{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,17]],"date-time":"2026-09-17T16:44:28Z","timestamp":1789663468867,"version":"4.0.1"},"reference-count":42,"publisher":"MDPI AG","issue":"12","license":[{"start":{"date-parts":[[2023,12,12]],"date-time":"2023-12-12T00:00:00Z","timestamp":1702339200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"IRCCS Foundation Ca\u2019 Granda Ospedale Maggiore Policlinico"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Life"],"abstract":"<jats:p>Background: Unilateral proximal interruption of the pulmonary artery (UPIPA) is a rare congenital disease, and its optimal management remains undefined in the existing literature. The occasional necessity for pneumonectomy is still supported by limited evidence. Methods: A systematic review of the literature was conducted using the PubMed search engine, focusing on UPIPA cases that received pneumonectomy. Thirty-one pertinent articles were selected and included in the analysis. A case reported from our institution was included in the analysis. Results: We found 25 adults and seven children affected by UPIPA who received an indication for pneumonectomy, plus an additional case that was reported by our institution. Among adult patients, the predominant indication was hemoptysis (57%), followed by suspected or confirmed lung cancer (23%). Approximately 46% of surgical procedures were classified as urgent or emergent. Postoperative complications were observed in 36% of cases, with no recorded mortality. In pediatric cases, pneumonectomy was primarily a life-saving intervention, performed urgently or emergently in 75% of instances. A possible late complication in pediatric patients involves a mediastinal shift leading to respiratory distress, which may be mitigated using an inflatable prosthesis. Conclusions: Pneumonectomy achieves complete resolution of UPIPA symptoms. In the adult population, its primary indication is hemoptysis, with procedures conducted in both elective and urgent\/emergent settings. Despite a mortality rate of zero, a notable proportion of patients may experience postoperative complications. In pediatric cases, the clinical presentation varies more extensively, and pneumonectomy is typically reserved for life-threatening situations, emphasizing the need for careful patient selection.<\/jats:p>","DOI":"10.3390\/life13122328","type":"journal-article","created":{"date-parts":[[2023,12,12]],"date-time":"2023-12-12T04:28:31Z","timestamp":1702355311000},"page":"2328","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Pneumonectomy for Unilateral Proximal Interruption of Pulmonary Artery: A Case Series from the Literature"],"prefix":"10.3390","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4136-8293","authenticated-orcid":false,"given":"Giovanni","family":"Mattioni","sequence":"first","affiliation":[{"name":"Thoracic Surgery and Lung Transplantation Unit, IRCCS Foundation Ca\u2019 Granda Ospedale Maggiore Policlinico, 20122 Milan, Italy"},{"name":"School of Thoracic Surgery, University of Milan, 20122 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8571-121X","authenticated-orcid":false,"given":"Mario","family":"Nosotti","sequence":"additional","affiliation":[{"name":"Thoracic Surgery and Lung Transplantation Unit, IRCCS Foundation Ca\u2019 Granda Ospedale Maggiore Policlinico, 20122 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9809-7140","authenticated-orcid":false,"given":"Lorenzo","family":"Rosso","sequence":"additional","affiliation":[{"name":"Thoracic Surgery and Lung Transplantation Unit, IRCCS Foundation Ca\u2019 Granda Ospedale Maggiore Policlinico, 20122 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4510-5167","authenticated-orcid":false,"given":"Alessandro","family":"Palleschi","sequence":"additional","affiliation":[{"name":"Thoracic Surgery and Lung Transplantation Unit, IRCCS Foundation Ca\u2019 Granda Ospedale Maggiore Policlinico, 20122 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3767-0449","authenticated-orcid":false,"given":"Davide","family":"Tosi","sequence":"additional","affiliation":[{"name":"Thoracic Surgery and Lung Transplantation Unit, IRCCS Foundation Ca\u2019 Granda Ospedale Maggiore Policlinico, 20122 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4303-6244","authenticated-orcid":false,"given":"Paolo","family":"Mendogni","sequence":"additional","affiliation":[{"name":"Thoracic Surgery and Lung Transplantation Unit, IRCCS Foundation Ca\u2019 Granda Ospedale Maggiore Policlinico, 20122 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2023,12,12]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"1253","DOI":"10.2214\/ajr.179.5.1791253","article-title":"\u201cAbsent\u201d Pulmonary Artery in One Adult and Five Pediatric Patients: Imaging, Embryology, and Therapeutic Implications","volume":"179","author":"Apostolopoulou","year":"2002","journal-title":"Am. J. Roentgenol."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"4988","DOI":"10.21037\/jtd.2017.11.49","article-title":"Isolated unilateral absence of pulmonary artery in adulthood: A clinical analysis of 65 cases from a case series and systematic review","volume":"9","author":"Wang","year":"2017","journal-title":"J. Thorac. Dis."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"1471","DOI":"10.1378\/chest.122.4.1471","article-title":"Isolated Unilateral Absence of a Pulmonary Artery","volume":"122","author":"Blom","year":"2002","journal-title":"Chest"},{"key":"ref_4","first-page":"105906","article-title":"The PRISMA 2020 statement: An updated guideline for reporting systematic reviews","volume":"88","author":"Page","year":"2021","journal-title":"BMJ"},{"key":"ref_5","first-page":"105","article-title":"Unilateral pulmonary artery atresia in an adult: A case report","volume":"26","author":"Smith","year":"2019","journal-title":"Respir. Med. Case Rep."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"e00511","DOI":"10.1002\/rcr2.511","article-title":"Isolated unilateral pulmonary artery agenesis presenting with massive hemoptysis","volume":"8","author":"Kang","year":"2020","journal-title":"Respirol. Case Rep."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"507","DOI":"10.1510\/icvts.2010.250795","article-title":"Right pulmonary artery agenesis and coronary-to-bronchial artery aneurysm","volume":"12","author":"Leborgne","year":"2011","journal-title":"Interact. Cardiovasc. Thorac. Surg."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"32","DOI":"10.1186\/s12957-020-1810-6","article-title":"Left lung cancer in a patient with congenital unilateral absence of the left pulmonary artery: A case report and literature review","volume":"18","author":"Wang","year":"2020","journal-title":"World J. Surg. Oncol."},{"key":"ref_9","first-page":"363","article-title":"Pneumonectomy for congenital isolated unilateral pulmonary artery agenesis","volume":"66","author":"Britton","year":"2011","journal-title":"Med. J. Malaysia"},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"370","DOI":"10.1016\/j.ejcts.2008.10.053","article-title":"Pulmonary artery agenesis and Kommerell\u2019s diverticulum presenting with hemoptysis","volume":"35","author":"Rousou","year":"2009","journal-title":"Eur. J. Cardio-Thorac. Surg."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"2192","DOI":"10.1016\/j.athoracsur.2014.08.062","article-title":"Pulmonary Artery Agenesis Associated with Emphysema and Multiple Invasive Non-Small Cell Lung Cancers","volume":"99","author":"Makdisi","year":"2015","journal-title":"Ann. Thorac. Surg."},{"key":"ref_12","first-page":"4752835","article-title":"Metastatic Non-Small-Cell Lung Cancer in the Setting of Unilateral Agenesis of the Left Pulmonary Artery: A Case Report and Comprehensive Literature Review","volume":"2019","author":"Agzarian","year":"2019","journal-title":"Case Rep. Oncol. Med."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"238","DOI":"10.1016\/j.crad.2006.10.006","article-title":"Imaging features of isolated unilateral pulmonary artery agenesis presenting in adulthood: A review of four cases","volume":"62","author":"Griffin","year":"2007","journal-title":"Clin. Radiol."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"48","DOI":"10.14797\/mdcj-12-1-48","article-title":"Chronic Recurrent Unilateral Pulmonary Infection: Result of Congenital Unilateral Agenesis of Pulmonary Artery","volume":"12","author":"Ramchandani","year":"2016","journal-title":"Methodist. Debakey Cardiovasc. J."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"100","DOI":"10.1007\/s11748-012-0107-2","article-title":"Bullous lung disease and bronchiectasis in unilateral absent right pulmonary artery","volume":"61","author":"Betigeri","year":"2013","journal-title":"Gen. Thorac. Cardiovasc. Surg."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"312","DOI":"10.1016\/j.athoracsur.2008.06.015","article-title":"Coiling of Major Aortopulmonary Collateral Arteries with Agenesis of the Left Pulmonary Artery Prior to Pneumonectomy","volume":"87","author":"Redmond","year":"2009","journal-title":"Ann. Thorac. Surg."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"924","DOI":"10.1093\/icvts\/ivab364","article-title":"Thoracoscopic pneumonectomy in patient with unilateral absence of the left pulmonary artery accompanied by the left lung cancer","volume":"34","author":"Kononets","year":"2022","journal-title":"Interact. Cardiovasc. Thorac. Surg."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"649","DOI":"10.1046\/j.1440-1827.2001.01251.x","article-title":"Isolated unilateral absence of a pulmonary artery: Influence of systemic circulation on alveolar capillary vessels","volume":"51","author":"Maeda","year":"2001","journal-title":"Pathol. Int."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"1553","DOI":"10.1016\/0003-4975(93)91108-Y","article-title":"Pneumonectomy for unremitting hemoptysis in unilateral absence of pulmonary artery","volume":"55","author":"Bekoe","year":"1993","journal-title":"Ann. Thorac. Surg."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"312","DOI":"10.1213\/01.ane.0000226089.53300.7a","article-title":"Differential Changes of Alveolar Gas Concentrations During Anesthetic Induction of a Patient with an Absent Right Pulmonary Artery","volume":"103","author":"Jiang","year":"2006","journal-title":"Anesth. Analg."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"525","DOI":"10.1007\/s00595-006-3182-0","article-title":"Isolated Unilateral Absence of a Pulmonary Artery Treated by Pneumonectomy in an Adult: Report of a Case","volume":"36","author":"Ohtsuka","year":"2006","journal-title":"Surg. Today"},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"1183","DOI":"10.1510\/icvts.2008.180430","article-title":"Isolated absence of the right pulmonary artery as a cause of massive hemoptysis","volume":"7","author":"Santos","year":"2008","journal-title":"Interact. Cardiovasc. Thorac. Surg."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"1748","DOI":"10.1016\/S0003-4975(01)02611-X","article-title":"Exsanguinating hemoptysis revealing the absence of left pulmonary artery in an adult","volume":"72","author":"Thomas","year":"2001","journal-title":"Ann. Thorac. Surg."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"255","DOI":"10.1016\/S0003-4975(02)03558-0","article-title":"Hemoptysis resulting from unilateral pulmonary artery agenesis","volume":"74","author":"Farghly","year":"2002","journal-title":"Ann. Thorac. Surg."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"166","DOI":"10.1016\/j.hlc.2011.10.012","article-title":"Unilateral Pulmonary Artery Agenesia Complicated by Massive Haemoptysis in An Adult Female","volume":"21","author":"Sanna","year":"2012","journal-title":"Heart Lung Circ."},{"key":"ref_26","first-page":"148","article-title":"Case of the Month #134. Unilateral absence of a pulmonary artery","volume":"59","author":"Ciura","year":"2008","journal-title":"Can. Assoc. Radiol. J."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"199","DOI":"10.1186\/s13019-023-02255-9","article-title":"Hemoptysis: Unilateral pulmonary artery atresia? a case report","volume":"18","author":"Maini","year":"2023","journal-title":"J. Cardiothorac. Surg."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"294","DOI":"10.1016\/0003-4975(91)91356-Z","article-title":"Neonatal pneumonectomy for isolated unilateral pulmonary artery agenesis","volume":"52","author":"Canver","year":"1991","journal-title":"Ann. Thorac. Surg."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"1116","DOI":"10.1016\/j.jpedsurg.2004.03.065","article-title":"Long-term follow-up in a patient after pneumonectomy and SILASTIC\u00ae prosthetic placement for unilateral pulmonary artery agenesis","volume":"39","author":"Nichol","year":"2004","journal-title":"J. Pediatr. Surg."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"119","DOI":"10.1007\/BF00798220","article-title":"Scimitar syndrome with absence of the right pulmonary artery: A case with volume-induced, reversible, left-sided pulmonary hypertension","volume":"13","author":"Beitzke","year":"1992","journal-title":"Pediatr. Cardiol."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"251","DOI":"10.1016\/S0003-4975(98)00404-4","article-title":"Unilateral absence of pulmonary artery and ventricular septal defect in an infant","volume":"66","author":"Imanaka","year":"1998","journal-title":"Ann. Thorac. Surg."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"1100","DOI":"10.1002\/ppul.25844","article-title":"A case of recurrent pneumothoraces in a newborn","volume":"57","author":"George","year":"2022","journal-title":"Pediatr. Pulmonol."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"973","DOI":"10.1378\/chest.106.3.973","article-title":"Three-Dimensional Reconstruction of Ultrafast Chest CT for Diagnosis and Operative Planning in a Child with Right Pneumonectomy Syndrome","volume":"106","author":"Newman","year":"1994","journal-title":"Chest"},{"key":"ref_34","first-page":"TD04","article-title":"Proximal Interruption of the Pulmonary Artery: A Case Series","volume":"9","author":"Anand","year":"2015","journal-title":"J. Clin. Diagn. Res."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"188","DOI":"10.1097\/00000441-199503000-00011","article-title":"Case Report: Congenital Absence of the Left Pulmonary Artery Accompanied by Ipsilateral Emphysema and Adenocarcinoma","volume":"309","author":"Roman","year":"1995","journal-title":"Am. J. Med. Sci."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"210","DOI":"10.1186\/s13643-016-0384-4","article-title":"Rayyan\u2014A web and mobile app for systematic reviews","volume":"5","author":"Ouzzani","year":"2016","journal-title":"Syst. Rev."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"56","DOI":"10.1097\/RTI.0000000000000373","article-title":"Proximal Interruption of the Pulmonary Artery","volume":"34","author":"Williams","year":"2019","journal-title":"J. Thorac. Imaging"},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"968652","DOI":"10.3389\/fped.2022.968652","article-title":"Proximal interruption of the pulmonary artery: A review of radiological findings","volume":"10","author":"Zhang","year":"2022","journal-title":"Front. Pediatr."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"335","DOI":"10.1016\/j.thorsurg.2015.04.006","article-title":"Management of Complications After Pneumonectomy","volume":"25","author":"Groth","year":"2015","journal-title":"Thorac. Surg. Clin."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"607","DOI":"10.1007\/s00246-010-9689-6","article-title":"Therapeutic Strategies in Children with an Isolated Unilaterally Absent Proximal Pulmonary Artery","volume":"31","author":"Krammoh","year":"2010","journal-title":"Pediatr. Cardiol."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"2235","DOI":"10.1016\/j.jtcvs.2013.11.041","article-title":"Pulmonary artery resuscitation for isolated ductal origin of a pulmonary artery","volume":"148","author":"Mery","year":"2014","journal-title":"J. Thorac. Cardiovasc. Surg."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"71","DOI":"10.1007\/s00246-017-1729-z","article-title":"Contralateral Pulmonary Hypertension Following Resuscitation of Unilateral Ductal Origin of a Pulmonary Artery: A Multi-institutional Review","volume":"39","author":"Agrawal","year":"2018","journal-title":"Pediatr. Cardiol."}],"container-title":["Life"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2075-1729\/13\/12\/2328\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T21:36:56Z","timestamp":1760132216000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2075-1729\/13\/12\/2328"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,12,12]]},"references-count":42,"journal-issue":{"issue":"12","published-online":{"date-parts":[[2023,12]]}},"alternative-id":["life13122328"],"URL":"https:\/\/doi.org\/10.3390\/life13122328","relation":{},"ISSN":["2075-1729"],"issn-type":[{"value":"2075-1729","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,12,12]]}}}