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Are transcatheter interventions effective in pulmonary artery stenosis associated with ALGS?

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Clinical practice for patients with peripheral pulmonary artery (PA) stenosis associated with Alagille syndrome (ALGS) includes isolated proximal stenting of the central PAs. In this study, researchers sought to assess the outcomes of transcatheter interventions in these patients.

Using a computer simulation, isolated proximal and “extensive” interventions, which included stenting and/or balloon angioplasty of proximal and lobar vessels, were performed on 6 PA models. Postinterventional PA pressures, which are the main indicator for successful treatment, were accurately predicted.

Proximal treatment achieved a median reduction of 14% in main PA systolic pressure, 27% in pulmonary vascular resistance, and 10% in right ventricular stroke work. Extensive treatment achieved a median reduction of 40%, 56%, and 45%, respectively.

The authors concluded that in patients with ALGS extensive transcatheter intervention is required to reduce PA pressures sufficiently but is ineffective for long-segment stenosis. Published data on surgical reconstruction demonstrated better Hemodynamical outcomes compared with transcatheter therapy.

Lan IS, Yang W, Feinstein JA, et al. Virtual Transcatheter Interventions for Peripheral Pulmonary Artery Stenosis in Williams and Alagille Syndromes. J Am Heart Assoc. 2022;11(6):e023532. doi: 10.1161/JAHA.121.023532. Epub 2022 Mar 5. PMID: 35253446.