Constrictive Pericarditis: A Practical Clinical Approach
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.
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Statement of Conflict of Interest: see page 377.