Safety and Feasibility of Riociguat Therapy for the Treatment of Chronic Pulmonary Arterial Hypertension in Infancy

Regan E Giesinger; Amy H Stanford; Brady Thomas; Steven H Abman; Patrick J McNamara

doi:10.1016/j.jpeds.2022.11.026

Safety and Feasibility of Riociguat Therapy for the Treatment of Chronic Pulmonary Arterial Hypertension in Infancy

J Pediatr. 2023 Apr:255:224-229.e1. doi: 10.1016/j.jpeds.2022.11.026. Epub 2022 Dec 1.

Authors

Regan E Giesinger¹, Amy H Stanford², Brady Thomas², Steven H Abman³, Patrick J McNamara²

Affiliations

¹ Department of Pediatrics, University of Iowa, Iowa City, IA. Electronic address: regan-giesinger@uiowa.edu.
² Department of Pediatrics, University of Iowa, Iowa City, IA.
³ Department of Pediatrics, University of Colorado, Denver, CO.

PMID: 36462687
DOI: 10.1016/j.jpeds.2022.11.026

Abstract

The effects of riociguat, an oral-soluble guanylate-cyclase stimulator, were studied in 10 infants with chronic pulmonary arterial hypertension. Respiratory status (n = 8/10), right heart dilation (n = 7/10), function (n = 9/10), and chronic pulmonary arterial hypertension (n = 8/10) improved. Median decrement in systolic (12 [4, 14]), diastolic (14 [7, 20]), and mean arterial (14 [10, 17]) pressures were noted; no critical hypotension or hypoxemia occurred.

Publication types

Case Reports

MeSH terms

Familial Primary Pulmonary Hypertension
Feasibility Studies
Guanylate Cyclase
Humans
Hypertension, Pulmonary* / drug therapy
Infant
Pulmonary Arterial Hypertension*

Substances

riociguat
Guanylate Cyclase