Key result
Absence of supranormal cardiac index during prostanoid therapy linked to ~14-fold higher adverse event risk.
Why the study?
Parenteral prostanoid therapy can cause supranormal cardiac index in pediatric pulmonary hypertension, but the incidence, hemodynamic factors, and clinical outcomes associated with it required evaluation.
Does the development of supranormal cardiac index improve outcomes in pediatric pulmonary hypertension patients treated with parenteral prostanoid therapy?
Cohort (n=22)
Does the development of supranormal cardiac index improve outcomes in pediatric pulmonary hypertension patients treated with parenteral prostanoid therapy?
Development of supranormal cardiac index after 3-6 months of parenteral prostanoid therapy in pediatric pulmonary hypertension is associated with a significantly lower risk of death, lung transplant, or Potts shunt.
Supports serial cardiac index assessment in pediatric PH on prostanoids; hypothesis-generating for targeting supranormal values in trials.
Parenteral prostanoid therapy (PPT) can result in supranormal cardiac index (SCI; >4 L/min/m 2 ) in pediatric pulmonary hypertension (PPH) patients. We evaluated the incidence, hemodynamic factors, and outcomes associated with SCI in PPH. This retrospective cohort study included 22 PPH patients on PPT from 2005 to 2020. Hemodynamic profiles were compared between the baseline and 3–6 month follow‐up catheterization in the SCI and non‐SCI cohorts. Cox regression analysis examined time to composite adverse outcome (CAO; Potts shunt, lung transplant, or death) controlling for initial disease severity. SCI developed in 17 (77%) patients, of whom 11 (65%) developed SCI within 6 months. The SCI cohort was characterized by significant augmentation of cardiac index (CI) and stroke volume (SV) as well as reductions in systemic vascular resistance (SVR) and pulmonary vascular resistance (PVR). Conversely, the non‐SCI cohort had unchanged SV despite a modest rise in CI as well as persistent vasoconstriction. After median follow‐up of 4.3 years (range 0.2–13 years), non‐SCI patients were at significantly increased risk for the CAO (5/5: three deaths, two Potts shunts) compared with SCI patients (5/17: two deaths, three lung transplants; adjusted hazard ratio 14.0 [95% confidence interval: 2.1–91.3], p < 0.001). A majority of PPH patients developed SCI within 6–12 months of starting PPT and demonstrated lower risk of adverse outcomes compared with non‐SCI patients. These data suggest that change in SVR and SV after 3–6 months of PPT may be early markers of therapeutic response and prognosis.
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Miles et al. (2023) conducted a cohort in Pediatric pulmonary hypertension (n=22). Supranormal cardiac index (SCI) following parenteral prostanoid therapy vs. Non-supranormal cardiac index (non-SCI) was evaluated on Composite adverse outcome (Potts shunt, lung transplant, or death) (adjusted HR 14.0, 95% CI 2.1-91.3, p=< 0.001). Absence of supranormal cardiac index during parenteral prostanoid therapy significantly increased the risk of death, Potts shunt, or lung transplant (adjusted HR 14.0; 95% CI 2.1-91.3; p<0.001).
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