Anomalous aortic origin of the pulmonary arteries: Case series and literature review

Abstract

Anomalous origin of the pulmonary arteries from the ascending aorta is a rare, but severe clinical entity necessitating a scrupulous evaluation. Either the right or the left pulmonary arteries can arise directly from the ascending aorta while the other pulmonary artery retains its origin from the right ventricular outflow tract. Such a finding can be isolated or can coexist with several congenital heart lesions. Direct intrapericardial aortic origin, however, must be distinguished with origin through a persistently patent arterial duct. In the current era, clinical manifestations usually become evident in the newborn rather than during infancy, as used to be the case. They include respiratory distress or congestive heart failure due to increased pulmonary flow and poor feeding. The rate of survival has now increased due to early diagnosis and prompt surgical repair, should now be expected to be at least 95%. We have treated four neonates with this lesion over the past 7 years, all of whom survived surgical repair. Right ventricular systolic pressure was significantly decreased at follow-up. Our choice of treatment was to translocate the anomalous pulmonary artery in end-to-side fashion to the pulmonary trunk. Our aim in this report is to update an Italian experience in the diagnosis and treatment of anomalous direct origin of one pulmonary artery from the aorta, adding considerations on the lessons learned from our most recent review of the salient literature.

Anomalous right pulmonary artery from aorta, surgical approach case report and literature review

“…Unequal partitioning of the "conotruncus" by eccentric coalescence of the right and left outflow tract ridges can similarly be discounted since the outflow cushions separate the intermediate and proximal parts of the developing outflow tract. 4 Lesions such as aortopulmonary window, or an anomalous origin of the right pulmonary artery from the aorta, are intrapericardial.…”

“…The term Hemitruncus is considered inadequate as per professor Anderson et al 3 because unlike—truncus arteriosus—two arterial valves aortic and pulmonary are present. About 300 cases have been reported in the literature, most of them in relation to surgical correction 4 . Choosing the appropriate technique for repair depends on the site of origin of the anomalous pulmonary artery from aorta.…”

<p>Hemitruncus Arteriosus in a 10-Day-Old Neonate with Patent Ductus Arteriosus and Thrombocytopenia</p>

“…In the disease, the pulmonary pressure increases with time and the mortality rate varies from 30% to 70% depending on case severity and receiving cardiac surgery as soon as possible. [1][2][3][4][5][6][7][8] Early surgical repair can prevent pulmonary vascular disease progression. 4 The surgical repair should be done within the first six months to prevent severe pulmonary vasculature disease.…”

Anomalous Aortic Origin of the Right Pulmonary Artery From the Ascending Aorta With Spontaneous Dissection and Thrombosis

“…2 It can occur in isolation or in association with other anomalies. 3 It affects more commonly the RPA. The AAOBPA is usually intrapericardial, which should differentiate this from other sources of pulmonary blood flow such as ductal origin of the pulmonary artery or major aortopulmonary collateral artery.…”

“…It is the unequal separation of the aortic sac itself that may provide a better explanation to this anomalous pulmonary artery branch origin. 8 In a recent review, 3 299 patients were reported with AAOBPA from 1962 to 2017, with repair performed in 73% of them. None of these cases reported any acute event in the anomalous artery such as in the current case where a dissection flap and thrombi were identified in the RPA, which explains the inability to identify the artery on preoperative CTA scan or echocardiogram due to near absence of forward flow from the aorta.…”