Timothy Klouda, Wai Wong, Francis Fynn-Thompson, Jesse Esch, Maureen B. Josephson, Usha S. Krishnan, Levent Midyat, Mary P. Mullen
Boston Children’s Hospital and Harvard. Children’s Hospital of Philadelphia and Perelman School of Medicine at the University of Pennsylvania Medical School. Columbia University Irving Medical Center and Morgan Stanley Children’s Hospital of New York Presbyterian. Children’s Hospital of Pittsburgh and University of Pittsburgh School of Medicine.
United States
Pediatric Transplantation
Pediatr Transplant 2026; 30:
DOI: 10.1111/petr.70263
Abstract
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.
Category
Surgical and Catheter-mediated Interventions for Pulmonary Vascular Disease
Lung Transplantation for Pulmonary Vascular Disease
Review Articles Concerning Pulmonary Vascular Disease
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: No