Constrictive pericarditis
A 69-year-old woman arrived at the emergency department reporting a 3-year history of shortness of breath during physical activity. She had a background of focal pleural plaques attributed to previous asbestos exposure from her employment at an insulation-manufacturing company. Physical examination revealed signs of fluid overload. A chest X-ray displayed calcification around the heart’s lining (pericardium), pleural effusions, and interstitial swelling (Panel A, lateral view). Further chest CT imaging confirmed significant pericardial calcification (Panel B). A transthoracic echocardiogram indicated normal ejection fraction but showed specific abnormalities such as a septal bounce in early diastole and hepatic vein diastolic reversal during exhalation.
Subsequent left and right heart catheterisation revealed ventricular interdependence and discordant pressure tracings. During inspiration, when right ventricular pressures peaked, left ventricular pressures were lowest, with this pattern reversing during exhalation (Panel C; right ventricular pressure, solid arrow; left ventricular pressure, dotted arrow). A distinctive “square root” sign (also known as a dip-and-plateau pattern; Panel C, red line) indicative of rapid early diastolic ventricular filling followed by abrupt cessation in late diastole was observed. The diagnosis of constrictive pericarditis due to asbestosis was established, leading to a pericardiectomy. Pericardial tissue analysis ruled out tuberculosis. At the 2-year follow-up, the patient’s condition had significantly improved.
Constrictive pericarditis (CP) arises from a thickened and inflexible pericardium, which limits the heart’s diastolic filling
In CP, the heart fills normally in early diastole but encounters a sudden reduction in filling once the pericardium reaches its elastic limit. Pericardiectomy stands as the sole definitive treatment for patients with persistent symptomatic CP. While many patients experience significant symptom relief post-surgery, pericardiectomy carries notable risks of perioperative complications and mortality, particularly for those in advanced stages of CP. Therefore, prompt diagnosis and early surgical intervention are crucial.
CP manifests through signs and symptoms stemming from increased central venous pressure and diminished cardiac output, leading to edema, fatigue, ascites, and liver dysfunction. These manifestations can sometimes be erroneously attributed to primary liver ailments, underscoring the challenge in diagnosing CP, which necessitates a keen clinical acumen.
Diagnosis
Diagnosing CP requires a high level of clinical suspicion due to the potential for its signs and symptoms to mimic those of other conditions. In severe cases, CP may present with common manifestations such as ascites (37%), hepatomegaly (53%), pleural effusion (35%), and peripheral edema (76%). This similarity often leads to misdiagnoses, particularly as chronic liver disease. The time from symptom onset to pericardiectomy averaged 11.7 months (range: 3 days to 29.1 years).
In patients with cirrhosis from other causes, jugular venous pressure (JVP) is typically normal or decreased (except in those with tense ascites), contrasting with the elevated JVP frequently observed in CP patients (93%). Ordinarily, inspiration leads to a drop in intrathoracic pressure, reflected in a reduction in jugular venous distention (JVD). However, in CP, impaired right heart filling prevents this drop, leading to an increase in JVD during inspiration (known as Kussmaul’s sign). While Kussmaul’s sign is present in 13-21% of CP cases, it’s not specific to CP and can occur in conditions like tricuspid stenosis, right ventricular infarction, and restrictive cardiomyopathy. Identifying JVD and Kussmaul’s sign should prompt physicians to investigate underlying cardiac conditions.
Transthoracic echocardiography plays a crucial role in diagnosing CP and often reveals pericardial thickening
Pathophysiological features observed in CP include enhanced interventricular interaction and a dissociation between intrathoracic and intracardiac pressure. These are demonstrated by sudden interventricular septum displacement in early diastole during inspiration (septal shudder and bounce), a significant drop in mitral E flow immediately post-inspiration, and a similar decrease in tricuspid E flow post-expiration, alongside hepatic vein reversal. These findings are both sensitive and specific to CP; however, echocardiography should be conducted meticulously with a high suspicion for CP. These indicators are notably associated with CP in patients also experiencing atrial fibrillation. Atrial enlargement may be evident, particularly in cases with prolonged constrictive physiology.
The early diastolic Doppler tissue velocity at the mitral annulus (E’) typically decreases with diastolic dysfunction and increased filling pressures. While transmitral inflow early velocity (E) progressively rises with higher filling pressures, the E/E’ ratio strongly correlates with pulmonary capillary wedge pressure (PCWP) and left ventricular end-diastolic pressure. Nevertheless, in many CP patients, E’ remains prominent despite diastolic dysfunction due to increased wall longitudinal movement. Consequently, the usual positive linear relationship between E/E’ is reversed in CP cases. Thus, when an enlarged atrium is coupled with normal E’ and E/E’, CP should be considered.
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