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Pericarditis, Constrictive-Effusive Last Updated: August 31, 2005 |
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| Synonyms and related keywords: transudative pericardial effusion, exudative pericardial effusion, sanguineous pericardial effusion, chylous pericardial effusion, chronic effusive pericarditis, visceral pericardial constriction, constrictive pericarditis
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AUTHOR INFORMATION
| Section 1 of 10   |
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| Author: Terrence X O'Brien, MD, FACC, Office of Research and Development, Ralph H Johnson Veterans Affairs Medical Center; Associate Professor, Department of Medicine, Division of Cardiology, Medical University of South Carolina Coauthor(s): D. Dirk Bonnema, MD, Research Fellow, Division of Cardiology, Department of Medicine, Medical University of South Carolina; Lonnie Nunnamker |
| Terrence X O'Brien, MD, FACC, is a member of the following medical societies:
American College of Cardiology,
American Heart Association,
American Society of Echocardiography, and
South Carolina Medical Association |
| Editor(s): Eric Vanderbush, MD, Chief, Clinical Assistant Professor, Department of Internal Medicine, Division of Cardiology, Harlem Hospital Center and Columbia University; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine;
Ronald J Oudiz, MD, Director of Pulmonary Hypertension, Associate Professor, Department of Medicine, Division of Cardiology, Harbor-UCLA Medical Center, David Geffen School of Medicine at UCLA;
Amer Suleman, MD, Consultant in Electrophysiology and Cardiovascular Medicine, Department of Internal Medicine, Division of Cardiology, Medical City Dallas Hospital;
and Michael E Zevitz, MD, Assistant Professor of Medicine, Finch University of the Health Sciences, The Chicago Medical School; Consulting Staff, Private Practice |
Disclosure
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INTRODUCTION
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Background: Effusive-constrictive pericarditis is a clinical syndrome characterized by concurrent pericardial effusion and pericardial constriction where constrictive hemodynamics are still present after the effusion is removed. Once detected, a pericardial effusion must be evaluated to determine its etiology and hemodynamic significance. Pericardial effusions vary in size and age and may be transudative, exudative, sanguineous, or chylous. An effusion persisting for months to years is chronic effusive pericarditis. The mechanism of effusive-constrictive pericarditis is thought to be visceral pericardial constriction.
Patients may present with a moderate-to-large pericardial effusion, jugular venous and arterial pressures within the reference range, with or without signs of cardiac tamponade. Symptoms are variable and may not be present at all. This syndrome can evolve as part of a clinical continuum initiated by pericarditis or a pericardial effusion, thus its etiologies mirror those of pericarditis, pericardial tamponade and chronic constrictive pericarditis (see Pericarditis, Constrictive). The hemodynamic definition of this syndrome is continued elevation of right atrial, end-diastolic right and left ventricular pressures after the removal of pericardial fluid returns the pericardial pressure to zero (or near zero).
Recognition of effusive-constrictive pericarditis is clinically important because treatment with a pericardiocentesis or a pericardial window may be inadequate. Rather, a visceral pericardiectomy may be indicated for optimal therapy since it is the visceral pericardium that is constricting.
Of importance, not all cases of effusive-constrictive pericarditis progress to chronic constrictive pericarditis. In some clinical situations, relief from the effusion is obtained by means of pericardiocentesis or a pericardial window, and medical treatment is used to manage the underlying condition. The constriction may be transitory and surgical pericardiectomy may be avoided. These situations usually occur in the first months of a chronic effusion and close monitoring is required.
The effusive-constrictive variant of pericarditis was first described in the 1960s. Hancock popularized this definition of a constrictive physiology with a coexisting pericardial effusion. In 2004, Sagrista-Sauldea reported 15 subjects from Barcelona, Spain, who were prospectively identified as having effusive-constrictive pericarditis. These individuals were among 190 consecutive subjects with clinical tamponade who underwent pericardiocentesis and concurrent catheterization. The etiologies of the effusive-constrictive pericarditis were infectious causes, irradiation, cardiac surgery, and idiopathic causes. Consistent with Hancock's data, Sagrista-Sauldea reported that most cases were due to idiopathic factors. Pathophysiology: Constrictive pericarditis and cardiac tamponade both restrict filling of the heart and raise systemic and pulmonary filling pressures. In tamponade, single forward flow occurs during systole (prominent X descent in atrial pressure tracings), whereas in constriction, a biphasic pressure tracing is greater during diastole (prominent Y descent). Patients with effusive-constrictive pericarditis may have tamponade-like pressure tracings, which change to constrictive-like tracings after pericardiocentesis. The visceral pericardial, not the parietal, is constrictive. In rare cases, a loculated effusion may lead to constriction with regional tamponade of 1 or more cardiac chambers. Almost any form of chronic pericardial effusion has the potential to organize into an effusive-constrictive state.
Effusive-constrictive pericarditis may be part of a clinical continuum. Stages of infective pericarditis have been observed that range from acute pericarditis and tamponade with effusion to constrictive pericarditis without effusion. Effusive-constrictive pericarditis is likely a middle phase in this evolution. Therefore, suspicion for this entity should be high in cases of indolent, subacute pericarditis, as well in cases of chronic pericardial effusion. Frequency:
- In the US: This is a rare disorder. As a complication of pericarditis, pericardial effusion, pericardial tamponade, or chronic constrictive pericarditis, the incidence of effusive-constrictive is proportional to the incidence of each of these entities. Cases in the United States are more often secondary to irradiation, cardiac surgery, uremia, or malignancy (see Differentials).
Mortality/Morbidity:
- The mortality of effusive-constrictive disease is directly related to its etiology. Patients with metastatic carcinoma in the pericardial space usually have a prognosis much poorer than that of patients with postviral or idiopathic pericardial effusion with constriction.
- Constrictive physiology increases the risk of morbidity, but no definitive statistics are available.
- Noncardiac metastatic effusions are often end stage, with reported mortality rates of 47% and 80% at 3 and 6 months, respectively.
Race: No reported racial predilection exists.
Sex: No reported sex predilection exists.
Age: Because the incidences of many of the diseases that can cause effusive-constrictive pericarditis occur more frequently in older age groups, an age association exists. However, this disease can affect people of any age.
History: - Symptoms can be hard to interpret but may include atypical or typical chest pain, chest heaviness or pressure, fatigability or peripheral edema.
- With more severe cases, patients may report dyspnea on exertion.
- Many patients are asymptomatic until the advanced stages of disease.
- Specific etiologies of effusive-constrictive pericarditis may have characteristic antecedent histories that may suggest pericardial disease (eg, tuberculosis, renal failure, malignancy, radiation therapy, cardiovascular surgery).
Physical: - Physical findings may be a continuum from those common with cardiac tamponade (see Cardiac Tamponade).
- Findings may include hypotension, jugular venous distension, and diminished heart sounds (classic Beck triad).
- Common findings may include pulsus paradoxus (paradoxical pulse), jugular venous pulse with a prominent X descent and absent Y descent, tachycardia, tachypnea, hepatomegaly, ascites, peripheral edema, pleural effusion (in the absence of left-sided congestive signs), or auscultation of a pericardial friction rub.
- The classic description of percussible cardiac dullness at the apex is considered unreliable.
- Careful attention to all physical findings is required to find clues as to the underlying etiology of the pericardial disease.
Causes: Because effusive-constrictive pericarditis is rare, the differential diagnosis is guided by few published series and case reports. (see Pericarditis, Constrictive). Effusive-constrictive pericarditis likely occurs at any point along a clinical continuum, from the occurrence of an effusion to the development of chronic pericardial constriction. - Neoplasm - Most commonly lung, breast, or hematologic
- Infectious disease - Particularly in immunocompromised states (most commonly tuberculosis and fungal)
- Connective tissue disease
- The etiology can often be suspected from the clinical setting in which the effusion occurs.
- The differential diagnosis of effusive-constrictive pericarditis requires a consideration of all the causes for pericardial effusions and pericardial tamponade and then a determination if the particular patient has constrictive physiology.
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DIFFERENTIALS
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Breast Cancer
Cardiac Tamponade
Cardiomyopathy, Restrictive
Hypothyroidism
Myocardial Infarction
Penetrating Chest Trauma
Pericardial Effusion
Pericarditis, Acute
Pericarditis, Constrictive
Pericarditis, Uremic
Tuberculosis
Uremia
Other Problems to be Considered:
Post-radiation syndromes
Neoplasias (metastatic)
Hematologic Neoplasias
Immunocompromised states with infection
Connective Tissue Disease |
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Patient Education
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Lab Studies:
- Laboratory studies include tests of serum CBC with differential and serum chemistries.
- The most important laboratory studies are those performed on pericardial fluid (always under the assumption that pericardiocentesis is clinically indicated). Hematocrit and cell count with differential, culture (including tuberculosis), glucose, total protein, enzymes (lactate dehydrogenase, adenosine deaminase), Gram staining, and cytology always should be sent on an initial pericardiocentesis.
- Other more specific laboratory testing is determined by the priorities of the differential diagnosis.
- Suspected tuberculosis pericarditis - Purified protein derivative of tuberculin (PPD), appropriate staining of pericardial fluid
- Suspected malignancy - Pericardial fluid for tumor markers or carbohydrate antigens (CAs, eg, CA 125)
- Suspected HIV pericarditis - Serum HIV testing
- Suspected infectious pericarditis - Serum aerobic and anaerobic blood cultures, viral titers or polymerase chain reaction (PCR) of pericardial fluid
- Suspected hypothyroid related pericarditis - Serum thyroid function testing
- Suspected connective tissue disease - Serum connective tissue serologies
Imaging Studies:
- The chest radiograph may consistently show an enlarged cardiac silhouette when the pericardial effusion is greater than 250 mL. The cardiac silhouette may be flask shaped and the lung fields without evidence of congestion, consistent with the absence of a congestive cardiomyopathy.
- These findings must be interpreted with caution, as they may also be observed in severe aortic insufficiency, congestive heart failure with severe tricuspid insufficiency, severe volume overload, or mitral regurgitation. However, the distinguishing characteristic is that pulmonary vascular congestion may be present with any of these and congestion is usually absent in pericardial disease.
- A small effusion may have a normal cardiac silhouette. This does not eliminate the diagnosis of effusive-constrictive pericarditis.
- Echocardiography is the most efficient way to detect an effusion because it has excellent sensitivity and specificity.
- Pericardial fluid is observed easily as an echolucent region (echo-free space) between the visceral pericardium (epicardium) and the parietal pericardium.
- The size of the effusion may be estimated, even if the effusion is localized. For example, small effusions usually must be observed in 2 views, particularly behind the left ventricle. Moderate effusions are visualized circumferentially, and large effusions exceed 1.0 cm in thickness on all views.
- Evidence for cardiac tamponade may be inferred from the echocardiogram. For example, early diastolic collapse of right ventricular free wall and/or late diastolic collapse of right atrium may be observed (see Cardiac Tamponade). Doppler investigation may demonstrate increased respiratory variation of mitral and tricuspid inflow, consistent with constrictive pericarditis. Other echocardiographic findings consistent with constrictive pericarditis include abnormal septal and posterior wall motion noted in the M-mode by using a parasternal short-axis view, a normal velocity of propagation (Vp) in color M-Mode, and a normal or supranormal early relaxation (Ea) on tissue Doppler imaging (see Pericarditis, Constrictive).
- A pericardial effusion can be distinguished from a pleural effusion with echocardiography (where pericardial effusions are anterior to the descending aorta).
- The diagnosis of effusive-constrictive pericarditis cannot be made primarily on the basis of CT or MRI findings. However, CT and MRI may provide excellent images of the pericardium and associated mediastinal structures.
- CT and MRI can be used to effectively image and confirm a thickened pericardium or detect a pericardial effusion if visualization with echocardiography is suboptimal (see Pericarditis, Constrictive).
- Some patients with effusive-constrictive pericarditis may have normal pericardial thickness; therefore, the diagnosis of effusive-constrictive pericarditis must be made hemodynamically.
Other Tests:
- The ECG may not show any specific findings for effusive-constrictive pericarditis. However, the ECG may show changes in the ST segment, T wave, or PR segment and/or low QRS voltage associated with pericarditis and/or effusion.
- Nonspecific ST- and T-wave abnormalities may be present.
- With a large effusion, a cardiac rocking motion may be observed on the ECG as electrical alternans.
Procedures:
- Cardiac catheterization and invasive hemodynamics
- The diagnosis of effusive-constrictive pericarditis is clinically suspected clinically but definitively established by recording right heart and intrapericardial pressures before and after pericardiocentesis.
- Before pericardial fluid is removed, cardiac tamponade (or near tamponade) hemodynamic physiology must be present to make the diagnosis of effusive-constrictive pericarditis. Hemodynamic pressure recordings would indicate elevated and equal (or nearly equal) intrapericardial pressures, right atrial and end-diastolic right and left ventricular pressures. There is usually an inspiratory decreased in right-heart filling pressures. A prominent X descent and absent Y descent may also be noted (see cardiac tamponade).
- Pericardiocentesis can decrease intrapericardial pressure to zero but may fail to restore cardiac hemodynamics to normal. This is because the visceral constrictive component of the syndrome causes a persistent elevation and equalization of intracardiac diastolic pressures. The constrictive physiology unveils a biphasic pressure tracing in the right atrium, now with a prominent Y descent and dip-and-plateau right ventricular pressure tracings, with absent or minimal respiratory variation.
- Persistent constriction after pericardiocentesis suggests a constrictive visceral pericardium and thus the diagnosis of effusive-constrictive pericarditis.
- Pericardiocentesis as a diagnostic test has a low yield, yet as a therapeutic procedure its diagnostic benefit is much improved. The risks and benefits of any invasive procedure must be considered before the start of testing (see Pericardiocentesis).
- Clinical circumstances determine when biopsy is preformed. For example, factors are (ie, how symptomatic the patient is and how likely a finding would change clinical management because, at a minimum, a surgical pericardial window procedure is required.
- Pericardioscopy is a developing technique that allows direct viewing of the epicardium with the possibility for biopsy. This is currently an experimental technique.
Histologic Findings: Pericardial biopsy samples may be examined for malignancy and inflammation by traditional and immunohistologic means. In advanced laboratories, polymerase chain reaction or in situ hybridization may be used to analyze for microbial DNA or RNA. Combined examination of pericardial fluid and biopsy results provides the greatest yield.
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TREATMENT
| Section 6 of 10 |
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Medical Care: - Curative therapy for hemodynamically compromising effusive-constrictive pericarditis is surgical intervention. However, medical management directed at the underlying etiology may be effective, as dictated by clinical circumstances.
- No randomized, blinded clinical trials have been completed to guide therapy.
- Medical therapy is primarily supportive.
- Depending on putative etiology, steroids, nonsteroidal anti-inflammatory agents, or antibiotics may be needed.
- Salt restriction may be indicated.
Surgical Care: - Pericardiocentesis or surgical drainage of effusion is performed as dictated by patient's clinical situation. These procedures are undertaken in circumstances of tamponade or hemodynamic compromise, when a purulent effusion is suspected, or in cases with a large persistent effusion.
- The most effective therapy for effusive-constrictive pericarditis is pericardiectomy with complete removal of the parietal and visceral membranes. The perioperative mortality rate with this procedure can be high. Surgery can be risky and requires considerable thought before it can be recommended. Difficulties include the length of the procedure, infection potential, technical expertise required, morbidity secondary to the wide exposure required, and the other medical problems often present in these patients that increase operative risk.
- In patients who may have a high mortality rate with thoracotomy yet have a significant chance of effusion recurrence with needle drainage alone, a pericardial-peritoneal window is an effective treatment for recurrent pericardial effusions.
Consultations: - A cardiologist can assist with echocardiographic interpretation, pericardiocentesis (see Pericardiocentesis), and invasive hemodynamics.
- A cardiothoracic surgeon may help when a pericardial window or pericardiectomy is being considered.
- In complicated cases, such as those involving tuberculosis pericarditis or purulent uremic pericarditis, multidisciplinary involvement may be required. Specialists in infectious disease, nephrology, cardiology, and/or cardiothoracic surgery may be consulted.
Diet: - No specific dietary changes are recommended.
- Often, these patients have chronic underlying diseases for which adequate nutrition is especially important.
Activity: - Activity generally is limited by the underlying disease or the decreased cardiac output that may occur with effusive-constriction.
- No specific prohibitions exist.
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MEDICATION
| Section 7 of 10 |
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No specific medical therapy exists. Whenever possible, treatment is directed at the underlying cause. Intravascular volume status must not be decreased excessively in the presence of tamponade physiology; diuretics must not be applied indiscriminately. On the other hand, after pericardial drainage, diuretics may be useful with constrictive physiology and evidence of volume overload.
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FOLLOW-UP
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Further Inpatient Care:
- Inpatient care is required to monitor patient if there are questions of hemodynamic compromise (see Cardiac Tamponade).
- Necessary pericardial procedures usually involve hospitalization.
Further Outpatient Care:
- The priorities of outpatient care reflect treating specific etiologies and monitoring patients for signs of worsening constrictive physiology or for the development of cardiac tamponade.
In/Out Patient Meds:
- In general, patients are given maintenance therapy with a diuretic to maintain euvolumia.
- Other medications depend on the specific etiology being treated.
Transfer:
- Transfer is required when necessary diagnostic or therapeutic modalities such as echocardiography, pericardiocentesis or cardiothoracic surgery are not available at the treating facility.
Complications:
- When visceral pericardiectomy is not chosen as the plan of care, the underlying disease may progress and cause recurrent and/or worsening effusive-constriction syndrome or constrictive pericarditis (see Pericarditis, Constrictive).
- Because effusive-constrictive pericarditis is rare, intrapericardial pressures are not routinely measured during pericardiocentesis in clinical practice. This protocol may result in failure to recognize intrapericardial pressure as near zero. The consequences of this oversight include missing the diagnosis of effusive-constrictive pericarditis.
Prognosis:
- The patient's prognosis depends on the underlying etiology and the rate of progression of the constrictive physiology.
- Visceral pericardiectomy is a delicate procedure, and only experienced surgeons should undertake this procedure.
- Because constrictive pericarditis (see Pericarditis, Constrictive) is potentially curable with surgery, the prognosis may be good.
Patient Education:
- Although the symptoms of effusive-constriction are nonspecific, patients should be counseled to report any new or worsened dyspnea, ascites, weight loss or gain, peripheral edema, fever, or chest pain or pressure.
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MISCELLANEOUS
| Section 9 of 10 |
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Medical/Legal Pitfalls:
- The initial potential pitfall is diagnosing a pericardial effusion, which usually is straightforward after echocardiography is performed
- Failure to establish a potential etiology and to diagnose constriction can worsen outcomes.
- Failure to consider the development of cardiac tamponade in the differential, which can quickly become life threatening, may be catastrophic.
- Failure to direct the evaluation of effusive-constrictive pericarditis along the lines of the locally available imaging technology and expertise is a pitfall; referral is required when diagnostic or invasive methods cannot be obtained otherwise.
Special Concerns:
- Acknowledgment for support for this chapter is given to the Office of Research and Development, Medical Research Service, Ralph H. Johnson Department of Veterans Affairs Medical Center, and the Gazes Cardiac Research Institute, Department of Medicine, Medical University of South Carolina, Charleston, South Carolina.
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BIBLIOGRAPHY
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