Researchers developed an approach to predict postinterventional pulmonary artery hemodynamics in patients with peripheral pulmonary artery stenosis (PPAS) associated with Alagille syndrome (ALGS) and Williams syndrome (WS).

“Our study represents the first to accurately model the severely nonlinear and hypertensive hemodynamics of PPAS in WS and ALGS and to further predict postinterventional pressures, the primary indicators of success in PPAS repair,” said the authors of the study published in the Journal of the American Heart Association.

They highlighted 4 major accomplishments:


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  • Development of an automated tuning framework to efficiently identify boundary conditions that best achieve clinically measured pulmonary artery pressures and flow splits
  • Autoregulatory adaptation of the cardiac output and downstream arterial resistance in response to intervention‐induced hemodynamic perturbations
  • Development of a spatial resistance “map” to allow clinicians to easily assess the clinical import of stenoses, constituting the foundation for a virtual PPAS treatment planning platform
  • Controlled hemodynamic comparison between different transcatheter strategies within the same patient cohort

They found that patients with ALGS and WS required extensive transcatheter interventions to reduce pulmonary artery pressures and right ventricular stroke work.

In patients with ALGS and WS without long‐segment stenosis, extensive stenting and/or angioplasty were able to decrease pulmonary artery pressures to half systemic levels. However, the improvements were limited when compared to those of surgical reconstruction. Moreover, transcatheter therapy was not effective in treating long‐segment stenosis.

The comparison of different transcatheter interventions within the same patient cohort also suggested that isolated proximal stenting has limited benefit in complex PPAS.

“Ultimately, the optimal treatment strategy must be chosen alongside consideration of other factors, including the availability of surgical expertise and the higher morbidity rates associated with transcatheter interventions,” the authors concluded.

Reference

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;0(0):e023532. doi:10.1161/JAHA.121.023532