Lung Transplantation and Reverse Potts Shunt for Pulmonary Hypertension in the Pediatric Population

Pulmonary hypertension (PH) is a potentially life-threatening disorder characterized by abnormalities of the pulmonary vasculature, causing elevated pulmonary artery pressures, which can result in right ventricular dysfunction. Patients with suprasystemic right ventricular pressure unresponsive to aggressive medical therapy have limited treatment options, including balloon atrial septostomy, reverse Potts shunt, or lung transplantation. The decision to proceed to a palliative reverse Potts shunt or lung transplantation, and the choice of one or the other, depends on surgical, medical, and psychosocial factors. In this manuscript, we discuss the definition, pathophysiology, and current treatments for PH in relation to pediatric lung transplantation, including referral indications and special considerations for transplant candidates. We review the reverse Potts shunt as a palliative option for patients with severe PH, discussing indications, patient outcomes, surgical techniques, and the relative risks/benefits compared to lung transplantation. Finally, we propose an algorithm to assist pediatric cardiologists, pulmonologists, intensivists, surgeons, and other healthcare providers in the decision-making between a palliative reverse Potts shunt procedure and lung transplantation for patients with severe PH based on currently available data.