Year: 2024
Type: article
Abstract: Video clip is available online.Pectus excavatum accounts for 90% of all chest wall deformities, occurring in 1 in every 300 to 1000 live births.1,2 Although usually sporadic, it has also been associated with a variety of conditions, most commonly Marfan syndrome, Ehlers-Danlos syndrome, osteogenesis imperfecta, Noonan syndrome, Turner syndrome, neurofibromatosis type 1, Poland syndrome, and homocystinuria.2 Unilateral absence of a pulmonary artery (UAPA) is an uncommon condition with an estimated prevalence of 1 in 200,000 to 1 in 300,000 young adults.However, the exact prevalence of UAPA in children is unknown, as many patients remain asymptomatic for a long period of time.3 UAPA is often associated with other cardiac abnormalities, most commonly tetralogy of Fallot, atrial septal defect, coarctation of the aorta, patent ductus arteriosus, right aortic arch, truncus arteriosus, and pulmonary atresia.4 Less frequently, UAPA can occur as an isolated lesion, with almost two-thirds of these cases involving the right pulmonary artery.5 Thus, congenital absence of the left pulmonary artery is extremely rare.
Source: JTCVS Techniques
Language: English
Subfield: Surgery
Field: Medicine
Domain: Health Sciences
Open Access status: gold
Estimated APC paid: $2,431
OpenAlex ID: W4401821262