Prognostic Value of Change in Cardiac Index After Prostacyclin Initiation in Pediatric Pulmonary Hypertension
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ORIGINAL ARTICLE
Prognostic Value of Change in Cardiac Index After Prostacyclin Initiation in Pediatric Pulmonary Hypertension Patrick D. Evers1 · Paul J. Critser2 · Michelle Cash2 · Melissa Magness2 · Russel Hirsch2,3 Received: 7 February 2020 / Accepted: 16 September 2020 © Springer Science+Business Media, LLC, part of Springer Nature 2020
Abstract Invasive hemodynamic assessment remains the gold standard for the diagnosis of pediatric pulmonary hypertension and for longitudinal assessment of response to therapy. This analysis sought to describe the changes in hemodynamic variables after initiation of prostacyclin therapy and determine which changes bear predictive power of adverse clinical outcomes. A retrospective chart review of established patients at Cincinnati Children’s Hospital with pulmonary arterial hypertension (PAH) who required prostacyclin therapy between 2004 and 2018 was performed. The baseline hemodynamic parameters at diagnosis as well as change in those parameters between initial catheterization and post-prostacyclin initiation catheterization were independent variables. Cox proportional hazard regression and recursive partitioning analysis were used to characterize which hemodynamic factors predicted the composite adverse outcome (CAO) defined as death, lung transplantation, or reverse Pott’s shunt surgery. During the study period, 29 patients met inclusion criteria in which there were 7 CAOs: 4 deaths, 3 lung transplants, and 2 reverse Pott’s shunts. Median time between catheterizations was 86 days and between the initiation of prostacyclin therapy and the second catheterization was 54 days. Cox regression revealed that only baseline pulmonary artery pressure (> 51 mmHg) and a failure to increase cardiac index illustrated statistically significant hazard for occurrence of the CAO (p 20 mmHg and indexed pulmonary vascular resistance ≥ 3 WU [10]. Patients were excluded from the analysis if adequate hemodynamic data could not be obtained (e.g., diagnostic catheterization prior to the initiation of therapy occurred at an outside hospital from which records were not available). This retrospective analysis was approved by the institutional review board of Cincinnati Children’s Hospital and Medical Center.
Data Elements Hemodynamic data extracted from the initial catheterization included indexed pulmonary vascular resistance (iPVR), transpulmonary gradient (TPG), mean pulmonary artery pressure (mPAP), right atrial mean pressure (mRA), right atrial a-wave (aRA), mixed venous saturation, pulmonary blood flow cardiac index (Qp), systemic blood flow cardiac index (Qs), and right ventricular stroke work (RVSW) which was calculated as peak RV pressure multiplied by stroke volume as previously described [11]. Incorporating data from the catheterization following prostacyclin initiation, additional variables included the numerical change (∆) in all the aforementioned variables in response to prostacyclin therapy. Given the heterogeneity of hemodynamics in the pediatric population across the age spectr
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