Anomalous Origin of One Pulmonary Artery from the Ascending Aorta

Keane, John F.; Maltz, David L.; Bernhard, William F.; Corwin, Robert D.; Nadas, Alexander S.

doi:10.1161/01.cir.50.3.588

Cited by 75 publications

(39 citation statements)

References 23 publications

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“…Clinically, these patients often present with progressive respiratory distress and evidence of congestive heart failure There is often a degree of cyanosis from the right to left shunting through the PFO of PDA and secondary to elevated end-diastolic pressure in the hypertensive, noncompliant right ventricle from increased pulmonary resistance [7,16]. Almost all patients had systemic or suprasystemic right ventricular pressure in our study.…”

Section: Discussionmentioning

confidence: 69%

“…The full quantity output flows to the single lung attached to the right ventricle, and the other lung is exposed to unrestricted aortic blood flow and pressure. If this abnormal circulation is allowed to continue uncorrected, early development of pulmonary vascular obstructive disease can be expected [7,16,24].…”

Section: Discussionmentioning

confidence: 99%

“…If not corrected early, progressive pathologic changes occur in the lungs with bilateral pulmonary hypertension which can progress to cause severe pulmonary vascular obstructive disease [16]. Surgical treatment is recommended as soon as a diagnosis is confirmed.…”

Section: Introductionmentioning

confidence: 99%

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Surgical Results of Anomalous Origin of One Pulmonary Artery Branch from the Ascending Aorta

et al. 2015

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We reviewed our surgical experience with anomalous origin of one pulmonary artery from the ascending aorta (AOPA). From 1989 to 2012, 12 children (five neonates) aged 3-734 days (mean 152 ± 222) with AOPA underwent operations. Eight patients had right AOPA, and four patients had left AOPA. The majority of the patients had elevated right ventricular pressure, with 58 % (7 of 12) demonstrating suprasystemic right ventricular pressure. Surgery was performed by direct anastomosis (group 1) in seven patients and by employing an autologous patch (group 2) in five patients. There were two postoperative mortalities caused by heart failure and pulmonary hypertensive crisis. The mean follow-up duration was 12.6 ± 8 years. Catheterization showed that the right ventricle-to-systemic pressure ratio decreased following operation (preoperative vs. postoperative; 1.13 ± 0.19 vs. 0.48 ± 0.03, p = 0.043). There was no difference in the perfusion of the affected lung as measured by the final lung perfusion scan, between the two groups (group 1 vs. group 2; 50.0 ± 10.3 vs. 42.7 ± 28.7 %, p = 0.158). Two patients required reoperations for pulmonary regurgitation and pulmonary artery stenosis. There were two catheter-based interventions. At 20 years, survival by the Kaplan-Meier was 91.7 ± 8.0 %, freedom from reoperation was 80.0 ± 17.9 %, and freedom from catheter intervention was 80.8 ± 12.2 %. Early repair of AOPA improves right ventricular pressure and overall hemodynamics with excellent survival and low risk of reintervention. The type of surgical repair did not significantly affect the long-term outcomes (measured via lung perfusion scan).

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Section: Discussionmentioning

confidence: 69%

Section: Discussionmentioning

confidence: 99%

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Surgical Results of Anomalous Origin of One Pulmonary Artery Branch from the Ascending Aorta

et al. 2015

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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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“…Keane et al . [5] in their review found that histologically there was no significant pulmonary vascular obstruction in the majority of the 18 infants who died ; while significant changes, mainly in the systemic lung, were observed in six of the ten older children . Griffiths et al .…”

Section: Discussionmentioning

confidence: 97%

Anomalous origin of right pulmonary artery from ascending aorta with right ventricular endocardial fibroelastosis

et al. 1985

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Anomalous Origin of One Pulmonary Artery from the Ascending Aorta

Cited by 75 publications

References 23 publications

Surgical Results of Anomalous Origin of One Pulmonary Artery Branch from the Ascending Aorta

Surgical Results of Anomalous Origin of One Pulmonary Artery Branch from the Ascending Aorta

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 right pulmonary artery from ascending aorta with right ventricular endocardial fibroelastosis

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