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. 2022 Jul 1;12(3):e12104.
doi: 10.1002/pul2.12104. eCollection 2022 Jul.

Abnormal platelet aggregation in pediatric pulmonary hypertension

Stephanie S Handler  1 , Jing Jin  2 , Michelle T Ogawa  3 , Jeffrey A Feinstein  3   4 , Clara Lo  5
Affiliations

Abnormal platelet aggregation in pediatric pulmonary hypertension

Stephanie S Handler et al. Pulm Circ. .

Abstract

Endogenous prostacyclin stimulates pulmonary vasodilation and inhibits platelet aggregation. For the synthetic analog treprostinil, used in the treatment of pulmonary hypertension (PH), conflicting, anecdotal evidence exists regarding its effects on clinically relevant platelet function. This study investigated whether treprostinil therapy results in inhibition of platelet aggregation in pediatric PH patients. This is a single institution, prospective, cohort study. Pediatric patients ≤18 years of age on medical therapy for PH underwent platelet function testing by light transmission aggregometry with U-46619-a stable analog of endoperoxide prostaglandin H2, exhibiting properties similar to thromboxane A2 (TXA2). Results were compared for those on continuous treprostinil therapy (TRE) versus those on other, non-prostacyclin therapies (non-TRE). Thirty-five patients were enrolled: 18 in the TRE group and 17 in the non-TRE group. There was no difference in platelet aggregation abnormalities between the two groups: 44% (n = 8) in the TRE group and 41% (n = 7) in the non-TRE group were abnormal. Furthermore, subgroup analysis showed no difference based on treprostinil dosing. This study demonstrated similar, moderately high rates of abnormal platelet aggregation in pediatric PH patients on continuous treprostinil therapy compared to those on other, non-prostacyclin therapies. The high rate of abnormal platelet aggregation in the entire cohort, however, warrants follow-up study to identify a potential inherent risk in this population.

Keywords: pediatrics; prostacyclin; pulmonary arterial hypertension.

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Conflict of interest statement

The authors declare no conflicts of interest.

Figures

Figure 1
Figure 1
PAP‐8 light transmission aggregometer (Bio/Data Corporation): This system measures the rate and extent of platelet aggregation, agglutination, and activation stimulated by individual agonists; platelet agglutination/aggregation is indicated by degree of light transmission.
Figure 2
Figure 2
Eighteen patients on treprostinil infusion: 8 with abnormal and 10 with normal aggregation results. Dose (Y‐axis) shown by group (abnormal vs. normal aggregation).
Figure 3
Figure 3
Eighteen patients on treprostinil infusion: 8 with abnormal and 10 with normal aggregation results. Duration of therapy (Y‐axis) shown by group (abnormal vs. normal aggregation).
See this image and copyright information in PMC

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