Despite the description of coexistent pericardial effusion and constrictive hemodynamics more than 50 years ago,^1,2 our current understanding of this entity, so-called effusive-constrictive pericarditis (ECP) is still limited. Due to the concomitant constrictive features, the hallmark of ECP is persistent elevation of right atrial pressure after tamponade has been relieved.³

Hancock and Sagrista-Sauleda's seminal publications provided important insights into the underlying hemodynamics of ECP and suggested that affected patients were at higher risk of requiring pericardiectomy than otherwise expected in acute pericarditis or pericardial effusions.^4,5 Over the past two decades, it has become apparent that the interplay between pericardial inflammation and pericardial noncompliance is a much more complex one, with ECP, transient constriction and constrictive pericarditis most likely being part of a clinical spectrum rather than separate entities.⁶

In ECP, decreased pericardial compliance occurs in combination with a pericardial effusion that is hemodynamically significant. In typical cardiac tamponade, right atrial pressures normalize after pericardiocentesis. Conversely, in ECP there is persistence of increased right atrial pressure post-centesis with constrictive features surfacing after the pericardial fluid has been drained (initially described as the development of deep x and y descents on right atrial tracings following pericardiocentesis).⁴

The prevalence of ECP has been reported to range from 1-2% to approximately 50% of patients undergoing pericardiocentesis.^5-9 These differences might be related to diagnostic methodology, such as invasive versus noninvasive diagnostic modalities and indication for pericardiocentesis (diagnostic vs. therapeutic). However, the prevalence of ECP appears to be intimately related to its underlying etiology; a low prevalence is reported in idiopathic pericardial effusion, whereas in some series more than half of patients presenting with tuberculous pericarditis having ECP.

ECP has been classically associated with a poor prognosis and high rates of pericardiectomy. In Sagrista-Sauleda's original series,⁵ more than half of survivors required pericardiectomy for persistent constrictive hemodynamics during follow-up. A systematic review reported a pericardiectomy rate of 65% among 20 patients with nonmalignant ECP.¹⁰ It should be noted, however, that many of those studies were published more than 10 years ago, when we were less aware of "atypical" forms of constrictive pericarditis and perhaps less aggressive with medical treatment for these variants. Nonetheless, the optimal medical treatment in the current era is still controversial. Some experts recommend non-steroidal anti-inflammatory agents as first line therapy (similar to the recommendations for acute pericarditis), whereas others favor corticosteroid therapy if constrictive features are present. The role of colchicine in ECP has not been formally evaluated; we generally recommend its use unless contraindicated. Although pericardiectomy can now be performed at low risk at high volume institutions,^11,12 surgery should be reserved for refractory cases and performed at centers with expertise in pericardial disorders.

Traditionally the diagnosis of ECP is based on invasive hemodynamics, defined by a failure of right atrial pressures to drop below 10 mmHg or by ≥50% post-pericardiocentesis.⁵ However, given the advances in the echocardiographic diagnosis of cardiac tamponade, most institutions no longer perform routine right heart catheterization prior to or at the time of pericardiocentesis. The presence of echo-Doppler features of constrictive hemodynamics post-centesis has been suggested to be indicative of ECP, but data to support that are limited to case reports or small case series.^9,13

In a recent JACC: Cardiovascular Imaging article,¹⁴ we reported the incidence and natural history of ECP diagnosed by transthoracic echocardiography in 205 consecutive patients undergoing pericardiocentesis at our institution. ECP was defined by the presence of >25% respiratory variation in mitral inflow early diastolic (E) velocities post-pericardiocentesis plus one or more of the following criteria: expiratory reversals in the hepatic vein Doppler, respirophasic septal shift, increased medial e' velocities or annulus reversus. Our observations raise several important questions regarding the epidemiology and prognosis of ECP:

1. Echo-Doppler features of constrictive pericarditis are common post-pericardiocentesis, with 16% of our cohort meeting criteria for ECP. A persistently dilated inferior vena cava following centesis was present in all cases and echolucent material in the pericardial space/pericardial thickening was seen in the majority of patients.
2. Not all patients with ECP presented with overt clinical tamponade (i.e., with associated evidence of hemodynamic instability). This is in agreement with the observations of others and suggests a continuum of patients presenting with pericardial effusion, increased pericardial pressures, and reduced pericardial compliance.⁸
3. Patients with ECP showed echo-Doppler features of constriction even prior to pericardiocentesis. The ECP group had higher medial e' velocities and more frequently showed respirophasic septal shift. In addition, loculated and fibrinous effusions were more common in the ECP group, suggesting more complex effusions. Thus, our findings suggest that ECP could be suspected even before the pericardial fluid is drained.
4. During a median follow-up of 3.8 years, pericardiectomy was required in a small minority of patients with ECP (2 out of 33), suggesting that the prognosis of these patients was good.

Our results suggest that the prognosis of ECP in the current era might be better than previously reported. However, close clinical follow-up is still required to ensure resolution of constrictive features. It is possible that previous studies had included patients with transient constriction and more advanced pericardial scarring (where small pericardial effusions are still commonly seen), contributing to their worse prognosis. Noteworthy, the majority of our patients had idiopathic or post-procedure/post-surgical effusions so our findings might not apply to patients with tuberculous pericarditis or radiation-related effusions.

As recommended by the pericardial disease guidelines,^15,16 transthoracic echocardiography is now the diagnostic imaging modality of choice for patients with pericardial disorders. As we manage increasing numbers of patients with iatrogenic effusions, it is crucial that future studies focus on the echo-Doppler features of ECP and its prognosis. Given the pivotal role of cross-sectional imaging in the assessment of pericardial inflammation, data on the use of cardiac tomography, magnetic resonance, and perhaps positon-emission tomography in the management of these patients is also warranted.

Acknowledgements

I thank Dr. Nandan Anavekar for his review and comments.

References

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Keywords: Pericarditis, Tuberculous, Pericardiocentesis, Pericardiectomy, Pericarditis, Constrictive, Pericardial Effusion, Cardiac Tamponade, Atrial Pressure, Paracentesis, Hepatic Veins, Vena Cava, Inferior, Anti-Inflammatory Agents, Non-Steroidal, Colchicine, Constriction, Cicatrix, Atrial Fibrillation, Iatrogenic Disease, Pericardium, Pericarditis, Echocardiography, Inflammation, Magnetic Resonance Spectroscopy, Tomography, Cardiac Catheterization, Adrenal Cortex Hormones, Cohort Studies

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