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

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.

A multi-modality imaging approach in chronic constrictive pericarditis is of paramount importance to diagnose, risk stratify, and plan surgery.

Median sternotomy approach is preferred for pericardiectomy in presence of extensively diseased pericardium.

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. Thickened calcific pericardium, encasing the heart is a rare occurrence in CP. 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 (). 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 in both tricuspid and mitral inflow velocities (e) and annulus reversus (medial e′ > lateral e′) were other features of chronic constrictive pericarditis (CCP) that were demonstrated in our patient (, and ). 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.

Figure 2

(A) A preoperative M-mode recording at mid-ventricular level is shown. It demonstrates a classically thickened pericardium (black star), abnormal septal motion (white arrows), and the diastolic flattening of the posterior wall after early rapid filling with near normal left ventricular systolic function. (B and C) A preoperative tissue Doppler imaging of medial and lateral mitral annular plane, respectively are shown. Medial annular velocity (15 cm/s) is more than lateral annular velocity (13 cm/s) due to compensatory exaggerated longitudinal motion of heart due to lateral restriction. This phenomenon is called annulus reversus. (D and E) Tissue Doppler imaging of medial and lateral mitral annular plane post-pericardiectomy, respectively are shown. Tethering effects of the diseased pericardium on lateral myocardial wall is abolished after pericardiectomy as evidence by increased lateral annular velocity (20 cm/s). Lateral annular velocity is more than medial annular velocity (12 cm/s) post-surgery, indicating reversal of annulus reversus phenomenon.

Figure 3

Doppler spectra across atrioventricular valve showing exaggerated respiratory variation. Increase in peak e velocity across tricuspid valve during inspiration >40% increase (A) and >25% during expiration across mitral valve (B) is characteristic of chronic constrictive pericarditis. Post-pericardiectomy, no respiratory variation is seen across tricuspid (C) and mitral valve (D). Yellow block arrows depict inspiratory and red block arrows depict expiratory phase.

(A) Chest X-ray in PA view shows calcified pericardium (black arrows), which is better delineated in B: lateral cine-fluoroscopic frame of the heart.

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 (). 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 (). Postoperative CT revealed denuded anterior and lateral surface of the heart with minimal residual pericardium over the posterior surface of heart (). Tissue Doppler velocities revealed improved ventricular filling with no respiratory variation across the tricuspid and mitral inflow (). 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.

Figure 4

Non-contrast computed tomography sections of the heart at various planes. Thickened, extensive, and asymmetric calcification around the heart (arrow heads) is clearly delineated in pre-operative computed tomography images in axial (A), sagittal (B), and coronal (C) planes. A non-contrast computed tomography reconstructed volume-rendered image precisely delineates the extent of ‘egg shell’ like calcification as shown in D (blue block arrows). Post-pericardiectomy, axial computed tomography images (E and F) show complete absence of the diseased pericardium over anterior and lateral surface, with minimal residual unresected calcified pericardium (black arrows) at the posterior surface of the heart.

Haematoxylin and eosin stained section of the resected pericardial tissue demonstrating areas of fibrosis (black arrow), calcification (black block arrow), and ossification (blue block arrow) with no evidence of granulomas (magnification ×100).

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. Cardiac filling occurs only in the early part of diastole and patients present with features of biventricular failure. 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%. 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. Obstructive airway disease and preoperative renal and or liver dysfunction are other independent non-cardiac risk factors for early and late adverse outcomes post-pericardiectomy. Pericardial decortication in these subgroups of patients, should be considered cautiously, as they may not benefit from surgery.,

One in four cases of CP are associated with pericardial calcification. However, extensive egg-shell like calcification, as present in index case, is rare. 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. Clinical examination and transthoracic echocardiogram along with haemodynamic catheterization confirm the diagnosis of CCP., 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. 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. 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.

Lead author biography

Dr. Mullasari Ajit Sankardas is currently Director of Cardiology at the Institute of Cardio-Vascular Diseases, Madras Medical Mission, Chennai, India. He is Examiner for the DNB-Cardiology, FNB-Cardiology and MRCP (PACES). He has an appointment as Adjunct Faculty at the Department of Biotechnology, IIT, Madras and Adjunct Professor at the Case Western Reserve University, Cleveland, USA. He is also an International co-ordinator for the Royal College of Physicians & Surgeons of Glasgow in India. He has published 192 papers and 205 abstracts in indexed journals. He is Founder Trustee for SANKALP, an organization for children with learning disabilities and Autism.

Supplementary material

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

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Table 1

Salient echocardiographic features of constrictive pericarditis in our patient

Features	Mechanics	Comments
Abnormal septal motion: Septal bounce/shudder/diastolic checking Septum bows into the left ventricle in inspiration and into the right ventricle on expiration.(2D echocardiography and M-mode)	Exaggerated interventricular interdependence. Rapid cessation of filling during early diastole due to pericardial constraint.	Equivalent to discordant relationship of ventricular systolic pressures during respiration on cardiac catheterization.
		Equivalent to square root sign/dip and plateau pattern of ventricular filling pressures on cardiac catheterization.
		Clinically equates to rapid y descent on JVP (Friedreich’s sign) and pericardial knock.
Respiratory variation during filling: Increase in peak e velocity across Tricuspid valve is >40% in inspiration. Mitral valve is >25% in expiration.	Dissociation of intra-thoracic and extra-thoracic pressures causing exaggerated interventricular interdependence.	Can also be measured in outflow tract, pulmonary vein, or hepatic veins.Hepatic veins demonstrate enhanced expiratory flow reversal with constriction
Augmented longitudinal motion of the heart.
Annular tissue velocity (e′): Medial e′ is normal or even elevated. Lateral e′ is decreased.	Tethering effects of disease pericardium limits lateral expansion of the heart resulting in medial e′ >lateral e′.	This phenomenon is called annulus reversus.Reversal of normal phenomenon where lateral annular tissue velocities are more than medial.
Inverse correlation between filling pressures and E/e′.	Haemodynamic studies revealed elevated filling pressures.However, due to compensatory exaggerated longitudinal movement of heart, E/e′ not elevated.	This phenomenon is called annulus paradoxus.Normally, the filling pressures are in direct correlation with E/e′
Systemic venous congestion: Congested and non-collapsing IVC	Impaired ventricular filling	Clinically evident by features of right heart failure.
Other parameters	Thickened pericardium with or without calcification. Normal ventricular wall thickness. Normal or near normal ventricular function. Absence of severe biatrial enlargement.