New therapies for pulmonary hypertension in neonates and children

Abstract

Abbreviations: PH-pulmonary hypertension PPHN-persistent pulmonary hypertension of the Newborn CHD-congenital heart disease PPH-primary pulmonary hypertension BPD-bronchopulmonary dysplasia PGI 2 -prostacyclin VSM-vascular smooth muscle ET-1-endothelin-1 iNO-inhaled nitric oxide HFOV-high frequency oscillatory ventilation NOS-nitric oxide synthase ADMA-asymmetric dimethyl arginine PDE-phosphodiesterase ECMO-extracorporeal membrane oxygenation

Iloprost as ‘rescue’ therapy for pulmonary hypertension of the neonate

“…Nebulized prostacyclin can selectively dilate pulmonary vessels in ventilated lung regions with no effect on unventilated areas thereby decreasing intrapulmonary shunts (2,4). Only one other case has been published on iloprost use in neonates (7), although some experience exists about PGI 2 administration into the lungs (9,10). Iloprost is supposed to have some advantages over PGI 2 (7,10): it is not dissolved in an alkaline solution, potentially dangerous for the lung, it has a longer half-life and its effect is more prolonged (7,10).…”

“…Only one other case has been published on iloprost use in neonates (7), although some experience exists about PGI 2 administration into the lungs (9,10). Iloprost is supposed to have some advantages over PGI 2 (7,10): it is not dissolved in an alkaline solution, potentially dangerous for the lung, it has a longer half-life and its effect is more prolonged (7,10). The positive effect of iloprost may depend on underlying pathology (1,2,4); in fact, it was less evident in CDH which is associated with pulmonary hypoplasia.…”

B-type natriuretic peptide inhibits angiotensin II-induced proliferation and migration of pulmonary arterial smooth muscle cells

“…Previously considered to be a disorder of vasoconstriction and vasodilatation, PAH has been conventionally treated with vasodilators such as prostanoids, endothelin receptor antagonists, or phosphodiesterase-5 inhibitors. 2 Given that the role of vascular remodeling may become progressively more important than vasoconstriction as PAH advances, expanding research efforts are directed toward identifying drugs that inhibit pulmonary vascular remodeling. 3,4 Thus, pharmacologic strategy targeting on anti-proliferation and anti-migration of PASMCs may have therapeutic role for PAH, but is still lacking.…”