Elsevier

Progress in Cardiovascular Diseases

Volume 59, Issue 4, January–February 2017, Pages 369-379
Progress in Cardiovascular Diseases

Constrictive Pericarditis: A Practical Clinical Approach

https://doi.org/10.1016/j.pcad.2016.12.008Get rights and content

Abstract

Constrictive pericarditis (CP) represents a form of severe diastolic heart failure (HF), secondary to a noncompliant pericardium. The true prevalence of CP is unknown but it is observed in 0.2–0.4% of patients who have undergone cardiac surgery or have had pericardial trauma or inflammation due to a variety of etiologies. Despite its poor prognosis if untreated, CP is a potentially curable disease and surgical pericardiectomy can now be performed at low perioperative mortality in tertiary centers with surgical expertise in pericardial diseases. Cardiologists should have a high index of suspicion for CP in patients presenting with predominant right-sided (HF), particularly when a history of cardiac surgery, pericarditis or pericardial effusion is present. Transthoracic two-dimensional and Doppler echocardiography is usually the first diagnostic tool in the evaluation of HF and can reliably identify CP in most patients by characteristic real-time motion of the heart and hemodynamic features. Computerized tomography and magnetic resonance imaging provide incremental data for the diagnosis and management of CP and are especially helpful when clinical or echocardiographic findings are inconclusive. Cardiac catheterization has been the gold-standard for the diagnosis of CP, but may not be necessary if non-invasive test(s) demonstrate diagnostic features of CP; it should then be reserved for selected cases or for assessment of concomitant coronary disease. Although most patients with CP require pericardiectomy, anti-inflammatory therapy may be curative in patients presenting with subacute symptoms, especially when evidence of marked ongoing inflammation is seen.

Introduction

Constrictive pericarditis (CP) represents a form of severe diastolic dysfunction, secondary to a poorly compliant, inelastic pericardium. As the name implies, the heart is constricted to a limited diastolic filling by the inflamed and/or scarred pericardium. The true prevalence of CP is unknown but it is reported in 0.2–0.4% of patients following cardiac surgery1., 2. and it can also occur after pericardial trauma or inflammation due to a variety of etiologies. The diagnosis of CP is frequently overlooked and patients frequently undergo numerous non-cardiac and cardiac tests as its manifestations mimic myocardial, coronary artery, pulmonary, or even gastrointestinal conditions. Physicians should consider CP in all patients presenting with symptoms of heart failure (HF), especially when left ventricular ejection is preserved. It should be actively sought since it is a potentially curable entity and carries significant morbidity and mortality if left untreated.3

Section snippets

Pathophysiology

The pericardium is normally an avascular, thin sac composed by two layers (serosal and fibrous). A physiologic amount of pericardial fluid (30–50 ml) allows the heart to move freely within the pericardial sac. The pericardium prevents acute distension of cardiac chambers and optimizes diastolic filling. In CP, varying degrees of inflammation and fibrosis affect the pericardium; increased pericardial thickness is present in 80% of affected patients4 (Fig 1). As a consequence, the pericardium

Etiology

The epidemiology of CP in the United States has changed markedly over the past 50 years, as the incidence of tuberculosis decreased and the number of cardiac surgeries substantially increased. Tuberculous pericarditis (TP), once representing almost 50% of cases of CP,6 is now exceedingly rare. In a review of pericardiectomies performed at Mayo Clinic between 1985 and 1995,7 idiopathic and post-cardiac surgery cases were the two most common etiologies (accounting for more than half of cases),

Clinical presentation and physical examination

The classic presentation of CP is of right-sided HF – leg edema, abdominal distention, hepatomegaly, and ascites. CP should always be suspected in patients with right-sided HF with an otherwise structurally normal heart, with a history of cardiac surgery or when HF appears out of proportion to echocardiographic findings. Not infrequently patients report a history of multiple paracenteses refractory to diuretic therapy. Fatigue secondary to low-cardiac output is common and dyspnea secondary to

Diagnosis

  • a.

    Electrocardiography

There are no pathognomic electrocardiographic findings for the diagnosis of CP. Nonspecific ST-T wave abnormalities are frequently present and AF is not uncommon. Broad notched P-waves in lead II (P mitrale), classically encountered in rheumatic mitral stenosis, have also been described in CP.19 This is felt to be secondary to LA enlargement towards the oblique sinus, an area where the pericardium is absent.

  • b.

    Chest radiography

The presence of pericardial calcification supports

Unusual forms of CP

  • a.

    Transient CP and effusive-CP

Transient constrictive pericarditis was initially described in patients who developed constrictive hemodynamics in the resolving phase of acute pericarditis.42 Those patients had a benign prognosis and did not require pericardiectomy. The definition of transient constriction has evolved and some investigators have defined transient constriction as CP that resolved after administration of anti-inflammatory therapy.15 This definition is supported by the European

Treatment

  • a.

    Medical

The treatment for CP is still surgical pericardiectomy. However, in subacute cases where evidence of ongoing inflammation is present, therapy with non-steroidal anti-inflammatory agents, colchicine or corticosteroids or triple therapy might be attempted. Elevated serum inflammatory markers (sedimentation rate and C-reactive protein) and cardiac MRI might be helpful in identifying patients that might respond to medical therapy.15 Patients with greater degree of gadolinium enhancement by

Conclusion

CP is a potentially curable disease; pericardiectomy can be performed at a low perioperative risk in experienced centers. Physicians should have a high index of suspicion for CP in patients presenting with predominant right-sided HF, particularly with a history of cardiac surgery or other conditions causing pericardial inflammation or trauma. TTE is currently the main diagnostic tool in the evaluation of CP, and other modalities such as CT, MRI or cardiac catheterization provide incremental

Statement of conflict of interest

None of the authors have any conflicts of interests with regard to this publication.

References (50)

  • J.K. Oh et al.

    Diagnostic role of Doppler echocardiography in constrictive pericarditis

    J Am Coll Cardiol

    (1994)
  • D.R. Talreja et al.

    Constrictive pericarditis in the modern era: novel criteria for diagnosis in the cardiac catheterization laboratory

    J Am Coll Cardiol

    (2008)
  • J. Sagrista-Sauleda et al.

    Transient cardiac constriction: an unrecognized pattern of evolution in effusive acute idiopathic pericarditis

    Am J Cardiol

    (1987)
  • Y.H. Cho et al.

    Completion pericardiectomy for recurrent constrictive pericarditis: importance of timing of recurrence on late clinical outcome of operation

    Ann Thorac Surg

    (2012)
  • E.A. Gillaspie et al.

    A 20-year experience with isolated pericardiectomy: analysis of indications and outcomes

    J Thorac Cardiovasc Surg

    (2016)
  • S.C. Bertog et al.

    Constrictive pericarditis: etiology and cause-specific survival after pericardiectomy

    J Am Coll Cardiol

    (2004)
  • M. Senni et al.

    Left ventricular systolic and diastolic function after pericardiectomy in patients with constrictive pericarditis: Doppler echocardiographic findings and correlation with clinical status

    J Am Coll Cardiol

    (1999)
  • K. Matsuyama et al.

    Clinical characteristics of patients with constrictive pericarditis after coronary bypass surgery

    Jpn Circ J

    (2001)
  • D.R. Talreja et al.

    Constrictive pericarditis in 26 patients with histologically normal pericardial thickness

    Circulation

    (2003)
  • D.E. Dines et al.

    Myocardial atrophy in constrictive pericarditis

    Proc Staff Meet Mayo Clin

    (1958)
  • R. Robertson et al.

    Constrictive pericarditis with particular reference to etiology

    Circulation

    (1962)
  • L.H. Ling et al.

    Detection of constrictive pericarditis: a single-Centre experience of 523 surgically confirmed cases [abstract]

    J Am Coll Cardiol

    (2009)
  • G. Szabo et al.

    Constrictive pericarditis: risks, aetiologies and outcomes after total pericardiectomy: 24 years of experience

    Eur J Cardiothorac Surg

    (2013)
  • U. Yetkin et al.

    Recent surgical experience in chronic constrictive pericarditis

    Tex Heart Inst J

    (2003)
  • M. Imazio et al.

    Risk of constrictive pericarditis after acute pericarditis

    Circulation

    (2011)
  • Cited by (66)

    • Evaluation of Pericardial Thickening and Adhesion Using High-Frequency Ultrasound

      2023, Journal of the American Society of Echocardiography
    • Recent advances in pericarditis

      2022, European Journal of Internal Medicine
    • Pericardiectomy for Constrictive Pericarditis: Analysis of Outcomes

      2021, Journal of Cardiothoracic and Vascular Anesthesia
    View all citing articles on Scopus

    Statement of Conflict of Interest: see page 377.

    View full text