International practice heterogeneity in pre-transplant management of pulmonary hypertension related to pediatric left heart disease
(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
- Hopper, Rachel K ; van der Have, Oscar LU ; Hollander, Seth A ; Dipchand, Anne I ; Perez de Sa, Valeria LU ; Feinstein, Jeffrey A and Tran-Lundmark, Karin LU
- organization
- publishing date
- 2023-01-03
- 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
-
- scopus:85145405356
- pmid:36593638
- 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
- 2024-09-06 02:11:58
@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}}, }