the filling pattern of the heart. The severity of the clinical symptoms is best correlated with findings from dynamic observation of the blood flow and from findings relative to the obstruction and poor filling of the right-sided cardiac chambers.
Various imaging methods (echocardiography, magnetic resonance imaging [MRI], computed tomography [CT]) can depict changes in cardiac chamber volumes throughout the cardiac cycle, as well as the timing of fluid motion backward into veins and forward into arteries. Both restrictive and constrictive diseases exhibit an abrupt reduction in filling, increased back pressure, and impaired stroke volume (volume of fluid ejected per heartbeat). Catheterization can be used to observe the pressure levels in various chambers during filling. The influence of respiration on filling contributes important diagnostic information, both for imaging and for catheterization.
In CP, the size of the heart is usually normal; flattening of the right ventricle and curving of the intraventricular septum to the left are sometimes found. The azygos vein and superior vena cava (SVC) are commonly dilated.
Restrictive disease (stiff muscle), such as that in dialysis patients with chronic amyloidosis, mimics the physiologic pattern of constrictive disease (stiff muscle). Amyloidosis does not cause constrictive pericarditis, but it can cause a restrictive cardiomyopathy that can mimic the clinical, imaging, and physiologic alterations of constrictive pericarditis. Symptoms of amyloidosis do not respond to pericardiectomy. Cardiac tamponade, a condition in which fluid accumulates in the pericardial space, is also in the differential diagnosis of impaired filling; however, it is usually not difficult to distinguish tamponade from restrictive or constrictive disease on images.
Echocardiographic findings can also suggest CP. Duplex Doppler waveforms and inflow velocities across the mitral and tricuspid valves can be assessed during inspiration and expiration. This assessment is not part of the routine protocol in most echocardiography laboratories, and unless the physician is alerted to the possibility of CP, the diagnosis is almost never suspected. Dynamic respiratory criteria can also be applied during right- and left-sided heart catheterization when equalization of end-diastolic pressures in the heart chambers and characteristic venous waveforms are present.
Pathophysiology
Relevant anatomy and physiology
The pericardium normally functions to provide a friction-free surface for myocardial contraction and filling, to limit acute dilatation of the cardiac chambers, and to prevent the spread of infection or malignancy from adjacent structures. The pericardium is a 2-layered sac that encloses most of the heart, folding back at the great vessels. Its normal thickness is less than 3 mm, and the normal fluid content between the 2 layers is less than 50 mL. The pericardium limits the position and motion of the heart, limits dilatation, and provides a barrier to the spread of disease from the lungs.
CP consists of a stiffened and usually thickened pericardium caused by the activation of fibrocytic deposition of collagen. The space between the layers of the pericardium may get obliterated or divided into localized pockets. Calcification may further contribute to stiffness of the pericardium. A stiffened pericardium leads to elevation and equalization of filling pressures and pulmonary wedge pressure. Elevated venous pressure results in rapid early filling, which is curtailed abruptly by the nonexpandable pericardium. This is known as a dip-and-plateau pattern, a finding that is sensitive for constrictive pericarditis but is not specific to it. It also occurs in restrictive cardiomyopathy and, sometimes, in severe chronic obstructive pulmonary disease (COPD).
Neck veins show a prominent Y descent, and the X descent may also be prominent (W pulsation pattern). This is distinct from findings in cardiac tamponade, which compresses the cardiac chambers throughout diastole, blunting the Y descent. The stiffened pericardium shields the chambers from changes in intrathoracic pressure; therefore, the normal decrease in return during inspiration is not observed and, instead, may increase (Kussmaul sign). Symptoms occur as a result of elevated venous pressure (ascites, edema), pulmonary congestion (dyspnea), and reduced cardiac output (fatigue, hypotension, tachycardia).
In healthy patients, as well as in those with systolic dysfunction, the normal compliance of the pericardium allows for the transmission of intrathoracic pressures across the pericardium. During inspiration, left atrial pressure decreases because of a decrease in intrathoracic pressure. The normal compliance of the pericardium also causes the left ventricular diastolic pressure to decrease; ventricular filling is unchanged throughout the respiratory cycle.
In CP, the pericardium is rigid and does not transmit intrathoracic pressure changes to the cardiac chambers. During inspiration, left ventricular diastolic pressure does not change, and left ventricular filling is impaired. The right ventricle can fill more during inspiration because the pericardial volume is fixed and left ventricular filling is impaired. The opposite physiologic process occurs during expiration, with improved left ventricular filling, greater Doppler velocities across the mitral valve, poor filling of the right ventricle, and lower Doppler velocities across the tricuspid valve.
In patients with CP, the pericardium is usually thickened and is always rigid and noncompliant.
Etiologies
Although the etiology of pericardial thickening is unknown in many cases, viral or other infection or inflammatory processes are often implicated.
Etiologies include the following:
- Viral infection
- Tuberculosis (15% of cases)
- Surgery
- Irradiation
- Hemodialysis
- Neoplasia with pericardial infiltration
- Bacterial, fungal, or parasitic infections
- Inflammation after myocardial infarction (Dressler syndrome)
- Asbestosis
- Autoimmune diseases
Frequency
United States
There are no reliable figures for the prevalence and incidence of CP in the United States. Although CP is not a common condition, it is underrecognized and undertreated. In the United States, the most common causes of CP are idiopathic; a viral etiology is usually implicated. Radiation therapy and previous surgery are increasingly common causes. The average age of patients with CP also appears to be increasing.
International
There are no reliable figures for the prevalence and incidence of CP worldwide. Diagnosable infections, such as tuberculosis and parasitic disease (eg, echinococcal infection), are more likely causes of CP outside the United States.
Mortality/Morbidity
- CP is fatal in patients with advanced symptoms. The mortality risk is markedly increased in patients with advanced symptoms; when CP is found in its early stages, surgery should be performed. On average, 95% of patients survive surgery. Intraoperative death is usually caused by acute dilation of the cardiac chambers after the constriction has been relieved.
- Complete relief of symptoms occurs in about 50% of survivors. Approximately 10% of patients have persistent symptomatic heart failure (New York Heart Association functional class III or IV) and poor late outcomes, particularly when residual myocardial dysfunction is also present. Patients may have liver failure and massive ascites.
Race
CP does not appear to have any racial predisposition.
Sex
Most surgically proved CP series report a male-to-female ratio of about 2:1; however, these series have included only a small number of patients.
Age
CP can occur in individuals of any age, but it is rare in children. The mean age of adult patients with CP at diagnosis appears to be increasing.
Anatomy
The pericardium is a fibroserous membrane that consists of an external fibrous layer, which is stout and dense, and an inner serous layer, which invests the heart and is reflected on the inner surface of the pericardium. Thus, the inner serous layer has a visceral component and a parietal component, which give the pericardium a friction-free surface that allows myocardial contraction and filling. The normal pericardium is 1 mm thick on CT scans or MRIs; thickening greater than 3 mm is abnormal.
Presentation
The symptoms of CP appear insidiously, with patients displaying peripheral edema, anasarca, and elevated right-sided heart pressures. Patients frequently present with symptoms that are not thought to be due to cardiac disease; these include exercise intolerance, dyspnea, liver failure, and renal failure. The diagnosis is rarely considered by the referring physician, who usually may think that the symptoms are due to another disease process. Patients may present with increasing weight gain, cardiac cirrhosis, and massive ascites.The physician is most likely to refer patients with these symptoms to the radiologist for abdominal ultrasonography, abdominal and pelvic CT, or hepatobiliary scanning, thinking that the patient's symptoms are related to a liver disorder.
CP responds dramatically to complete surgical pericardiectomy when it is performed early in the disease process; therefore, it is important to consider CP when making the diagnosis.
Preferred Examination
In the minority of patients in whom the diagnosis of CP is suspected on a clinical basis, the first test should be plain posteroanterior (PA) and lateral chest radiography. If the results show characteristic pericardial calcification, the diagnosis of CP is essentially established. As many as 50% of patients with CP have pericardial calcification. The classic and most common distribution is largely over the right ventricle; this appearance can be considered pathognomonic.
If the plain radiographic findings are negative and clinical suspicion of CP is present, echocardiography with an evaluation of Doppler inflow velocities of the mitral and tricuspid valves during inspiration and expiration should be performed. Ventricular interdependence is present in all patients with significant constrictive physiologies.
No imaging test is definitive for the diagnosis of CP in all patients who have the disease. In a patient in whom the clinical findings are suggestive of the disease, noninvasive imaging findings that are consistent with CP are usually sufficient to prompt definitive surgery or an invasive evaluation in the catheterization laboratory.
The diagnosis of CP is more difficult in patients without calcification. Pericardial thickening of 4 mm or more on CT scans or MRIs is considered evidence of CP for patients with the appropriate symptoms; however, focal thickening of 4 mm or more commonly occurs in patients without physiologic or clinical evidence of constrictive physiology. The converse is also true. Rarely, a constrictive physiology can be present without abnormal thickening of the pericardium.
Limitations of Techniques
All of the noninvasive imaging techniques have limitations in the diagnosis of CP. Ultimately, the diagnosis is made at surgery; however, in the appropriate patient, CT, ultrasonography, conventional radiography, MRI, echocardiography, and right- and left-sided heart catheterization results can be highly suggestive of CP. A major problem is differentiating restrictive cardiomyopathy from CP. Furthermore, it is difficult to preoperatively predict which patients are likely to respond to total pericardiectomy.
Differential Diagnoses
Other Problems to Be Considered
Davies disease
Endomyocardial fibroelastosis
Cardiac amyloidosis
Idiopathic interstitial fibrosis
More on Constrictive Pericarditis |
 Overview: Constrictive Pericarditis |
| Imaging: Constrictive Pericarditis |
| Follow-up: Constrictive Pericarditis |
| Multimedia: Constrictive Pericarditis |
| References |
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References
Aqel RA, Lloyd SG, Gupta H, Zoghbi GJ. Three-vessel coronary artery disease, aortic stenosis, and constrictive pericarditis 27 years after chest radiation therapy: a case report. Heart Surg Forum. 2006;9(4):E728-30. [Medline].
Correa SD, Amsterdam EA. Images in cardiovascular medicine. Constrictive pericarditis. Circulation. Mar 3 1998;97(8):806. [Medline].
Di Cesare E. MRI of the cardiomyopathies. Eur J Radiol. Jun 2001;38(3):179-84. [Medline].
Fernando Guadalajara J, Vera-Delgado A, Gaspar-Hernandez J. Echocardiographic Aspects of Restrictive Cardiomyopathy: Their Relationship with Pathophysiology. Echocardiography. Apr 1998;15(3):297-314. [Medline].
Gomes Ferreira SM, Gomes Ferreira A Jr, do Nascimento Morais A. Constrictive chronic pericarditis in children. Cardiol Young. Mar 2001;11(2):210-3. [Medline].
Gujral V, Lopez-Candales A, Abdelhadi R, Crock F, Gulyasy B, et al. Constrictive pericarditis: an unusual clinical entity suggested during dobutamine stress echocardiography. Int J Cardiol. Nov 10 2006;113(2):e58-61. [Medline].
Guneri S, Nazli C, Kinay O. Chylous ascites due to constrictive pericarditis. Int J Card Imaging. Feb 2000;16(1):49-54. [Medline].
Inglessis II, Dec GW. Constrictive pericarditis. Curr Treat Options Cardiovasc Med. Jun 1999;1(1):63-71. [Medline].
Johnston SD, Johnston PW, O'Rourke D. Carcinoid constrictive pericarditis. Heart. Nov 1999;82(5):641-3. [Medline].
Kameda Y, Funabashi N, Kawakubo M, Uehara M, Hasegawa H, Kobayashi Y, et al. Heart in an eggshell--Eggshell appearance calcified constrictive pericarditis demonstrated by three-dimensional images of multislice computed tomography. Int J Cardiol. Jan 22 2007;[Medline].
Karadede A, Ulgen MS, Temamogullari AV. A complicated case of pericardial hydatid cyst manifesting as constrictive pericarditis. Can J Cardiol. May 2000;16(5):673-6. [Medline].
Langer C, Butz T, Horstkotte D. Multimodality in imaging calcific constrictive pericarditis. Heart. Sep 2006;92(9):1289. [Medline].
Marshall A, Ring N, Lewis T. Constrictive pericarditis: lessons from the past five years' experience in the South West Cardiothoracic Centre. Clin Med. Nov-Dec 2006;6(6):592-7. [Medline].
McCully RB, Higano ST, Oh JK. Diagnosis of constrictive pericarditis. Circulation. May 11 1999;99(18):2476. [Medline].
Mehta A, Mehta M, Jain AC. Constrictive pericarditis. Clin Cardiol. May 1999;22(5):334-44. [Medline].
Moosdorf R. Indications, results, and pitfalls in the surgery of constrictive pericarditis. Herz. Dec 2000;25(8):794-8. [Medline].
Palka P, Lange A, Donnelly JE. Differentiation between restrictive cardiomyopathy and constrictive pericarditis by early diastolic doppler myocardial velocity gradient at the posterior wall. Circulation. Aug 8 2000;102(6):655-62. [Medline].
Pepine CJ, Nissen SE, eds. CathSAP: Cardiac Catheterization and Interventional Cardiology Self-assessment Program. American College of Cardiology. 1999.
Ridruejo E, Laurini J, Cingolani O. [Fulminant hepatic failure. Atypical form of cardiac failure presentation]. Medicina (B Aires). 2000;60(4):491-4. [Medline].
Robles P, Rubio A, Olmedilla P. Value of multidetector cardiac CT in calcified constrictive pericarditis for pericardial resection. Heart. Aug 2006;92(8):1112. [Medline].
Trent JC, Lau TK, Kawashima A. Constrictive pericarditis from an embolized hypodermic needle: radiographic, CT and MR imaging findings. Int J Card Imaging. Apr 2000;16(2):117-23. [Medline].
Van der Merwe S, Dens J, Daenen W. Pericardial disease is often not recognised as a cause of chronic severe ascites. J Hepatol. Jan 2000;32(1):164-9. [Medline].
Watanabe A, Sakata J, Kawamura H. Primary pericardial mesothelioma presenting as constrictive pericarditis: a case report. Jpn Circ J. May 2000;64(5):385-8. [Medline].
Wettstein M, Haussinger D. [Constrictive pericarditis--a differential diagnosis in therapy refractory ascites]. Dtsch Med Wochenschr. Dec 1 2000;125(48):1462-5. [Medline].
Zimand S, Benjamin P, Frand M. Constrictive pericarditis presented by generalized edema (anasarca). Isr Med Assoc J. Apr 2000;2(4):316-7. [Medline].
Further Reading
Keywords
CP, constriction of the cardiac chambers, pericardium, thickened pericardium, pericardial thickening, stiffened pericardium, pericardial stiffening
