ArticlePulmonary circulation2015
Peripheral pulmonary artery stenosis as a cause of pulmonary hypertension in adults.
Article in Pulmonary circulation, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 20 papers.
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Who cites it
20 citing papers in PubMed, 39 citations in OpenAlex.
- Non-Invasive Evaluation of Pulmonary Artery Stenosis in Congenital Heart Disease Patients Using Phase Contrast MRI.Cardiovascular engineering and technology · 2026Article
- Pulmonary Artery Stenting for Widespread Distal Congenital Stenoses.JACC. Case reports · 2025Article
- Efficacy and prognosis of multimodal angiography-interventions for chronic pulmonary artery stenosis and pulmonary hypertension.American journal of translational research · 2025Article
- Prevalence and Clinical Characteristics of HeterozygousJournal of the American Heart Association · 2024Article
- Endovascular interventions in cancer patients with compromise of the mediastinal vasculature: a review.Mediastinum (Hong Kong, China) · 2024Review
- The results of genetic analysis and clinical outcomes after stent deployment in adult patients with isolated peripheral pulmonary artery stenosis.The European respiratory journal · 2023Article
- Adult-onset idiopathic peripheral pulmonary artery stenosis.The European respiratory journal · 2023Article
- Exercise feature and predictor of prognosis in patients with pulmonary artery stenosis-associated pulmonary hypertension.ESC heart failure · 2022Article
- Balloon Pulmonary Angioplasty for Takayasu Arteritis and Peripheral Pulmonary Artery Stenosis Mimicking Chronic Thromboembolic Pulmonary Hypertension.Life (Basel, Switzerland) · 2022Review
- Tracheobronchial Tree Ossification in a 5-Year-Old Boy with Keutel Syndrome: A Case Report.The journal of Tehran Heart Center · 2022Article
- Ring Finger Protein 213 in Moyamoya Disease With Pulmonary Arterial Hypertension: A Mini-Review.Frontiers in neurology · 2022Review
- The treatment dilemma in adult patients with peripheral pulmonary artery stenosis of diverse etiologies.The Egyptian heart journal : (EHJ) : official bulletin of the Egyptian Society of Cardiology · 2021Article
- A histopathological report of a 16-year-old male with peripheral pulmonary artery stenosis and Moyamoya disease with a homozygousRespiratory medicine case reports · 2020Article
- Non-invasive Multimodality Cardiovascular Imaging of the Right Heart and Pulmonary Circulation in Pulmonary Hypertension.Frontiers in cardiovascular medicine · 2019Review
- Acute Pulmonary Vasodilator Testing and Long-Term Clinical Course in Segmental Pulmonary Vascular Disease.Pediatric cardiology · 2018Article
- A review of imaging modalities in pulmonary hypertension.Annals of thoracic medicineReview
- Congenital Pulmonary Vein Stenosis and Pulmonary Artery Branch Stenosis: A Rare Combination.Journal of cardiovascular echographyArticle
- Peripheral pulmonary stenosis with Noonan syndrome treated by balloon pulmonary angioplasty.Pulmonary circulationArticle
- Transcatheter Interventions in Patients With Adult Congenital Heart Disease.Journal of the Society for Cardiovascular Angiography & InterventionsReview
- Mimickers of chronic thromboembolic pulmonary hypertension on imaging tests: a review.Pulmonary circulationArticle
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Authors and funding
4 authors at 1 institution in 2 countries.
Funding
Abstract
Peripheral pulmonary artery stenosis (PPAS) is an underrecognized condition in the adult population. PPAS can lead to pulmonary hypertension but is likely misdiagnosed as either idiopathic pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension. We retrospectively identified adult patients with PPAS either in its isolated form or related to other congenital defects from January 1998 to September 2012. We reviewed the patients' clinical data by using our hospital electronic medical records and/or their paper charts. We identified 6 adult patients with PPAS with an age range of 16-56 years (1 woman and the rest men). Presenting signs and symptoms were thoracic murmurs, progressive dyspnea, and syncope. Three patients had Williams-Beuren syndrome. Pulmonary angiography showed that PPAS was predominantly located in main branches or lobar pulmonary arteries in 5 patients, while in 1 patient the arterial narrowing was at the level of the segmental pulmonary arteries. Right heart catheterization showed a mean pulmonary artery pressure (PAP) ranging from 35 to 60 mmHg. Balloon dilation was performed in all patients, predominantly in the lobar arteries, and it caused a decrease in mean PAP that ranged from 16% to 46% in 5 patients. In 1 patient the mean PAP did not decrease. All but 1 patient had follow-up echocardiograms at 1 year that showed stable echocardiographic findings. Pulmonary hypertension due to PPAS continues to presents a diagnostic challenge. Therefore, a high index of suspicion during the initial evaluation of pulmonary hypertension is essential for its prompt diagnosis and adequate treatment.
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