Chronic calcific effusive constrictive pericarditis: a rare entity within the spectrum of pericardial diseases—a case report

Abstract

Effusive constrictive pericarditis is a rare complication of pericardial inflammation wherein a rapidly forming pericardial effusion results in tamponade physiology in combination with an inflamed and constricting visceral pericardium. Consequently, after pericardial draining, the right atrial pressures remain elevated indicating impaired ventricular filling despite evacuating the life-threatening effusion. Effusive constrictive pericarditis has traditionally been viewed as a transitional state from acute pericarditis with an effusion to a more chronic constrictive pericarditis, and more recently has been postulated to belong to a spectrum of inflammatory pericardial diseases rather than a mutually exclusive disease state. We present a rare case of calcific effusive constrictive pericarditis. A 55-year-old male with a remote history of tuberculosis exposure presents with 7 months of dyspnoea and recent anasarca. Baseline chest X-ray (CXR) (Panel A) shows a rim of calcium in the anterior chest reflecting a calcified pericardium anterior to the right ventricle which was corroborated on a gated computed tomography (CT) chest without contrast (Panel B, arrow). Despite significant shadowing on a transthoracic echocardiogram, baseline non-invasive haemodynamics demonstrated annulus reversus and an expiratory hepatic vein flow reversal ratio in diastole >0.8 consistent with constrictive physiology (Panel C, asterisk demonstrating diastolic reversal, Video 1). A baseline cardiac magnetic resonance (CMR) demonstrated a large loculated exudative pericardial effusion (increased fluid signal on T1 and T2 weighted sequences, Panel D, asterisk), with inversion of the right ventricle (Video 2 and 3). Otherwise, the pericardium was thickened (Panel D, arrow) with a mildly increased pericardial signal on delayed hyperenhancement sequences suggesting end-stage inflammatory pericarditis. The patient underwent pericardiocentesis, removing 200 cc of bloody effusion and subsequently radical pericardiectomy (Panels H and I). On follow-up, the dyspnoea and anasarca had improved and an echocardiogram demonstrated normalization of the annulus reversus and the hepatic flow suggesting resolution of constrictive physiology. (Panel A) Lateral chest X-ray demonstrating a calcified rim in the anterior chest (red arrow). (Panel B) Gated CT chest without contrast demonstrating a calcified pericardial rim anteriorly (red arrow). (Panel C) Tissue Doppler imaging of the medial and lateral mitral annulus reflecting annulus reversus and a hepatic vein pulse wave Doppler demonstrating increased expiratory diastolic flow reversal. (Panel D) Dark blood T1 weighted axial image on CMR demonstrating a thickened pericardium (white arrow) surrounding a pericardial effusion with increased signal suggesting an exudative effusion. (Panel E) Intraoperative images from the radical pericardiectomy and explanted pericardium with areas of calcification throughout. Supplementary material Supplementary material is available at European Heart Journal - Case Reports online. Supplementary Material ytaa322_Supplementary_Data Click here for additional data file.