Anesthetic management for percutaneous reverse Potts shunt creation in children with refractory idiopathic pulmonary arterial hypertension: A case series

Percutaneous reverse Potts shunt improves right ventricular function in patients with suprasystemic idiopathic pulmonary arterial hypertension. There are no data regarding the anesthesia in this high-risk procedure. We report our experience of the anesthetic management for the creation of percutaneous reverse Potts shunt in children with suprasystemic idiopathic pulmonary arterial hypertension. This study included 10 patients presenting with symptomatic idiopathic pulmonary arterial hypertension despite undergoing medical treatment. All patients underwent gradual induction of anesthesia to maintain hemodynamic stability (etomidate, n = 8; ketamine, n = 4). Four patients needed extracorporeal life support: 2 were rescued after cardiac arrest and 2 had elective extracorporeal life support due to preprocedural dysfunctional right ventricle and/or small left ventricle volumes with reduced cardiac output. All patients were admitted to the pediatric cardiac intensive care unit (4 [2-5] days). All patients with extracorporeal life support died. None of the six survivors needed pulmonary transplantation. Both ketamine and etomidate support hemodynamics. High-dose opioid technique has the advantage of blunting noxious stimuli and subsequent increase in pulmonary vascular resistance. We recommend using multimodal monitoring with transesophageal echocardiography. The 100% mortality of extracorporeal life support patients, probably too sick to undergo such procedure, may question its usefulness. Further studies should identify suitable candidates for percutaneous reverse Potts shunt creation.