Stenting of isolated pulmonary artery stenosis in an adult patient with RNF213 p.Arg4810Lys variant

Misaki Kanezawa; Hiroto Shimokawahara; Ayane Miyagi; Hiromi Matsubara

doi:10.1016/j.cjca.2024.05.003

Stenting of isolated pulmonary artery stenosis in an adult patient with RNF213 p.Arg4810Lys variant

Can J Cardiol. 2024 May 9:S0828-282X(24)00356-8. doi: 10.1016/j.cjca.2024.05.003. Online ahead of print.

Authors

Misaki Kanezawa¹, Hiroto Shimokawahara², Ayane Miyagi¹, Hiromi Matsubara¹

Affiliations

¹ Department of Cardiology, NHO Okayama Medical Center, Okayama, Japan.
² Department of Cardiology, NHO Okayama Medical Center, Okayama, Japan. Electronic address: hiroto.shimokk@gmail.com.

PMID: 38734204
DOI: 10.1016/j.cjca.2024.05.003

Abstract

Isolated peripheral pulmonary artery stenosis (PPAS) in adulthood related to the RNF213 p.Arg4810Lys variant is characterized by bilateral nonuniform pulmonary artery stenoses without congenital heart disease or systemic congenital syndromes. Progressive stenoses worsening results in elevated pulmonary artery pressure (PAP), eventually leading to patient fatality. However, established treatments for PPAS are currently lacking. We present the case of a 40-year-old woman diagnosed with isolated PPAS with the RNF213 p.Arg4810Lys variant. She underwent 27 stent deployments in 20 procedures, leading to dramatic symptomatic and hemodynamic improvements, with a mean PAP decrease from 83 to 25 mmHg.

Publication types

Case Reports