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

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 ever accepted for HTx ranged from 3-14.4 (median 6) WU m 2 . To treat elevated pretransplant 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.

Pulmonary hemodynamics before and after pediatric heart transplantation

“…27 Nevertheless, the positive aspect of left VAD especially in CMP patients improving outcome after pHTx has been shown. 28,29 The pulmonary hemodynamics after pHTx differ between CHD and CMP patients. In contrast to CMP patients, failing Glenn or Fontan patients show an acute increase of PH immediately after pHTx, which normalizes during long-term follow up.…”

“…Nevertheless, the positive aspect of left VAD especially in CMP patients improving outcome after pHTx has been shown 28,29 …”

Pulmonary vascular disease as a complication of pediatric congenital heart diseases

“…A recent international survey on the pre heart transplant management of PH-LHD in children revealed a significant heterogeneity in practices among recognized expert centers ( 47 ).…”