Hemodynamic and prognostic impact of the diastolic pulmonary arterial pressure in children with pulmonary arterial hypertension-a registry-based analysis

Christian Apitz; Rolf M F Berger; D Dunbar Ivy; Tilman Humpl; Damien Bonnet; Maurice Beghetti; Dietmar Schranz; Heiner Latus

doi:10.21037/cdt-20-934

Hemodynamic and prognostic impact of the diastolic pulmonary arterial pressure in children with pulmonary arterial hypertension-a registry-based analysis

Cardiovasc Diagn Ther. 2021 Aug;11(4):1037-1047. doi: 10.21037/cdt-20-934.

Authors

Christian Apitz^{1

2}, Rolf M F Berger³, D Dunbar Ivy⁴, Tilman Humpl⁵, Damien Bonnet⁶, Maurice Beghetti⁷, Dietmar Schranz², Heiner Latus^{2

8}

Affiliations

¹ Division of Pediatric Cardiology, University Children's Hospital Ulm, Ulm, Germany.
² Pediatric Heart Center, University Children's Hospital Frankfurt/Giessen, Frankfurt, Germany.
³ Center for Congenital Heart Diseases, Pediatric Cardiology, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands.
⁴ Pediatric Cardiology, Children's Hospital Colorado, University of Colorado School of Medicine, Aurora, CO, USA.
⁵ Division of Neonatology and Critical Care Medicine, University Children's Hospital Bern, Bern, Switzerland.
⁶ Centre de Référence Malformations Cardiaques Congénitales Complexes, M3C-Necker Hospital for Sick Children, Assistance Publique des Hôpitaux de Paris, Pediatric Cardiology, University de Paris, Paris, France.
⁷ Pediatric Cardiology Unit, Children's University Hospital, Centre Universitaire Romand de Cardiologie et Chirurgie Cardiaque Pédiatrique, University of Geneva and Lausanne, Geneva, Switzerland.
⁸ Department of Pediatric Cardiology and Congenital Heart Disease, Deutsches Herzzentrum München, Technische Universität München, München, Germany.

Abstract

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.

Keywords: Pulmonary arterial hypertension (PAH); acute vasoreactivity testing (AVT); cardiac catheterization; pediatric cardiology; pulmonary arterial pressure (PAP); pulmonary vascular resistance.