Origin of the left pulmonary artery from the aorta: embryologic considerations

Aru, Giorgio M.; English, William P.; Gaymes, Charles H.; Heath, Bobby J.

doi:10.1016/s0003-4975(00)02356-0

Cited by 32 publications

(23 citation statements)

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“…1 By contrast, our case study patients presented with a left arching aorta. 1,6,8 A frequent association of AORPA is the CATCH 22 syndrome, 7,9-14 which was not found in one of our cases, based on the absence of the 22q11 deletion anomaly following a fluorescent immuno-sorbent hybridisation (FISH) chromosomal analysis. The other patient was not tested for this chromosomal aberration.…”

Section: Discussionmentioning

confidence: 69%

“…15 The pathogenesis of an anomalous pulmonary artery originating from the aorta is said to be related to an abnormality in the development of the aortic or branchial arches. 1,6 The failure of resorption of the fifth branchial arch and presence of the sixth brachial arch usually lead to the anomalous origin of the right pulmonary artery from the aorta. 1,6 By contrast, the anomalous origin of the left pulmonary artery from the aorta is reported to be due to developmental absence of the fifth and sixth branchial arches.…”

Section: Discussionmentioning

confidence: 99%

“…1,6 The failure of resorption of the fifth branchial arch and presence of the sixth brachial arch usually lead to the anomalous origin of the right pulmonary artery from the aorta. 1,6 By contrast, the anomalous origin of the left pulmonary artery from the aorta is reported to be due to developmental absence of the fifth and sixth branchial arches. Abnormalities of the branchial or aortic arches are associated with neural crest-cell aberrations and therefore may be associated with the CATCH 22 syndrome.…”

Section: Discussionmentioning

confidence: 99%

“…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.…”

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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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Section: Discussionmentioning

confidence: 69%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 99%

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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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“…Edasery and colleagues proposed that an origin of the RPA from the aorta was a consequence of asymmetric division of the truncus arteriosus, resulting in the right sixth arch on the aortic side [49]. Other proposed theories include incomplete leftward migration of the RPA during its development [50] or failure of fusion of one of the sixth arches with the MPA, resulting in persistence of the aortic sac from which the sixth arches originate [51]. An association with chromosome 22q11 deletion has been suggested [52].…”

Section: Aortic Origin Of a Branch Pulmonary Arterymentioning

confidence: 99%

Abnormalities of the Ductus Arteriosus and Pulmonary Arteries

Tacy

2009

Echocardiography in Pediatric and Congenital Heart Disease

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The ductus arteriosus is a normal vascular structure in mammalian embryos that connects the main pulmonary artery (MPA) with the descending aorta or a subclavian artery. In left aortic arch the ductus arteriosus inserts into the proximal descending aorta at its junction with the aortic isthmus, distal to the origin of the left subclavian artery. In right aortic arch the ductus arteriosus can connect the right descending aorta with the MPA (right-sided ductus arteriosus), the proximal left subclavian artery with the MPA (left-sided ductus arteriosus), or both (bilateral ductus arteriosus). In the fetus, the ductus arteriosus carries blood from the right ventricle (RV) to the aorta, bypassing the high-resistance pulmonary vascular bed. Normally, the ductus arteriosus closes during the first day of life. Persistent ductal blood flow beyond the newborn period is termed patent ductus arteriosus (PDA). IncidenceThe incidence of PDA varies considerably depending on the postnatal age of subjects and their degree of prematurity. PDA is common in preterm infants and is considered a functional abnormality as opposed to a structural congenital cardiac anomaly. In term infants the normal ductus arteriosus may stay open for several days after birth. Echo-Doppler studies in healthy newborns have shown functional closure of the ductus arteriosus in almost all subjects by 4-7 days after birth [1,2]. Based on analysis of 40 published articles spanning several decades, Hoffman and Kaplan estimated the median incidence of PDA in term infants at 56.7 per 100 000 live births [3]. EtiologyThe etiology of isolated PDA in full-term infants older than 1 week is not known. Recent investigations have identified the genes affecting the development of the fourth and sixth pharyngeal arches, and noted their association with cardiovascular defects similar to those observed in DiGeorge syndrome [4]. Morphology Developmental considerationsThe normal anatomy and congenital malformations of the ductus arteriosus and branch pulmonary arteries are rooted in the development of the embryonic sixth aortic arches (see Chapter 30). The embryo has two dorsal aortas, which communicate with the ventrally located aortic sac via several pairs of branchial arch arteries. During development, there is an orderly appearance and regression of six pairs of primitive aortic arches. The fate of the fourth and sixth arches is most important to the topics covered in this chapter. The left fourth arch persists as the distal left aortic arch between the left common carotid and left subclavian arteries, and the left dorsal aorta persists to become the segment that extends between the left subclavian artery and the site of the entrance of the ductus arteriosus. The right fourth arch becomes the brachiocephalic trunk and proximal right subclavian artery, whereas the right dorsal aorta largely involutes. The proximal portions of the right and left sixth arches form the proximal right and left pulmonary arteries, respectively. The distal right sixth arch regresses, whereas the...

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2003

The Natural and Modifed History of Congenital Heart Disease

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Origin of the left pulmonary artery from the aorta: embryologic considerations

Cited by 32 publications

References 4 publications

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 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

Abnormalities of the Ductus Arteriosus and Pulmonary Arteries

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