Eggshell calcification of the heart in constrictive pericarditis

Abstract

Constrictive pericarditis (CP) is an inflammatory disease of pericardium. Pericardial calcification in X-ray provides a clue for the diagnosis of CP. An extensive “eggshell” type of calcification is rarely seen in CP. We hereby report a case of CP with eggshell calcification of pericardium, encircling whole of the heart. A need for multimodality imaging and hemodynamic assessment followed by surgical pericardiectomy is discussed.

Key Words: Contrictive pericarditis; Calcification; Pericardiectomy; Right heart failure

Core tip: Idiopathic contrictive pericarditis (CP) with extensive pericardial calcification is rarely seen. We hereby report a case of CP with extensive “eggshell” calcification of heart, who presented with right heart failure. A need for multimodality imaging and hemodynamic assessment is discussed in the article.

INTRODUCTION

Constrictive pericarditis (CP) is an inflammatory disease of pericardium. Its etiology includes infection, connective tissue disorders, chest trauma, irradiation, post-cardiac surgery and idiopathic-type[1]. Extensive pericardial calcification is rarely seen in CP. We hereby report a case of CP presented with extensive “eggshell” like calcification of whole of pericardium.

CASE REPORT

A 43-year-old male presented with shortness of breath, NYHA class-III of 6-mo duration. There was no history of fever, productive cough, joint pain, orthopnea and pedal edema. Clinical examination revealed a jugular venous pulse of 18 cm, prominent X and Y descents. Cardiac auscultation revealed a pericardial knock. Two-dimensional echocardiogram showed 10-mm thick, calcified pericardium; about 25%-variation in mitral diastolic flow velocities with respiration and a dilated inferior vena cava of 24-mm dimension. Fluoroscopy revealed dense circumferential calcification all around the heart (Figure 1). A computed tomography (CT) scan confirmed the circumferential thick pericardial calcification like an eggshell, encircling the heart (Figures 2 and 3). Angiography revealed normal epicardial coronaries without any external compression. Hemodynamic data revealed elevated mean right atrial (RA) pressure of 20 mmHg. Right ventricle (RV) and left ventricle (LV) end-diastolic pressure was 23 and 28 mmHg, respectively. Pulmonary artery systolic and mean pulmonary capillary wedge pressures were 44 and 24 mmHg, respectively. There was near equalization of elevated RA, RV and LV end-diastolic pressures. Right atrial pressure tracing showed prominent X and Y descent (Figure 4A), and ventricular pressure tracing showed typical “dip-and-plateau configuration” (Figure 4B) suggestive of CP. He had surgical pericardiectomy for densely calcified CP. Following median thoracotomy, thickened, calcified, firmly adherent pericardium was resected and excised from anterior and left lateral aspect of the heart (Figure 5). The densely adherent pericardium from surface of right atrium and posterior wall was not resected. Histopathology of pericardium revealed dystrophic calcification without any evidence of granulomatous or giant cell inflammation. Mycobacterial and fungal cultures of the excised pericardium were negative. He had uneventful recovery and was discharged on 7^th post-operative day. A post–operative CT scan showed absence of calcified pericardium along antero-lateral surface of the heart (Figures 2C and D). He remained asymptomatic during 1-year follow-up.

Figure 1 Fluoroscopy images in antero-posterior (A), lateral (B) and left anterior oblique 30º (C) views show circumferential pericardial calcification around the heart.

Figure 2 Computed tomography. Non-contrast computed tomography reconstructed images in coronal (A) and sagittal (B) planes show thick, calcified pericardium around the heart. A repeat CT following partial pericardiectomy in coronal (C) and sagittal (D) planes show residual calcified pericardium at right atrial and posterior surface of the heart. Calcified pericardium is absent along the antero-lateral surface of the heart. CT: Computed tomography.

Figure 3 Pre-operative non-contrast computed tomography reconstructed volume rendered image shows pericardial “eggshell” calcification around the heart.

Figure 4 Hemodynamic tracing during catheterization. A: Right atrial (RA) pressure tracing shows prominent X and Y descent; B: Ventricular pressure tracing shows typical “dip-and-plateau configuration” during diastole. RV: Right ventricle; LV: Left ventricle.

Figure 5 Surgical image shows resected thick and calcified pericardium (white arrow) from anterior surface of heart.

DISCUSSION

Constrictive pericarditis is a sequale of chronic inflammation of pericardium. Among the various etiologies, idiopathic type is a common cause for CP[1,2], which was present in index case. The clinical presentation is mostly of right heart failure. Any patient presented with dyspnea, raised jugular venous pulse, hepatic and systemic venous congestion should be evaluated for CP. Pericardial calcification in chest X-ray can suggest CP, however it is present in only 5%-27% of cases[3]. Extensive “eggshell” like calcification is rarely seen in CP[4], as was present in index case. Echocardiography is an initial imaging test for diagnosis of CP[5]. In patients with equivocal echocardiography findings, CT or Cardiac Magnetic Resonance can confirm the diagnosis[5]. The extent of severe calcification and involvement of adjacent structures is clearly defined by CT, as demonstrated in index case. Cardiac catheterization is required for hemodynamic assessment and to rule out coronary compression by calcified thickened pericardium[6]. A multi-modality imaging and hemodynamic assessment is essential for proper evaluation of CP[5]. Surgical pericardiectomy is the definite treatment for CP. Those with calcified and firmly adherent pericardium, median thoracotomy is preferred over antero-lateral thoracotomy[7]. Calcified CP also has higher surgical risk, incomplete pericardial resection and poor hemodynamic outcomes following surgery[7]. However, index case had a successful outcome following partial antero-lateral pericardiectomy via median sternotomy approach. In conclusion, we present a case of idiopathic CP with extensive eggshell calcification of the heart, who had successful surgical pericardiectomy, and had a favourable long term outcome.

COMMENTS

Case characteristics

A 43-years-old male presented with shortness of breath, New York Heart Association class-III of 6-mo duration.

Clinical diagnosis

Clinical examination revealed a raised jugular venous pulse with prominent X and Y descents.

Differential diagnosis

Cardiac auscultation revealed a pericardial knock. Two-dimensional echocardiogram showed 10-mm thick, calcified pericardium with significant trans-mitral diastolic flow velocities variation.

Imaging diagnosis

Fluoroscopy revealed dense circumferential calcification all around the heart. A computed tomography scan confirmed the circumferential thick pericardial calcification like an eggshell, encircling the heart. Cardiac catheterization revealed near equalization of elevated right atrial, right ventricle, and left ventricle end-diastolic pressures and ventricular pressure tracing showed typical “dip-and-plateau” pattern suggestive of constrictive pericarditis.

Pathological diagnosis

He had surgical pericardiectomy for densely calcified CP. Histopathology of pericardium revealed dystrophic calcification without any evidence of granulomatous or giant cell inflammation. An importance of multi-modality imaging with hemodynamic assessment is discussed in management of constrictive pericarditis.

Peer-review

This is an interesting article on calcified constrictive pericarditis. The paper is nicely documented.