Management of a rare triad of trauma-induced pulmonary haemorrhage in an adult with Fontan physiology: a case report

Patients with Fontan circulation represent a high-risk population when facing acute cardiopulmonary decompensation. The use of extracorporeal membrane oxygenation (ECMO) in this patient cohort is rare and technically demanding due to the unique circulatory physiology with passive pulmonary blood flow and predisposition to thromboembolic events.

We report the case of a 17-year-old male patient with a history of extracardiac Fontan palliation who developed a life-threatening diffuse pulmonary haemorrhage following blunt chest trauma. The bleeding pattern precluded both interventional and surgical management. Despite initial stabilization attempts, the patient developed progressive respiratory failure refractory to mechanical ventilation, necessitating the initiation of VV-ECMO therapy as a bridge to recovery.

Ongoing pulmonary bleeding further complicated the course, precluding the implementation of systemic anticoagulation. Consequently, a heparin-free ECMO strategy was employed. Tailored administration of coagulation factors allowed for the preservation of ECMO circuit patency while maintaining haemostasis. Following gradual respiratory improvement, the patient was successfully weaned from ECMO and discharged without residual organ dysfunction.

This case demonstrates the need for personalized ECMO strategies, even in highly challenging haemodynamic scenarios such as Fontan physiology. Favourable outcomes can be achieved when personalized interdisciplinary management is provided in high-volume expert centres for mechanical circulation support (MCS).