Constrictive pericarditis is a chronic disorder that results from pericardial inflammation, fibrosis, and possibly calcification, with subsequent loss of elasticity. Although many cases are idiopathic, causes of constrictive pericarditis include chest radiation therapy, cardiac surgery, trauma, postmyocardial infarction syndromes, and systemic diseases that affect the pericardium (eg, tuberculosis, connective tissue disease, malignancy, infections).
The pericardium in constrictive pericarditis is rigid and noncompliant. Ventricular filling occurs rapidly in early diastole and terminates abruptly due to the pericardial restraint. With disease progression, the impairment in diastolic filling leads to an increase in intracardiac filling pressures that occur in order to maintain forward cardiac output.
The noncompliant pericardium prevents the complete transmission of respiratory changes in thoracic pressure to the cardiac chambers. As a result, filling of the right and left ventricles varies significantly with respiration due to marked changes in the early diastolic gradient emptying into these chambers (ie, dissociation of thoracic-cavitary pressures). During inspiration, the decrease in thoracic pressure leads to relatively less left ventricular filling, while the increase in caval blood flow augments right ventricular preload. Reciprocal changes in ventricular loading occur during expiration (Fig. 40-5).
Hemodynamic features of constrictive pericarditis. (Top left) During inspiration, there augmentation of flow into the right ventricle with ventricular septal shifting (arrow) leading to increases in tricuspid valvular (TV) flow and decreases in mitral valvular (MV) flow, both of which can be detected by Doppler echocardiography. (Top right) Expiration leads to reciprocal changes with shifting of the ventricular septum toward the right ventricle. (Middle left) Expiratory flow reversals in the hepatic vein detected by Doppler echocardiography. (Middle right) Respiratory variation in MV flow, as measured by Doppler interrogation of the early (E) and late (A) diastolic filling velocity. (Bottom left) Dissociation of intracavitary and intrathoracic pressures seen on invasive cardiac catheterization. There is significant respiratory variation in left ventricular filling, which can be seen as changes in the diastolic gradient between the pulmonary capillary wedge pressure and the left ventricle (gray). (Bottom right) Enhancement of ventricular interdependence. Reciprocal respiratory changes in the filling of each ventricle occur, leading to discordance in pulse pressure, systolic pressure, or stroke volume between the right and left ventricles during respiration.
The total cardiac volume is fixed by the noncompliant pericardium. Because the ventricular septum is not involved, bulging of the septum toward the left occurs during inspiration and returns toward the right during expiration, leading to marked enhancement of ventricular interdependence. The dissociation of thoracic and intracavitary pressures and the enhanced ventricular interdependence leads to reciprocal changes in filling and emptying of the right and left ventricles, which manifest as alterations in right and left-sided forward stroke volumes during respiration.
Symptoms of diminished cardiac output (eg, fatigue) and evidence of volume overload characterize the clinical presentation of constrictive pericarditis. The jugular venous pressure is elevated in nearly all patients, with prominent x and y descents. Physical findings that also may be present include a Kussmaul sign, pericardial knock, pulsus paradoxus, pleural effusions, congestive hepatomegaly, and peripheral edema or ascites. In patients with long-standing constrictive pericarditis, hepatic failure and cirrhosis may be present.
Patients with constrictive pericarditis also may present with pericardial effusion, with or without cardiac tamponade (“effusive constrictive pericarditis”). In these patients, there are persistent symptoms and hemodynamic derangements following relief of the pericardial effusion. These abnormalities are principally due to disease involvement of the visceral layer of pericardium, whose thickening may be difficult to detect with conventional noninvasive cardiac imaging.
Doppler and two-dimensional echocardiography is the primary imaging modality for the evaluation of patients with suspected constrictive pericarditis. Dissociation of thoracic and intracavitary pressures results in respiratory variation in ventricular filling, which manifests as respiratory variation in the mitral and tricuspid inflow velocities (>25% in most cases). Patients with constriction also demonstrate expiratory flow reversals in the hepatic veins due to thoracic-cavitary dissociation and enhanced ventricular interdependence (Fig. 40-6). Early diastolic tissue Doppler velocity at the mitral annulus (E’) usually is accentuated (>10 cm/s), due to exaggeration of diastolic function of the left ventricle along the longitudinal axis.11 Other findings supportive of the diagnosis of constrictive pericarditis are respiratory shift of the ventricular septum, increased pericardial thickness, and plethora of the inferior vena cava. Pericardial thickening, with or without calcification, can be seen with echocardiography and also detected with cardiac computed tomography or magnetic resonance imaging.
Early ventricular filling in cardiac tamponade versus constrictive pericarditis. (Left) In cardiac tamponade, there is blunting of the y descent due to impairment of ventricular filling throughout the entire diastolic period. Note pulsus paradoxus also is present in the arterial tracing (asterisk). (Right) In patients with constrictive pericarditis, there are rapid x and y descents. The y descent of the right atrial pressure tracing corresponds to the early rapid filling phase of the ventricular pressure tracing, which demonstrates the typical dip and plateau pattern (arrow). Early rapid filling is a prominent feature of constrictive pericarditis, but also may be seen in other forms of heart failure. FA, femoral artery; LV, left ventricle; RA, right atrial.
When the clinical findings and non-invasive studies cannot definitively establish the diagnosis of constrictive pericarditis in suspected patients, invasive hemodynamic evaluation with cardiac catheterization is indicated. The accentuation of early diastolic ventricular filling from elevated filling pressures in constriction may be seen as a “square-root” sign. This abnormality is distinct from the hemodynamic findings of cardiac tamponade, but may be seen in other forms of heart failure (Fig. 40-6). Equalization of the end-diastolic pressures in all four cardiac chambers frequently is observed, though this finding may only be present during inspiration.
The most accurate method for diagnosing constrictive pericarditis with cardiac catheterization entails the use of dynamic respiratory criteria. In patients with constrictive pericarditis, the inspiratory fall in thoracic pressure affects the pulmonary wedge pressure, but ventricular pressure is relatively shielded from respiratory pressure changes by the pericardial scar. This dissociation of intrathoracic and intracavitary pressures can be seen as respiratory changes in the gradient between the pulmonary wedge (or left atrial) pressure and left ventricle during early diastole.
The most specific hemodynamic finding in patients with constrictive pericarditis is discordant changes in right and left ventricular pressures during respiration due to enhancement of ventricular interdependence.12,13 These alterations manifest as reciprocal changes in peak systolic pressure, stroke volume, and pulse pressure in both ventricles during respiration. The degree of ventricular interdependence can be quantitated by measuring the systolic areas under the left ventricular and right ventricular pressure curves. In one study, quantitation of ventricular interdependence had a high sensitivity and predictive accuracy (>97%) for identifying patients with surgically proven constrictive pericarditis.14 Other findings supporting the diagnosis of constrictive pericarditis at cardiac catheterization are the presence of epicardial fixation of the coronary arteries and pericardial calcification on fluoroscopy.
In patients with restrictive cardiomyopathy and other forms of heart failure, neither enhancement of ventricular interaction nor dissociation of intrathoracic and intracavitary pressures are present. In these patients, inspiration lowers the pulmonary wedge and left ventricular diastolic pressures equally. Therefore, the pressure gradient for left ventricular filling remains virtually unchanged during respiration. Because there is not significant enhancement of ventricular interdependence, the left ventricular and right ventricular pressures move concordantly throughout the respiratory cycle.
In the vast majority of patients with constrictive pericarditis, cardiac surgery with pericardiectomy is the definitive treatment for relief of heart failure. Due to the significant technical challenges of the procedure, this surgery is best performed in experienced centers where a complete pericardiectomy can be provided. Medical therapy with diuretics can improve symptoms or be palliative in patients who are not surgical candidates, but the chronic nature of the disorder can prove to be drug-refractory. Predictors of poor outcome after surgical pericardiectomy include advanced age, severe symptoms, pulmonary hypertension, renal insufficiency, left ventricular dysfunction, and radiation therapy as the underlying etiology of constrictive pericarditis.15,16 In one study, the 7-year survival after pericardiectomy respectively was 27%, 66%, and 88%, for patients with constrictive pericarditis due to radiation, prior cardiac surgery, and an idiopathic etiology.17
There is a subset of patients who have a transient form of constrictive pericarditis where there is either spontaneous resolution or a significant response to medical therapy. These patients constitute a minority of those presenting with constrictive hemodynamics (<25%), and more frequently have idiopathic, viral, or postsurgical causes.18-20 Thus, it may be reasonable to perform a trial of medical therapy (eg, nonsteroidal anti-inflammatory drugs) before surgery in some patients presenting with constrictive pericarditis, particularly those with mild symptoms, a potentially reversible cause of acute inflammation, and no evidence of chronic constriction.
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