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‘Egg-cased heart’: a multimodality imaging approach to chronic constrictive pericarditis with egg-shell calcification: a case report

Abstract Background Constrictive pericarditis (CP), also known as Pick’s disease, is a sequela of chronic inflammation of the pericardium. Pericardial calcification is a common occurrence in CP; however, extensive egg-shell like calcification is rare. Our case, highlights, how a multi-modality imaging in a middle aged female helped to diagnose chronic constrictive pericarditis (CCP) with egg-shell like calcification encasing the heart. Case summary Middle aged female with features of right heart failure, was diagnosed as CP based on two-dimensional echocardiography and cardiac catheterization. Computed tomography (CT) scan chest showed extensive egg-shell like calcification encasing the heart, suggestive of calcific CP. Subsequently, she underwent pericardiectomy, through median sternotomy approach and is currently on follow-up with asymptomatic cardiac status. Discussion Extensive pericardial calcification encasing the heart like an egg-shell is rare in CCP. Likelihood of incomplete pericardial resection is high in calcific CP and hence a median sternotomy is preferred over anterolateral thoracotomy. A preoperative non-contrast CT scan defines the thickness, anatomic extent the calcification and its adherence to myocardium and surrounding structures. A reconstructed volume-rendered image delineates the extent of calcification precisely, thus determining the optimum surgical approach. A multi-modality imaging in CP, especially in calcific CP, is thus of paramount importance.


Introduction
Constrictive pericarditis (CP), also known as Pick's disease, is a sequela of chronic inflammation of the pericardium. The result of scarring is impaired diastolic filling of ventricles, leading to elevated filling pressures and systemic venous congestion. 1 Thickened calcific pericardium, encasing the heart is a rare occurrence in CP. [2][3][4] Delineation of the exact location of pericardial calcification and thickness is essential as it facilitates risk stratification and surgical planning. Our case was diagnosed as chronic calcific CP (egg-shell like calcification) and underwent successful pericardial resection. This case highlights the need of multi-modality imaging in the diagnosis and management of chronic calcific CP.

Case presentation
A 45-year-old female, with no significant past medical history, presented with features of refractory right heart failure. Jugular venous pressure (JVP) was 18 cm with prominent Y descent. Kussmauls sign, increase in JVP or failure to fall on deep inspiration, was present. An early diastolic sound characteristic of pericardial knock was best heard at left sternal border. Her baseline biochemistry was normal with no evidence of hepatic or renal dysfunction. Chest X-ray showed cardio-thoracic ratio of 0.5 with radiopaque density suggestive of calcium around cardiac silhouette which was better appreciated on fluoroscopy ( Figure 1). Transthoracic two-dimensional echocardiogram showed mildly dilated atria with a near normal left ventricular systolic function, normal ventricular wall thickness, and hyperechoic shadow surrounding the ventricle suggestive of thickened pericardium. Septal bounce, exaggerated respiratory variation  Can also be measured in outflow tract, pulmonary vein, or hepatic veins.
Hepatic veins demonstrate enhanced expiratory flow reversal with constriction  Table 1, Figures 2 and 3). Right heart catheterization revealed elevated and equalization of diastolic pressure, classical dip and plateau pattern with discordance of right and left ventricular systolic pressures during respiration. Pulmonary artery pressures were 32/10 (m22) mmHg. Epicardial coronaries were normal on angiogram with no external compression or pinching detected.
Subsequent imaging with non-contrast computed tomography (CT) chest detected the presence of a thickened, extensive, asymmetric, and circumferential egg-shell like calcific pericardium encasing the heart (Figure 4). A volume-rendered CT precisely delineated the extent of calcification. A pericardiectomy through median sternotomy approach was performed. However, densely adherent pericardium over posterior surface was not resected. Histopathology revealed moderate fibrosis with calcification with no evidence of granulomas ( Figure 5). Postoperative CT revealed denuded anterior and lateral surface of the heart with minimal residual pericardium over the posterior surface of heart ( Figure 4E and F). Tissue Doppler velocities revealed improved ventricular filling with no respiratory variation across the tricuspid and mitral inflow ( Figure 3C and D). Inferior vena cava size was normal with normal collapsibility although septal bounce was still present on echocardiogram. She is currently on follow-up for more than 2 years with asymptomatic cardiac status. She showed no features of constriction on follow-up transthoracic echocardiogram and continues to have near normal left ventricular function.

Discussion
Chronic constrictive pericarditis, often called as Pick's disease-Pick described the presence of liver involvement and preponderant ascites in CCP-is a state of 'inflow stasis'. The pericardial constraint imposed by the inelastic and thick pericardium in CCP, limits the normal diastolic filling. 1 Cardiac filling occurs only in the early part of diastole and patients present with features of biventricular failure. 1 Unless pericardiectomy is performed, symptoms persists and progress in majority of patients. The timing of pericardiectomy is thus of paramount importance. Pericardial resection remains the only causal treatment available for CP and should not be delayed after symptom onset. Removal of the thickened, inflamed, and adherent pericardium is technically challenging and associated with a high perioperative mortality, ranging from 2% to 20%. [2][3][4] New York Heart Association class IV symptoms, concomitant coronary artery disease, reduced left ventricular function, and radiation-induced disease are risk factors for poor outcomes. [3][4][5] Obstructive airway   A multimodality imaging approach to CCP . . . . disease and preoperative renal and or liver dysfunction are other independent non-cardiac risk factors for early and late adverse outcomes post-pericardiectomy. [3][4][5] Pericardial decortication in these subgroups of patients, should be considered cautiously, as they may not benefit from surgery. 3,5 One in four cases of CP are associated with pericardial calcification. However, extensive egg-shell like calcification, as present in index case, is rare. 6 Likelihood of incomplete pericardial resection is high in calcific CP which portends a poor prognosis. Efforts should be made to excise as much of the diseased pericardium as possible and hence a median sternotomy is preferred over anterolateral thoracotomy. 1 Clinical examination and transthoracic echocardiogram along with haemodynamic catheterization confirm the diagnosis of CCP. 1,7 Chest X-ray and cine-fluroscopy help to ascertain the presence or absence of calcification. A non-contrast CT scan defines the extent of calcification and involvement of the myocardium and surrounding structures. By delineating the exact location and severity of pericardial thickening (normally less than 2 mm) and calcification, a preoperative CT scan facilitates risk stratification and surgical planning. Cardiac magnetic resonance (CMR) can be considered as an adjunct to above imaging modalities. Although less sensitive than CT scan to detect pericardial thickness and calcification, CMR provides better information regarding the haemodynamics of pericardial constraint. 7 It is of particular importance to identify transient and reversible form of CP. Late gadolinium enhancement (LGE) with thickened pericardium predict resolution of constriction following anti-inflammatory drugs therapy. 7 In cases of non-calcific CP, demonstration of no residual inflammation (a negative LGE sequence) has recently emerged as an important step before pericardiectomy.
A multi-modality imaging is of paramount importance in management of CCP.

Supplementary material
Supplementary material is available at European Heart Journal -Case Reports online.