Kussmaul's Sign in Effusive Constrictive Pericarditis

Case report

A 67-year old man with mitral valve prolapse and moderate regurgitation was admitted because of dyspnoea, bilateral ankle swelling and hypotension. Close inspection of the jugular veins identified Kussmaul’s sign, a typical increase in the central venous pressure during inspiration (Figure 1; arrows). He had no history or clinical evidence of infection, tumours, uraemia, trauma, surgery or radiation. Transthoracic echocardiography revealed moderate diffuse pericardial effusion (PE) (Figure 2, arrows) with paradoxical interventricular septum bounce (see video 1*). Persistence of Kussmaul’s sign and symptoms of acute right heart failure aher pericardiocentesis (170 ml exudate, no infections and neoplastic cells) prompted the clinical suspicion of idiopathic effusive-constrictive pericarditis. Diagnosis was supported by cardiac magnetic resonance (CMR), showing mild residual PE and diffuse thickening of the pericardium (Figure 3, arrows) with contrast enhancement at the pericardial edges (Figure 4, arrows) and septal bounce (see video 2*). Diagnosis of effusive-constrictive pericarditis was confirmed by typical elevated ventricular filling pressures at cardiac catheterisation (equilibration of ventricular diastolic pressures with dip-plateau waveform) and open surgery (pericardiectomy) showing diffuse parietal (Figure 5A–B) and visceral pericardial thickening (Figure 5C–D). One year follow-up showed complete clinical relief with almost no residual pericardial thickening at CMR.