A rare cause of effusive-constrictive pericarditis

Abstract

Effusive-constrictive pericarditis (ECP) is an uncommon diagnosis, frequently missed due to its heterogeneous presentation, but a potentially reversible cause of heart failure. A 62-year-old Caucasian male presented with remittent right heart failure and mild-moderate pericardial effusion. Following an initial diagnosis of idiopathic pericarditis, indomethacin was started, but the patient shortly relapsed, presenting with severe pericardial effusion and signs of cardiac tamponade, requiring pericardiocentesis. ECP was diagnosed on cardiac catheterization. Cardiac computed tomography showed non-calcified, mildly thickened and inflamed parietal pericardium. Pericardiectomy was performed with symptoms remission. On histological examination of pericardium, chronic non-necrotizing granulomatous inflammation was noted. Polymerase chain reaction assay was positive for non-tuberculous mycobacteria. This case represents a rare finding of ECP with unusual presentation due to atypical mycobacteriosis in a non-immunocompromised patient and in a non-endemic area. Pericardiectomy can be an effective option in cases unresponsive to anti-inflammatory treatment, even in the absence of significant pericardial thickening or calcification.

Keywords: Cardiac tamponade; Effusive-constrictive pericarditis; Non-tuberculous mycobacteria; Pericardiectomy; Pericardiocentesis.

Figure 1

Transthoracic echocardiogram: diastolic (A) and systolic (B) parasternal long‐axis views showing moderate pericardial effusion with initial proto‐diastolic collapse of the right ventricle (red star).

Figure 2

Cardiovascular magnetic resonance: four‐chamber cine sequence showing circumferential pericardial effusion (A, white arrow) and bilateral pleural effusion (A, black asterisk). Mid‐cavity short‐axis (B) and two‐chamber long‐axis T1‐weighted post‐contrast sequences (C) showing circumferential pericardial effusion with proteinaceous characteristics (white asterisks) and absence of myocardial and pericardial late gadolinium enhancement.

Figure 3

Cardiac catheterization after pericardiocentesis shows combined left ventricular (A, red line) and right ventricular (A, blue line) catheterization. The red box highlights the ‘dip‐and‐plateau’ pressure pattern (otherwise known as the ‘square root sign’). Right atrium catheterization (B) shows persistently elevated right atrial pressure after pericardiocentesis (medium pressure: 17 mmHg).

Figure 4

Cardiac computed tomography: thickening of parietal pericardium (arrows), with no evidence of pericardial calcification. Bilateral pleural effusion is also present.

Figure 5

Surgical pathological analysis of the pericardiectomy specimen. Macroscopic view (A) and histological panoramic view (B) confirming diffuse severe fibrous thickening (B, Heidenhain trichrome stain). At histology, multiple foci of chronic inflammation are evident (C, haematoxylin–eosin, 50×; D, CD3+ T‐lymphocytes; E, CD68+ macrophages; F, CD4+ T‐helper lymphocytes; G, CD8+ cytotoxic T‐lymphocytes; H, CD20+ B‐lymphocytes; all D–H 100×). Scale bars represent 500 μm (B), 100 μm (C), and 50 μm (D–H).