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Constrictive Pericarditis
Acknowledgment
The authors acknowledge the American Society of Echocardiography for allowing us to reproduce Figs. 122.1 to 122.3 .
Constrictive pericarditis (CP) is a relatively rare disorder in which the stiff, poorly compliant pericardium interferes with ventricular filling, causing elevation of ventricular diastolic pressure and, as a result, increased atrial pressures. This condition is responsible for a unique form of diastolic dysfunction and diastolic heart failure characterized by abnormal passive compliance, without myocardial involvement or systolic dysfunction and without active diastolic relaxation abnormalities. CP may be acute and transient or chronic and progressive. Recognition of this disorder is extremely important because pericardiectomy may offer a complete cure. The differential diagnosis includes restrictive cardiomyopathy (RCM), another form of ventricular compliance abnormality in which the culprit is the poorly compliant ventricular myocardium.
CP and RCM may imitate one another, and the differential diagnosis may be extremely difficult. The symptoms, physical findings, and many commonly used diagnostic techniques frequently fail to make the correct diagnosis. Sometimes the diagnosis can be established only after exploratory thoracotomy.
During the past two decades, echocardiography has been proven to be the technique of choice for the diagnosis of CP. Echocardiographic diagnosis requires state-of-the-art equipment, understanding of the underlying pathophysiology, and attention to details that can be easily missed on a routine examination. Clinical and echocardiographic findings in CP and RCM are summarized in Tables 122.1 and 122.2 .
TABLE 122.1
Clinical Data in Constrictive Pericarditis Versus Restrictive Cardiomyopathy
| Constriction | Restriction | |
|---|---|---|
| History | ||
| Right heart failure | Present | Present |
| Left heart failure (pulmonary congestion) | Uncommon | More common |
| Low cardiac output | Present | Present |
| Palpitations, arrhythmia | Present | Present |
| Physical Examination | ||
| Pericardial knock | Present | Absent |
| S2, S4 | Absent | Present |
| Apical impulse | Decreased | Present |
| Pericardial knuckle | Present | Absent |
| Cardiac Catheterization | ||
| High diastolic pressure | Present | Present |
| Square root sign | Present | Present |
| LV vs RV diastolic pressure | Equal | Unequal (left > right) |
| Other Diagnostic Tests | ||
| Pericardial calcification | May be present | Absent |
| Pericardial thickening | Present | Absent |
| Low-voltage ECG | May be present | May be present (amyloid) |
| Myocardial biopsy | Normal | Abnormal |
ECG, Electrocardiogram; LV, left ventricular; RV, right ventricular.
TABLE 122.2
Echocardiography in Constrictive Pericarditis Versus Restrictive Cardiomyopathy
| Constriction | Restriction | |
|---|---|---|
| M-Mode and Two-Dimensional Echocardiography | ||
| IVC plethora | Present | Present |
| Premature pulmonic valve opening | Present | Absent |
| Septal bounce | Present | Absent |
| Doppler Echocardiography | ||
| Mitral E-wave velocity | Increased | Increased |
| Mitral E/A ratio | Increased | Increased |
| Mitral deceleration time (ms) | <160 | <160 |
| E-wave respiratory variations | >25% | Absent |
| Hepatic vein expiratory flow reversal | Present | Absent |
| Increased expiratory pulmonary venous flow velocity | Present | Absent |
| M-mode color-flow propagation velocity | Normal or increased | Decreased |
| Mitral Ring Tissue Doppler | ||
| e′ velocity | Normal or increased | Decreased |
| E/e′ ratio | Normal or decreased | Increased |
| Lateral e′ < medial e′ (annulus reversus) | Present | Absent |
| Longitudinal strain | Preserved | Diminished |
IVC, Inferior vena cava.
Demographics and Presenting Symptoms
The causes of CP include pericardial inflammation, infection, blunt trauma, radiation, and complications of cardiac surgery. In 30% to 70% of patients, the cause cannot be established (idiopathic CP). The distribution of the causes varies geographically. In the United States and other Western countries, iatrogenic CP (as a result of cardiac surgery and radiation therapy) is more common. It is estimated that 0.3% of all patients who undergo cardiac surgery will develop CP. Longer survival in patients with Hodgkin lymphoma also leads to increasing numbers of patients who develop CP 10 to 20 years after mantle radiation. In developing countries, infection is a more common cause, with tuberculous, bacterial, fungal, and parasitic CP being more prevalent.
Symptoms include those of elevated atrial pressure and low cardiac output. Characteristically, diastolic left and right heart pressures are elevated and equal; however, elevated right atrial (RA) pressure produces symptoms at a lower pressure. Symptoms include general malaise, weakness, leg edema, ascites, and shortness of breath.
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