The main pulmonary trunk divides into the left and the right pulmonary artery. Pulmonary artery agenesis refers to the absence of one or both pulmonary arteries.
Pulmonary artery agenesis refers to a rare congenital absence of pulmonary artery due to a malformation in the sixth aortic arch. It can occur bilaterally, with both left and right pulmonary arteries being absent, or unilaterally, the absence of either left or right pulmonary artery (UAPA). About 67% of UAPA occurs isolated in the right lung.[1] The absence of pulmonary artery can be an isolated disorder, or accompanied by other related lesions,[2] most commonly Tetralogy of Fallot.[3]
Back in 1868, Fraentzel was the first to report isolated unilateral absence of pulmonary artery (IUAPA) in literature.[4][5] Subsequently, literature has documented a total of 420 cases.[6] The estimated prevalence of IUAPA is 1 in 200,000 adults.[2] No sex preference is observed.[6] Patients with severe complications are usually diagnosed early in age while adult patients are mainly asymptomatic. The overall mortality rate reaches 7%.[7]
Individuals may exhibit a variety of symptoms, or they may not exhibit any symptoms at all. Recurrent lung infections and exercise intolerance are some of the most common symptoms.[8] Serious complications include hemoptysis and pulmonary hypertension. These non-specific symptoms make UAPA challenging to diagnose.[9] Multiple medical imaging techniques are often employed in combination in order to obtain a comprehensive diagnosis.[8]
^Malani, Susheel Kumar; Kashyap, Prashant; Nalawade, Digvijay (2023). "A rare case of unilateral agenesis of left pulmonary artery presenting as severe pulmonary arterial hypertension". Global Cardiology Science & Practice. 2023 (3): e202315. doi:10.21542/gcsp.2023.15. ISSN 2305-7823. PMC 10422877. PMID 37575286.
^ abGovindaraj, V; Joseph, J; Kumar, B Nagamalli; Soman, R (2017). "Isolated left-sided pulmonary artery agenesis with left lung hypoplasia: A report of two cases". Journal of Postgraduate Medicine. 63 (4): 262–264. doi:10.4103/jpgm.JPGM_562_16. ISSN 0022-3859. PMC 5664872. PMID 28862244.
^Shakibi, Jami G.; Rastan, Houshang; Nazarian, Iraj; Paydar, Mansoureh; Aryanpour, Iraj; Siassi, Bijan (1978). "Isolated Unilateral Absence of the Pulmonary Artery". Japanese Heart Journal. 19 (3): 439–451. doi:10.1536/ihj.19.439. PMID 691278.
^Canver, C. C.; Pigott, J. D.; Mentzer, R. M. (August 1991). "Neonatal pneumonectomy for isolated unilateral pulmonary artery agenesis". The Annals of Thoracic Surgery. 52 (2): 294–295. doi:10.1016/0003-4975(91)91356-z. ISSN 0003-4975. PMID 1863153.
^Fraentzel, O. (May 1868). "Ein Fall von abnormer Communication der Aorta mit der Arteria pulmonalis". Archiv für Pathologische Anatomie und Physiologie und für Klinische Medicin (in German). 43 (3): 420–426. doi:10.1007/BF01915623. ISSN 0945-6317.
^Jan Ten Harkel, A. Derk; Blom, Nico A.; Ottenkamp, Jaap (2002-10-01). "Isolated Unilateral Absence of a Pulmonary Artery: A Case Report and Review of the Literature". Chest. 122 (4): 1471–1477. doi:10.1378/chest.122.4.1471. ISSN 0012-3692. PMID 12377882.
^ abKruzliak, Peter; Syamasundar, Rao P.; Novak, Miroslav; Pechanova, Olga; Kovacova, Gabriela (2013-08-01). "Unilateral absence of pulmonary artery: Pathophysiology, symptoms, diagnosis and current treatment". Archives of Cardiovascular Diseases. 106 (8): 448–454. doi:10.1016/j.acvd.2013.05.004. ISSN 1875-2136. PMID 23938302.
^Darwazah, Ahmad K.; Alhaddad, Imad A. (2016-07-16). "Pulmonary artery agenesis associated with coronary collaterals among adults". Journal of Cardiothoracic Surgery. 11 (1): 109. doi:10.1186/s13019-016-0504-1. ISSN 1749-8090. PMC 4947334. PMID 27422770.
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