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Pseudo-Harlequin syndrome: a case report.
Álvaro Velasco1,2, Roberto Jiménez Manso1, Laura Domínguez Pérez1
1Department of Cardiology, Hospital Universitario 12 de Octubre, Av. de Córdoba, s/n, Madrid 28041, Spain.
European Heart Journal. Case Reports
|July 8, 2026
Summary
A teenager with Danon disease experienced refractory shock despite ECMO support. Surgical intervention for pericardial tamponade, mimicking Harlequin syndrome, successfully restored perfusion and organ function.
Area of Science:
- Cardiology
- Cardiovascular Surgery
- Critical Care Medicine
Background:
- A 16-year-old male with Danon disease presented with severe biventricular dysfunction (INTERMACS 3) and reversible pulmonary hypertension.
- The patient was awaiting heart transplantation and receiving ambulatory levosimendan when he developed refractory cardiogenic shock.
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