Rachel K. Hopper, Oscar van der Have, Seth A. Hollander, Anne I. Dipchand, Valeria Perez de Sa, Jeffery A. Feinstein, Karin Tran-Lundmark
Stanford University and Lucille Packard Children’s Hospital. Lund University. Skane University Hospital. University of Toronto and the Hospital for Sick Children
United States, Sweden and Canada
Pediatric Transplantation
Pediatr Transplant 2023;
DOI: 10.1111/petr.14461
Abstract
Background: Elevated pulmonary vascular resistance (PVR) in the setting of left heart failure may contribute to poor outcomes after pediatric heart transplant (HTx), but peri-transplant management is variable.
Methods: We sought to characterize international practice by surveying physicians at pediatric HTx centers.
Results: We received 49 complete responses from 39 centers in 16 countries. Most respondents are pediatric cardiologists (90%), practice at centers offering heart (86%) and lung (55%) transplant, and perform pre-HTx acute vasoreactivity testing (AVT, 88%) in patients with elevated PVR. Half (51%) reported defining a PVR cutoff for HTx eligibility as ≤6 WU m2 (56%) post-AVT (84%). The highest post-AVT PVR ever accepted for HTx ranged from 3-14.4 (median 6) WU m2 . To treat elevated pre-transplant PVR, phosphodiesterase type 5 inhibitors are most common (65%) followed by oxygen (31%), nitric oxide (14%), endothelin receptor antagonists (11%), and prostacyclins (6%). Nearly a third (31%) do not routinely use pulmonary vasodilators without implantation of a left ventricular assist device (LVAD). Case scenarios highlight treatment variability: in a restrictive cardiomyopathy scenario, HTx listing with post-transplant vasodilator therapy was favored, whereas in a Shone’s complex patient with fixed PVR, LVAD ± pulmonary vasodilators followed by repeat catheterization was most common. Management of dilated cardiomyopathy with reactive PVR was variable. Most continue vasodilator therapy until HTx (16%), PVR normalizes (16%) or ≤6 months.
Conclusions: Management of elevated PVR in children awaiting HTx is heterogenous. Evidence-based guidelines are needed to allow for longitudinal determination of optimal outcomes and standardized care.
Category
Class 2. Pulmonary Hypertension Associated with Left Ventricular Systolic or Diastolic Dysfunction
Class 2. Pulmonary Hypertension Associated with Valvular Disease of the Left Side of the Heart
Diagnostic Testing for Pulmonary Vascular Disease. Invasive Testing
Medical Therapy. Efficacy or Lack of Efficacy
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Filed (PHiled). Greater than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: No