Iatrogenic esophageal dysmotility as a barrier to transplantation in pulmonary arterial hypertension
Michael S. Miller,
Shelsey W. Johnson,
Alexander R. Opotowsky,
Michael J. Landzberg,
Nirmal S. Sharma,
Hilary J. Goldberg,
Alexandra K. Wong,
Alison S. Witkin,
Josanna Rodriguez-Lopez,
Ronald H. Goldstein,
Bradley A. Maron,
Bradley M. Wertheim
Affiliations
Michael S. Miller
Department of Medicine, Brigham and Women’s Hospital and Harvard Medical School, Boston, Massachusetts
Shelsey W. Johnson
Division of Pulmonary and Critical Care Medicine, Department of Medicine, Brigham and Women’s Hospital and Harvard Medical School, Boston, Massachusetts; Department of Pulmonary, Allergy, Sleep, and Critical Care Medicine, Veterans Affairs Boston Healthcare System, Boston, Massachusetts
Alexander R. Opotowsky
Department of Pediatrics, University of Cincinnati School of Medicine, Cincinnati, Ohio; Adult Congenital Heart Program, Heart Institute, Cincinnati Children’s Hospital Medical Center, Cincinnati, Ohio
Michael J. Landzberg
Division of Cardiovascular Medicine, Department of Medicine, Brigham and Women’s Hospital and Harvard Medical School, Boston, Massachusetts; Department of Pediatrics, Boston Children’s Hospital, Boston, Massachusetts
Nirmal S. Sharma
Division of Pulmonary and Critical Care Medicine, Department of Medicine, Brigham and Women’s Hospital and Harvard Medical School, Boston, Massachusetts; Department of Pulmonary, Allergy, Sleep, and Critical Care Medicine, Veterans Affairs Boston Healthcare System, Boston, Massachusetts
Hilary J. Goldberg
Division of Pulmonary and Critical Care Medicine, Department of Medicine, Brigham and Women’s Hospital and Harvard Medical School, Boston, Massachusetts
Alexandra K. Wong
Department of Medicine, Division of Pulmonary and Critical Care Medicine, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts
Alison S. Witkin
Department of Medicine, Division of Pulmonary and Critical Care Medicine, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts
Josanna Rodriguez-Lopez
Department of Medicine, Division of Pulmonary and Critical Care Medicine, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts
Ronald H. Goldstein
Department of Pulmonary, Allergy, Sleep, and Critical Care Medicine, Veterans Affairs Boston Healthcare System, Boston, Massachusetts; The Pulmonary Center, Division of Pulmonary, Allergy, Sleep and Critical Care Boston University School of Medicine, Boston, Massachusetts
Bradley A. Maron
Department of Medicine, University of Maryland School of Medicine, Baltimore, MD and the University of Maryland-Institute for Health Computing, Bethesda, Maryland
Bradley M. Wertheim
Division of Pulmonary and Critical Care Medicine, Department of Medicine, Brigham and Women’s Hospital and Harvard Medical School, Boston, Massachusetts; Department of Pulmonary, Allergy, Sleep, and Critical Care Medicine, Veterans Affairs Boston Healthcare System, Boston, Massachusetts; Corresponding author: Bradley M. Wertheim, Brigham and Women’s Hospital, New Research Building, 0630-O, 77 Ave Louis Pasteur, Boston, MA 02115.
Esophageal dysmotility is identified as a contraindication to lung transplantation at some centers due to increased risks of acute rejection, pulmonary infection, and chronic lung allograft dysfunction. Phosphodiesterase-type 5 inhibitors (PDE5i) are a cornerstone pharmacotherapy for pulmonary arterial hypertension (PAH) and are known to exert off-target effects that may impact lung transplant candidacy, including impaired esophageal contractility and decreased lower esophageal sphincter tone. We report 2 patients with PAH who were initially declined listing for lung transplantation due to iatrogenic esophageal dysmotility induced by PDE5is. Upon discontinuation of PDE5i therapy, these patients experienced significant improvement in esophageal motility within 14 days and met the criteria for transplant listing at their centers. Recognizing and mitigating the off-target effects of PDE5i medications is critical for maximizing access to transplant for patients with PAH.
