Constrictive pericarditis is a pericardial syndrome where the pericardium becomes relatively rigid and inelastic, may be thickened and calcified or not, and impairs mid to late diastolic filling. Constrictive pericarditis is the final pathway of several different diseases or causes, usually causing pericarditis and pericardial effusion and progressing towards pericardial fibrosis and calcification [1–3].

In constrictive pericarditis, the typical impairment of diastolic filling is mid to late diastolic with a suddenly blocked early diastolic filling causing several signs on physical examination (pericardial knock) and echocardiography (septal notch on M-mode, 2D echo recordings). In addition the fibrotic evolution of the pericardium is responsible for the obliteration of pericardial space with dissociation of intrathoracic pressures from intracardiac pressures as in cardiac tamponade. On this basis, the overall pericardial volume becomes fixed and the increase of one ventricle (e.g. right ventricle during inspiration due to increased venous return) can only occur with the decrease of the other (e.g. left ventricle). This causes and exaggerated interventricular interdependence with inspiratory decrease of the left ventricle volume and flows and contemporary increase right ventricle volume and flow with septal bulging that can be seen on echocardiography [1–3].

The typical presentation of a patient with constrictive pericarditis includes signs and symptoms of right heart failure usually with preserved right and left ventricular function in the absence of previous or concomitant myocardial disease or advanced forms. Patients complain about dyspnoea, fatigue, peripheral oedema, breathlessness, hepatomegaly, pleural effusions, ascites and abdominal swelling. The delay between the initial pericardial inflammation and the onset of constriction is variable and it is possible a direct evolution from subacute/chronic pericarditis to constrictive pericarditis [3]. In specific forms such as in tuberculous pericarditis or effusion, the evolution usually occurs within 3–6 months [3, 4].

In advanced cases, pericardial fibrosis and scarring may extend into the myocardium, thus causing myocardial fibrosis and dysfunction. In specific forms (e.g. radiation pericarditis), the same aetiological agent (radiation) affecting the pericardium also affects the myocardium (as well as valves and coronary arteries), thus causing ventricular dysfunction as well.

Constrictive pericarditis is commonly the final evolution of any type of pericarditis and pericardial effusion. The risk of developing such evolution is especially related to the aetiology: low (<1 %) in viral and idiopathic pericarditis, intermediate (2–5 %) in immune-mediated pericarditis and neoplastic pericardial diseases, and high (20–30 %) in bacterial pericarditis, especially purulent pericarditis [5].

There are few contemporary series of constrictive pericarditis reported by tertiary referral centres after pericardiectomy (Stanford, Mayo Clinic, Cleveland Clinic, and Groote Schuur Hospital) (Table 15.1) [6–9].

The most common reported causes were idiopathic or viral (42–49 %), post-cardiac surgery (11–37 %), post-radiation therapy (9–31 %), mostly for Hodgkin’s disease or breast cancer, connective tissue disorder (3–7 %), post-infectious (tuberculous or purulent pericarditis in 3–6 %) and miscellaneous causes (malignancy, trauma, drug- induced, asbestosis, sarcoidosis, uremic pericarditis in less than 10 %) in developed countries [6–9]. However, tuberculosis is a major cause all over the world, especially because of developing countries, where tuberculosis is endemic [4].

In final stages, when constriction is chronic and permanent, it may be difficult to distinguish the initial cause, since the final picture of fibrosis and scarring on bioptic samples, and the clinical presentation are non-specific [3].

According to 2015 ESC guidelines on the management of pericardial diseases, the diagnosis of constrictive pericarditis is based on the association of signs and symptoms of right heart failure and instrumental evidence of an impaired diagnostic filling due to pericardial constriction by one or more imaging methods including echocardiography, CT, CMR and cardiac catheterization [4]. Although classical and advanced cases show prominent pericardial thickening and calcifications in chronic forms (Figs. 15.1 and 15.2), constriction may also be present with normal pericardial thickness in up to 20 % of cases [10].

The main differential diagnosis is with restrictive cardiomyopathy (Table 15.2). The essential differential features between the two pathological conditions are represented by the affected part of the heart. In “simple” (not advanced or complicated by ventricular dysfunction) constrictive pericarditis, it is the pericardium to be involved and not the myocardium, while in restrictive cardiomyopathy, it is the left ventricle at the myocardial level to be involved. In constrictive pericarditis, this implies that diastolic dysfunction affects the mid-late diastole with dissociation of intrathoracic with intracardiac pressures and essentially preserved myocardial function.