Pericarditis, Constrictive-Effusive

Introduction
Background

Effusive-constrictive pericarditis is a clinical syndrome characterized by concurrent pericardial effusion and pericardial constriction where constrictive hemodynamics are persistent after the pericardial effusion is removed. The mechanism of effusive-constrictive pericarditis is thought to be visceral pericardial constriction. Pericardial effusions vary in size and age and may be transudative, exudative, sanguineous, or chylous. An effusion persisting for months to years may evolve into effusive-constrictive pericarditis.1,2,3,4,5,6,7,8,9,10

The pericardium consists of two layers, a parietal layer and visceral layer. The visceral pericardium is composed of 1 or 2 cell layers of mesothelial cells and adheres closely with the epicardium. The parietal pericardium is separated from the visceral pericardium by a small amount of fluid that serves as a lubricant. Any supraphysiological accumulation of this fluid is identified as a pericardial effusion.1,2,11,12,13 In general, a pericardial effusion should be evaluated to determine its etiology and hemodynamic significance.

Patients with effusive-constrictive pericarditis may present with symptoms caused from a limitation of intercardiac end-diastolic volume. These findings are secondary to not only the pericardial effusion but also pericardial constriction. Symptoms, as well as history and physical findings, vary and a moderate-to-large pericardial effusion may occur.

Jugular venous and arterial pressures may be within the reference range, with or without signs of cardiac tamponade. 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 the continued elevation of right atrial, end-diastolic right ventricular and left ventricular diastolic pressures after the removal of pericardial fluid returns the pericardial pressure to zero (or near zero).1,3,14

Recognition of effusive-constrictive pericarditis is clinically important because treatment with pericardiocentesis or a pericardial window may be inadequate as it would not address the visceral pericardium. Rather, a visceral pericardiectomy may be indicated for optimal therapy since it is the visceral pericardium that is constricting.

Importantly, 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.3 In 2004, Sagrista-Sauldea et al reported 15 subjects from Barcelona, Spain who were identified as having effusive-constrictive pericarditis.14 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. 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 cardiac chambers, thereby increasing both 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 tamponadelike pressure tracings, which change to constrictivelike tracings after pericardiocentesis. This is because the visceral pericardium, 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 even though the absolute number of cases is relatively low.4

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
United States

Effusive-constrictive pericarditis is a rare disorder. As a complication of pericarditis, pericardial effusion, pericardial tamponade, or chronic constrictive pericarditis, the incidence of effusive-constrictive pericarditis 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, or are idiopathic (see Differentials).6
International

Effusive-constrictive pericarditis 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 developing countries are more often secondary to infectious causes (eg, tuberculosis) than other causes (see Differentials).15 In a prospective study of 1184 patients with pericarditis, Sagrista-Sauldea et al reported that 6.9% of 218 patients with tamponade had confirmed effusive-constrictive pericarditis.14
Mortality/Morbidity

* The mortality of effusive-constrictive disease is directly related to its etiology. For example, 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

Since 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.
Clinical
History

* Symptoms of effusive-constrictive pericarditis can be hard to interpret but may include atypical or typical chest pain, chest heaviness, or pressure.
* Other symptoms include dyspnea on exertion, fatigability, or peripheral edema.
* Many patients are asymptomatic until the advanced stages of disease. In more severe cases, impaired mental status may be evident as a result of decreased cardiac output.
* 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).2

Physical

* Physical findings may be a continuum, including findings common with cardiac tamponade (see Cardiac Tamponade).16
* Findings may include hypotension, jugular venous distension, and diminished heart sounds (classic Beck triad).
* Other 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), renal dysfunction, liver dysfunction and/or auscultation of a pericardial friction rub.
* The classic description of percussible cardiac dullness at the apex may be 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.

* Leading causes
o Idiopathic factors
o Irradiation
o Cardiac surgery
o Neoplasm - Most commonly lung, breast, or hematologic
o Infectious disease - Particularly in immunocompromised states (most commonly tuberculosis and fungal, although streptococcus species have been reported)17,18
o Myocardial infiltration
o Connective tissue disease
o Uremia
* 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.

http://emedicine.medscape.com/article/157216-overview