Origin of the Right Pulmonary Artery from the Ascending Aorta

Fontana, Gregory P.; Spach, Madison S.; Effmann, Eric L.; Sabiston, David C.

doi:10.1097/00000658-198707000-00016

Cited by 70 publications

(77 citation statements)

References 56 publications

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“…Most commonly, the surgical correction was achieved within the first month of age, 3,4,8,13,15,20 although successful repair has been reported in adults. 16 Fontana et al 28 found a 30% 1-year survival in a series of 23 cases with anomalous origin of one pulmonary artery from the aorta which was not treated surgically. Successful correction of this anomaly in the first days of life, even in premature infants, has been reported.…”

Section: Discussionmentioning

confidence: 98%

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

Prifti¹,

Bonacchi²,

Murzi³

et al. 2003

Heart and Vessels

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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.

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Section: Discussionmentioning

confidence: 98%

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

Prifti¹,

Bonacchi²,

Murzi³

et al. 2003

Heart and Vessels

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“…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.…”

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confidence: 99%

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

Pepeta

Takawira

Cilliers

et al. 2011

CardioVascular Journal of Africa

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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...

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“…2 It involves more frequently the right than the left pulmonary artery. 1 Early development of pulmonary hypertension and irreversible pulmonary vascular changes occur inevitably. 2 In patients with AOPA, the affected lung is exposed to unrestricted aortic flow and systemic pressure, while the contralateral lung receives the entire right ventricular output in addition to flow contributed by associated anomalies, such as PDA, ventricular septal defect, and ASD.…”

Section: Discussionmentioning

confidence: 99%

“…Embryologically, anomalous origin of the right pulmonary artery from the proximal posterior aspect of the ascending aorta is due to the incomplete leftward migration of the right sixth aortic arch. 1 Early diagnosis is essential, so that early surgical repair can be performed to improve outcome. 1 Early development of pulmonary hypertension and irreversible pulmonary vascular changes occur inevitably.…”

Section: Discussionmentioning

confidence: 99%

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Anomalous origin of one pulmonary artery from the ascending aorta (hemitruncus) in a premature infant: A case report and literature review

Ren

Song

et al. 2013

J of Clinical Ultrasound

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Origin of the Right Pulmonary Artery from the Ascending Aorta

Cited by 70 publications

References 56 publications

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

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

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

Anomalous origin of one pulmonary artery from the ascending aorta (hemitruncus) in a premature infant: A case report and literature review

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