Pseudo-Harlequin syndrome: a case report

This case describes a teenager with Danon disease, severe biventricular dysfunction (INTERMACS 3), and combined, reversible pulmonary hypertension, who was receiving ambulatory levosimendan while awaiting elective heart transplantation when he developed refractory shock.

A 16-year-old male was transferred in SCAI-D cardiogenic shock despite high-dose vasoactive therapy, with rising lactate and acute kidney injury. A multidisciplinary Heart/Shock team indicated hybrid central-peripheral veno-arterial-venous extracorporeal membrane oxygenation (ECMO) with left ventricle unloading. After initial stabilization, he developed falling ECMO flows, increasingly negative drainage pressures, hypotension, oliguria, and lactate elevation. Echocardiography revealed collapsed ventricles and mild pericardial effusion, but tamponade seemed unlikely due to an open pericardium. Progressive upper-body congestion and cyanosis later appeared, mimicking Harlequin syndrome. Surgical exploration revealed circumferential pericardial clots causing tamponade. Their removal resulted in immediate perfusion, ECMO flow, and organ recovery.

This represents an atypical tamponade presentation under ECMO, mimicking Harlequin physiology. We propose the term 'pseudo-Harlequin syndrome'. Suspicion for tamponade must remain high in ECMO patients, even when classical signs are absent.