The treatment dilemma in adult patients with peripheral pulmonary artery stenosis of diverse etiologies

Zahra Hosseini,Mohammadreza Baay,Ehsan Khalilipur,Zahra Khajali,Kiara Rezaei Kalantari,Bahram Mohebbi,Iman Harirforoosh,Ata Firouzi

doi:10.1186/s43044-021-00190-5

Abstract

BackgroundPeripheral pulmonary artery stenosis (PPAS) is a rare and underdiagnosed phenomenon that is reported infrequently in adult patients. Most patients with PPAS have concomitant congenital heart diseases, a history of palliative surgical therapies during childhood, or syndromic characteristics. Acquired cases are rare, and they are underestimated in adulthood and managed inappropriately.Case presentationThis case series describes 3 adult patients with PPAS of diverse etiologies and discusses their underlying causes, diagnostic modalities, and treatment strategies.ConclusionsIn patients with PPAS, sufficient heed should be paid to endovascular interventions such as balloon dilation and primary or bailout stenting, not least vis-à-vis the type and size of balloons or stents as well as complications and preventive strategies.

Highlights

Peripheral pulmonary artery stenosis (PPAS) is a rare and underdiagnosed phenomenon that is reported infrequently in adult patients
In patients with PPAS, sufficient heed should be paid to endovascular interventions such as balloon dilation and primary or bailout stenting, not least vis-à-vis the type and size of balloons or stents as well as complications and preventive strategies
Peripheral pulmonary artery stenosis (PPAS) is a rare and underdiagnosed condition that is seldom reported in adulthood

Summary

Conclusions

PPAS is a rare congenital or acquired phenomenon whose misdiagnosis or inappropriate treatment is not unusual. A high index of suspicion is required to distinguish patients who present with irreversible PH and damage. The main treatment strategy is the balloon dilation of the most stenotic segments. With respect to appropriate balloon size selection in PPAS, we recommend that the balloon diameter-to-average proximal and distal vessel diameter (pre- and post-stenotic vessel segments) ratio be considered 1.1/1. Given that in most patients, the stenotic segments are frequently bilateral and multiple, BPA is usually performed in several sessions. In those with the involvement of more proximal parts (i.e., the main PA or the bifurcated site) or those who do not respond to BPA, scoring balloons and stenting are alternative options.

Background

Coarctation at the bifurcation of the MPA extending into the RPA and the LPA

Findings

Discussion