ArticleJournal of thoracic disease2017
Isolated unilateral absence of pulmonary artery in adulthood: a clinical analysis of 65 cases from a case series and systematic review.
Article in Journal of thoracic disease, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 46 papers.
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46 citing papers in PubMed, 69 citations in OpenAlex.
- Congenital Absence of the Left Pulmonary Artery Associated With Right-Sided Aortic Arch Presenting as Hemoptysis in an Adult: A Case Report.Clinical case reports · 2026Article
- Vascular etiologies of unilateral pulmonary fibrosis: Case series and literature overview.Radiology case reports · 2026Article
- Interruption of the right middle and lower lobe pulmonary arteries combined with arterial aneurysm formation at the origin of the right subclavian artery branch: a case report.Frontiers in cardiovascular medicine · 2026Article
- Article
- Circumflex to right lung fistula embolization for acute coronary syndrome secondary to steal syndrome: a case report.European heart journal. Case reports · 2025Article
- Decreased Hemithorax in an Adult.JMA journal · 2025Article
- Successful balloon angioplasty of right pulmonary coarctation with left pulmonary artery interruption in a cat: a long-term outcome follow-up case report.The Journal of veterinary medical science · 2025Article
- Breathing With Half a Pulmonary Circuit: Three Cases of Unilateral Pulmonary Artery Agenesis.Respirology case reports · 2025Article
- Right Pulmonary Artery Agenesis in an Adult: A Case Report of Silent Pulmonary Hypoplasia.Cureus · 2025Article
- An exceptional case of coronary artery collateralization in unilateral pulmonary artery agenesis: implications for right heart failure in the elderly.Journal of geriatric cardiology : JGC · 2025Article
- Case report: unilateral absence of the left pulmonary artery with left gastric artery collateral supply and hematologic disorder.Oxford medical case reports · 2025Article
- Isolated unilateral absence of the right pulmonary artery with hemoptysis in adulthood: a case report.AME case reports · 2025Article
- Severe pulmonary hypertension associated with unilateral pulmonary artery hypoplasia: A case report.ARYA atherosclerosis · 2025Article
- Left Lower Lobectomy for Lung Cancer Complicated by Unilateral Absence of the Left Pulmonary Artery: A Case Report.Surgical case reports · 2025Article
- Isolated unilateral absence of the pulmonary artery (IUAPA) presenting with progressive interstitial shadows: A case report.Respirology case reports · 2024Article
- Isolated left hypoplasia of the pulmonary artery accompanied by lung hypoplasia and chronic bronchial disease in a cat.The Journal of veterinary medical science · 2024Article
- A rare association of the absence of left pulmonary artery with ventricular septal defect, pulmonary hypertension, and more interestingly, patent ductus arteriosus in an adult patient: Case report and literature review.Clinical case reports · 2024Article
- Unusual Developmental Vascular Anomalies: Insights From a Chest Physician's Perspective.Cureus · 2024Article
- Delayed Diagnosis of Unilateral Absence of Pulmonary Artery Presenting with Recurrent Bronchopneumonia.CASE (Philadelphia, Pa.) · 2024Article
- Three-dimensional Computed Tomography of Asymmetrical Interstitial Lung Disease.Internal medicine (Tokyo, Japan) · 2024Article
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Authors and funding
4 authors at 1 institution in 1 country.
Funding
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Abstract
backgroundIsolated unilateral absence of pulmonary artery (UAPA) in adulthood is a rare congenital anomaly. Although some case reports exist, the clinical symptomatology, lung parenchymal features, collateral circulation and therapeutic approaches in adult patients with isolated UAPA remain unknown. The objectives of this study are to investigate the clinical characteristics, elucidate the correlation between clinical symptomatology and radiology, and summarize treatment of adult patients with isolated UAPA.
methodsCases of adult patients with isolated UAPA who had been diagnosed at our hospital and identified from PubMed, EMBASE and Web of Science from 1990 to 2016 were analyzed.
resultsHemoptysis was present in 41.5% of patients, exertional dyspnea in 41.5%, and recurrent respiratory infection in 35.4%. Lung parenchymal abnormalities were found on chest computed tomography (CT) scan, including bronchiectasis, which occurred in 30.2% of the patients, interstitial changes in 14.0%, and multiple bullae in 14.0% of the patients. Exertional dyspnea was more frequent in patients with pulmonary hypertension than in those without pulmonary hypertension (P<0.001). Recurrent respiratory infection were more frequent in patients with bronchiectasis than in those without bronchiectasis (P<0.001). Hypertrophic bronchial, phrenic, internal thoracic and intercostal arteries were found in 71.9%, 46.9%, 43.8%, and 43.8% of the patients, respectively. Pneumonectomy reduced hemoptysis in seven cases. Oral phosphodiesterase inhibitors or endothelin receptor antagonist improved exertional dyspnea in three cases with pulmonary hypertension.
conclusionsClinicians should be aware of undiagnosed cases of isolated UAPA in adults with unexplained hemoptysis or exertional dyspnea. Early recognition and management of isolated UAPA in adult patients are crucial to avoid the devastating effect of massive hemoptysis or severe pulmonary hypertension (PHT) in the long term.
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