Anomalous origin of the left pulmonary artery from the aorta. Our experience and literature review

Abstract

Three patients with anomalous origin of the left pulmonary artery (AOLPA) from the aorta underwent surgical correction. Two patients presented with an isolated malformation and one with an associated ventricular septal defect. One of the patients with an isolated malformation presented with an AOLPA from the descending thoracic aorta. Implantation of the AOLPA to the main PA trunk was performed by direct anastomosis in two patients and by interposition of a synthetic graft in the other patient with AOLPA from the descending aorta. All patients survived the operation. Low cardiac output was identified in one patient. The mean residual gradient through the anastomotic site was 21 mmHg at follow-up. We found almost 72 cases with AOLPA reported in the literature with a high mortality rate in patients not undergoing surgery. The most frequently found associated malformation is tetralogy of Fallot and ventricular septal defect. AOLPA from the aorta is a rare but important entity, necessitating a scrupulous preoperative and intraoperative evaluation. Patients with AOLPA can undergo surgical repair with excellent results.

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

“…7 In this patient, there were welldeveloped central pulmonary arteries and the patient underwent successful complete surgical repair. The aetiology of AOPA is unclear, but one case of AORPA has been reported to be associated with foetal valproate syndrome.…”

“…Abnormalities of the branchial or aortic arches are associated with neural crest-cell aberrations and therefore may be associated with the CATCH 22 syndrome. 7,[9][10][11][12][13][14] Precursors of the trunco-aortic sac are mainly of mesodermal origin, 16 therefore AOPA is unlikely to be associated with the trunco-aortic sac abnormalities. 1 The origin of AOPA anatomically is usually very proximal, arising from the ascending aorta just distal to the aortic sinus.…”

“…1 The origin of AOPA anatomically is usually very proximal, arising from the ascending aorta just distal to the aortic sinus. 1,2,7,17 Other origins of the pulmonary arteries, such as at the base of the innominate artery, from a patent ductus arteriosus, or from the thoracic aorta distal to the subclavian artery have been described and can be labelled as 'distal types of AOPA'. They are not the same entity that is described in this case study.…”

“…20 The mechanism by which both lungs are affected involves neurogenic crossover from the involved lung to affect the protected lung, thereby producing reflex vasoconstriction in the protected lung. 3,7,20 Diastolic run-off due to shunting of blood away from the aorta into the aberrant pulmonary artery may affect coronary perfusion and result in left ventricular dysfunction. 14 The diagnosis of AOPA can be easily achieved by careful echocardiography.…”

“…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.…”