Safety and Clinical Outcomes of Endovascular Treatment of Adult-onset Pulmonary Artery Stenosis

Mujeeb A. Sheikh, MD; Mohammed A. Chowdhury, MD, MRCP (UK); George V. Moukarbel, MD


J Invasive Cardiol. 2016;28(5):202-208. 

In This Article

Abstract and Introduction


Background. Pulmonary artery stenosis (PAS) in adults is a rare condition. The role of endovascular stent implantation as a therapeutic option has not been clearly defined.

Methods and Results. We performed a retrospective review of all cases of adult-onset PAS published in Pubmed/Medline from 1990 to 2013. A total of 126 cases of adult-onset PAS were identified, out of which 46 cases (37%) were treated with an endovascular intervention. Symptomatic improvement was reported in 98% of cases. Average translesional gradient at baseline was 47 ± 20 mm Hg, which reduced to 8 ± 11 mm Hg after stenting (P<.001). Mean preprocedural pulmonary systolic artery pressures were 79 ± 26 mm Hg, which reduced post procedure to 50 ± 20 mm Hg (P=.02). There were no immediate adverse events reported related to procedure. In-stent restenosis was reported in 7 cases on follow-up.

Conclusion. Endovascular treatment appears safe and effective for symptom relief in adult-onset PAS and should be considered as an alternative treatment option in patients with prohibitive surgical risk.


Pulmonary artery stenosis (PAS), particularly branch stenosis, is one of the most common forms of vascular stenosis associated with congenital heart disease.[1,2] There has been extensive experience with pulmonary artery stenting in children, demonstrating significant improvement in symptoms and improved outcomes.[3–5] In contrast to pediatric PAS, adult-onset PAS is rare and has been associated with a wide variety of clinical conditions including fibrosing mediastinitis, lung parenchymal tumors, primary vascular tumors, and lung transplantation. PAS in these cases results from extrinsic compression or direct vessel involvement from inflammatory or neoplastic process in the adjacent structures. Reported clinical manifestations of PAS are variable and range from dyspnea due to inadequate pulmonary perfusion to right heart failure from chronic pressure overload. Multiple published anecdotal cases and case series have documented efficacy of endovascular stent implantation in the treatment of symptomatic adult-onset PAS. We therefore reviewed the published literature on adult-onset PAS treated with endovascular approach and provide a comprehensive report on the etiology, clinical presentation, diagnostic modalities, procedural details, and outcome of these patients.