Key result
Decreases in dPAP and mPAP during acute vasoreactivity testing were associated with time to death (18.3% overall mortality) or transplantation (5.3%) in children with IPAH/HPAH.
Why the study?
To analyze the diagnostic and prognostic impact of dPAP in pure precapillary PH by comparing dPAP- and mPAP-derived metrics and evaluating their association with transplant-free survival in children with IPAH/HPAH.
Do hemodynamic metrics derived from diastolic pulmonary arterial pressure (dPAP) at rest and during acute vasoreactivity testing predict transplant-free survival in children with idiopathic or heritable pulmonary arterial hypertension?
Cohort (n=246)
Do hemodynamic metrics derived from diastolic pulmonary arterial pressure (dPAP) at rest and during acute vasoreactivity testing predict transplant-free survival in children with idiopathic or heritable pulmonary arterial hypertension?
Hemodynamic responses during acute vasoreactivity testing provide stronger prognostic information for transplant-free survival than resting hemodynamics in pediatric patients with pulmonary arterial hypertension.
May refine prognostication in pediatric PAH beyond resting hemodynamics; leaves open therapeutic implications pending prospective validation.
BACKGROUND: Diastolic pulmonary arterial pressure (dPAP) is regarded to be less sensitive to flow metrics as compared to mean PAP (mPAP), and was therefore proposed for the assessment of a precapillary component in patients with postcapillary pulmonary hypertension (PH). To analyze the diagnostic and prognostic impact of dPAP in patients with pure precapillary PH, we purposed to compare the correlation between dPAP and mPAP, as well as hemodynamically-derived calculations [ratio of PAP to systemic arterial pressure (PAP/SAP), pulmonary vascular resistance index (PVRI), transpulmonary gradient (TPG)], using both dPAP and mPAP, at rest and during acute vasoreactivity testing (AVT) in children with idiopathic or heritable pulmonary arterial hypertension (IPAH/HPAH). Furthermore, we aimed to assess the association of these metrics (at baseline and changes after AVT) with transplant-free survival. METHODS: We conducted a retrospective analysis of the TOPP (Tracking Outcomes and Practice in Pediatric Pulmonary Hypertension) registry including 246 IPAH/HPAH patients. Of these, 45 children (18.3%) died, and 13 (5.3%) received lung transplantation during the observation period. RESULTS: dPAP and mPAP-derived variables showed almost linear relationship. Higher mPAP/mSAP, and dPAP-/mPAP-derived PVRI at rest was associated with time to death/transplantation. At maximum AVT-response, the decrease of dPAP and mPAP, diastolic pulmonary gradient (DPG) and TPG, as well as dPAP/dSAP and mPAP/mSAP was associated with time to death/transplantation, showing higher significance than corresponding baseline values. Remarkably, no predictive value was found for PVRI-reduction during AVT, neither dPAP- nor mPAP-derived. CONCLUSIONS: There is a strong relationship between dPAP and mPAP-derived variables. According to our results, hemodynamics during AVT (irrespectively of dPAP- or mPAP-derived) may have more prognostic implications than resting hemodynamics in children with IPAH/HPAH, except for PVRI.
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Apitz et al. (2021) conducted a cohort in Idiopathic or heritable pulmonary arterial hypertension (IPAH/HPAH) (n=246). Hemodynamics during acute vasoreactivity testing (AVT) vs. Resting hemodynamics was evaluated on Transplant-free survival (time to death/transplantation). Decreases in dPAP and mPAP during acute vasoreactivity testing were associated with time to death (18.3% overall mortality) or transplantation (5.3%) in children with IPAH/HPAH.
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