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International practice heterogeneity in pre-transplant management of pulmonary hypertension related to pediatric left heart disease

Hopper, Rachel K ; van der Have, Oscar LU orcid ; Hollander, Seth A ; Dipchand, Anne I ; Perez de Sa, Valeria LU ; Feinstein, Jeffrey A and Tran-Lundmark, Karin LU (2023) In Pediatric Transplantation 27(2). p.1-9
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 m
2 (56%) post-AVT (84%). The highest post-AVT PVR... (More)

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 m
2 (56%) post-AVT (84%). The highest post-AVT PVR ever accepted for HTx ranged from 3-14.4 (median 6) WU m
2 . 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.

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author
; ; ; ; ; and
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
Humans, Child, Hypertension, Pulmonary/complications, Heart Transplantation, Heart Failure/complications, Vascular Resistance/physiology, Heart-Assist Devices, Vasodilator Agents, Treatment Outcome, Retrospective Studies
in
Pediatric Transplantation
volume
27
issue
2
article number
e14461
pages
1 - 9
publisher
Wiley-Blackwell
external identifiers
  • pmid:36593638
  • scopus:85145405356
ISSN
1399-3046
DOI
10.1111/petr.14461
language
English
LU publication?
yes
additional info
© 2023 Wiley Periodicals LLC.
id
c68ca043-4abb-4726-9982-9a76b093e651
date added to LUP
2023-02-12 18:10:03
date last changed
2024-06-13 18:05:40
@article{c68ca043-4abb-4726-9982-9a76b093e651,
  abstract     = {{<p>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.</p><p>METHODS: We sought to characterize international practice by surveying physicians at pediatric HTx centers.</p><p>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 m<br>
 2 (56%) post-AVT (84%). The highest post-AVT PVR ever accepted for HTx ranged from 3-14.4 (median 6) WU m<br>
 2 . 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.<br>
 </p><p>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.</p>}},
  author       = {{Hopper, Rachel K and van der Have, Oscar and Hollander, Seth A and Dipchand, Anne I and Perez de Sa, Valeria and Feinstein, Jeffrey A and Tran-Lundmark, Karin}},
  issn         = {{1399-3046}},
  keywords     = {{Humans; Child; Hypertension, Pulmonary/complications; Heart Transplantation; Heart Failure/complications; Vascular Resistance/physiology; Heart-Assist Devices; Vasodilator Agents; Treatment Outcome; Retrospective Studies}},
  language     = {{eng}},
  month        = {{01}},
  number       = {{2}},
  pages        = {{1--9}},
  publisher    = {{Wiley-Blackwell}},
  series       = {{Pediatric Transplantation}},
  title        = {{International practice heterogeneity in pre-transplant management of pulmonary hypertension related to pediatric left heart disease}},
  url          = {{http://dx.doi.org/10.1111/petr.14461}},
  doi          = {{10.1111/petr.14461}},
  volume       = {{27}},
  year         = {{2023}},
}