BACKGROUND : Portopulmonary hypertension (POPH) is part of Group 1 of the clinical classification of pulmonary hypertension and represents 5-15% of patients with pulmonary hypertension, with a 5-year mortality rate of 40%. The implementation of preoperative pulmonary antihypertensive treatment allows liver transplantation depending on clinical response, which constitutes potential curative treatment. Uncontrolled pulmonary hypertension is a major risk factor of perioperative morbimortality in the context of liver transplantation. In case of major hemodynamic instability, extracorporeal membrane oxygenation (ECMO) can be placed to manage circulatory failure. We describe a case of a patient with POPH in whom an emergency ECMO was implanted during liver transplantation complicated by an intraoperative worsening of pulmonary vascular resistances leading to cardiac arrest.

CASE REPORT : A 16-year-old patient with POPH had an orthotopic liver transplantation (OLT) after management of pulmonary hypertension with a triple antihypertensive therapy, which was complicated by hemorrhagic shock. Management of hemorrhagic shock led to greatly increased pulmonary vascular resistances, which led to a perioperative cardiac arrest, necessitating the implantation of a veno-arterial ECMO, allowing the completion of critical surgical steps before admission to the intensive care unit.

CONCLUSIONS : POPH is a challenge in the perioperative setting. OLT is a therapeutic option in that setting. ECMO may be necessary for patients with POPH in the perioperative hemodynamic management during OLT. In highly selected cases, VA-ECMO implantation and timing should be discussed by a multidisciplinary team before induction. The emergency perioperative implantation of ECMO is a realistic alternative.