Virtual Transcatheter Interventions for Peripheral Pulmonary Artery Stenosis in Williams and Alagille Syndromes

Abstract

Background Despite favorable outcomes of surgical pulmonary artery (PA) reconstruction, isolated proximal stenting of the central PAs is common clinical practice for patients with peripheral PA stenosis in association with Williams and Alagille syndromes. Given the technical challenges of PA reconstruction and the morbidities associated with transcatheter interventions, the hemodynamic consequences of all treatment strategies must be rigorously assessed. Our study aims to model, assess, and predict hemodynamic outcomes of transcatheter interventions in these patients. Methods and Results Isolated proximal and “extensive” interventions (stenting and/or balloon angioplasty of proximal and lobar vessels) were performed in silico on 6 patient‐specific PA models. Autoregulatory adaptation of the cardiac output and downstream arterial resistance was modeled in response to intervention‐induced hemodynamic perturbations. Postintervention computational fluid dynamics predictions were validated in 2 stented patients and quantitatively assessed in 4 surgical patients. Our computational methods accurately predicted postinterventional PA pressures, the primary indicators of success for treatment of peripheral PA stenosis. Proximal and extensive treatment achieved median reductions of 14% and 40% in main PA systolic pressure, 27% and 56% in pulmonary vascular resistance, and 10% and 45% in right ventricular stroke work, respectively. Conclusions In patients with Williams and Alagille syndromes, extensive transcatheter intervention is required to sufficiently reduce PA pressures and right ventricular stroke work. Transcatheter therapy was shown to be ineffective for long‐segment stenosis and pales hemodynamically in comparison with published outcomes of surgical reconstruction. Regardless of the chosen strategy, a virtual treatment planning platform could identify lesions most critical for optimizing right ventricular afterload.

Temporal evolution of mechanical stimuli from vascular remodeling in response to the severity and duration of aortic coarctation

“…R c /R tot was then iteratively adjusted using reported ranges 32 to achieve measured ow and BP at outlet branches for each time point. External tissue support was prescribed to limit non-physiological high-frequency oscillations of models using spring and damping constants of 10 3 g/(cm 2 s 2 ) and 10 4 g/(cm 2 s), respectively 33,34 . Simulations were run for ve cardiac cycles to ensure periodicity and converged solutions 35 .…”

Cardiovascular fluid dynamics: a journey through our circulation

“…Yang, Feinstein & Vignon-Clementel (2016) and Yang et al (2018) performed virtual pulmonary artery reconstruction for patients with Alagille syndrome and achieved accurate predictions of postoperative pulmonary artery flows via adaptive outflow boundary conditions. Recently, Lan et al (2022) performed virtual intervention in Williams and Alagille syndrome patients and showed that stenting only proximal lesions is often insufficient to reduce overall vascular resistance and thus right ventricular pressures (figure 4b).…”

A probabilistic neural twin for treatment planning in peripheral pulmonary artery stenosis

“…Peripheral pulmonary artery stenosis (PPAS) is a condition occurring most frequently in conjunction with congenital heart diseases such as Williams or Allagile Syndrome, 1 or less commonly after surgical interventions or as an isolated congenital condition 2 . The varying comorbidities and complex positioning of stenosis continue to pose significant challenges in the diagnosis and treatment of PPAS 3 .…”