Anomalous origin of the left pulmonary artery from the ascending aorta in two children with pulmonary atresia, subaortic ventricular septal defect and right-sided major aorto-pulmonary collateral arteries

Abstract

Anomalous origin of the left pulmonary artery from the ascending aorta in two children with pulmonary atresia, sub-aortic ventricular septal defect and right-sided major aorto-pulmonary collateral arteries L PEPETA, FF TAKAWIRA, PE ADAMS, NH NTSINJANA, BJ MITCHELL, AM CILLIERS AbstractWe report two rare cases of an anomalous origin of the left pulmonary artery (AOLPA) from the ascending aorta, associated with pulmonary atresia, a ventricular septal defect and a left aortic arch. The cases are unusual because AOLPA is more commonly associated with a right aortic arch and it is more usual for the right pulmonary artery to originate anomalously from the ascending aorta. The pulmonary blood supply to the right lung in both patients was absent and provided instead by major aorto-pulmonary collateral arteries which were stenosed at multiple levels. The AOLPA in both patients originated from the postero-lateral aspect of the ascending aorta just distal to the sino-tubular junction. Only one patient showed the more common association of an unusual aortic arch branching pattern in the form of an anomalous right subclavian artery.Neither patient was in heart failure and the chest X-ray in both revealed differential pulmonary perfusion with prominent vascularity of the left lung. Cardiac catheterisation showed systemic pressures within the anomalous left pulmonary artery. Karyotyping revealed normal chromosomes, and fluorescent in-situ hybridisation done in one patient was negative for chromosome 22q11.2 microdeletion. Both patients have been managed conservatively.Keywords: pulmonary atresia with ventricular septal defect, anomalous origin of pulmonary artery, collateral arteries, pulmonary hypertension, branchial arches, CATCH22 syndrome An anomalous origin of the pulmonary artery from the aorta (AOPA) is very rare, with the first case of this nature reported by Fraentzel in 1868. 1 The incidence of anomalous left pulmonary artery from the aorta (AOLPA) was reported in one study to be around 17%, 1 with anomalous origin of the right pulmonary artery (AORPA) from the ascending aorta as the more common variant of AOPA, seen in about 83% of cases. Without surgery, the mortality in patients with AOPA is extremely high, reaching as high as 80%. [2][3][4][5] This is due to severe, rapidly progressive pulmonary vascular disease and congestive cardiac failure. Both isolated AOLPA and that associated with other defects have been well described. 1,6,7 The commonest associated congenital cardiac lesion is tetralogy of Fallot, which is seen in about 75% of cases. 1 AOLPA is also associated with a right arch of the aorta in 63% of cases, and in about 38% of cases, an anomalous origin of the right subclavian artery from the descending aorta has been reported as well. 1 Of note, both our cases had a left aortic arch and had no main and right pulmonary arteries, with the only source of blood supply to the right lung being partially stenosed major aorto-pulmonary collateral arteries (MAPCAs).To the best of our knowledge, this series repr...

Development of the human aortic arch system captured in an interactive three‐dimensional reference model

“…However, the central pulmonary arteries have been reported to originate from the ascending aorta, which is one of the aortic sac derivatives [Kutsche and van Mierop, ]. Furthermore, there are forms of pulmonary atresia associated with the absence of the pulmonary trunk and its bifurcation, in which the distal pulmonary artery branches are still present [Pepeta et al, ], or an isolated right subclavian artery connected to the bifurcation of the pulmonary trunk [Miller et al, ]. These observations suggest that the region encompassing the pulmonary trunk and its bifurcation represents another derivative of the aortic sac, rather than being part of the sixth PAAs.…”

Surgical Correction of Anomalous Origin of One Pulmonary Artery Without Grafts in Infants

“…Early repair is preferred to avoid pulmonary hypertension, congestive heart failure, and irreversible pulmonary vascular disease. Therefore, surgical treatment is recommended as soon as the diagnosis is confirmed during the neonatal and infant period . Most repairs require the use of an artificial or homologous conduit due to inadequate vasculature length for the connection between the aberrant pulmonary artery (PA) and the main pulmonary artery (MPA).…”

“…Therefore, surgical treatment is recommended as soon as the diagnosis is confirmed during the neonatal and infant period. 7,8 Most repairs require the use of an artificial or homologous conduit due to inadequate vasculature length for the connection between the aberrant pulmonary artery (PA) and the main pulmonary artery (MPA). However, complications involving these grafts include anastomotic stenosis and age-dependent replacement of the conduits during the follow-up period.…”

Left Pulmonary Artery from the Ascending Aorta: A Case Report and Review of Published Cases

“…Of these cases, 9 also had so-called "absence" of the leaflets of the pulmonary valve [26,38,44,51,54,60,61]. When "pulmonary atresia with ventricular septal defect" is considered as a separate entity, a further 6 (5.3%) of the 113 cases exhibited this finding, although these were likely also examples of tetralogy of Fallot [17,[66][67][68]. Double outlet right ventricle was noted in 3 (cases 2.7%) [69][70][71].…”

“…Patency of the arterial duct was present in 16 (14.2%), with the duct being right-sided in 14 (87.5%) of these [3,17,24,30,33,60,69,[79][80][81][82][83][84][85]. Of the 113 cases with sufficient anatomic details, an aberrant left subclavian artery was reported in 6 cases, and an aberrant right subclavian artery in 2 cases [3,24,33,68]. The only genetic condition reported in these cases was 22q11.1 deletion which was documented in 8 (7.1%) of cases [2,41,46,59,85,86].…”