Anomalous origin of a pulmonary artery from the ascending aorta: surgical repair resolving pulmonary arterial hypertension.
OBJECTIVE
To emphasize the diagnostic possibility of the anomalous origin of one pulmonary artery from the ascending aorta in infants with clinically refractory heart failure and no intracardiac structural defect.


METHODS
Retrospective study of 4 infants with refractory heart failure undergoing 2-dimensional echocardiographic study with subcostal, suprasternal, and parasternal views, and hemodynamic and angiocardiographic study in the anteroposterior projection.


RESULTS
Three of the 4 infants had their right pulmonary artery originating from the ascending aorta as their major diagnosis. In the fourth patient, the left pulmonary artery originated from the ascending aorta in association with a large interventricular septal defect. The pressure level in both pulmonary arteries in all infants was that of the systemic level. All patients underwent surgery, which consisted of translocation of the anomalous pulmonary artery from the aorta. Neither immediate nor late cardiac deaths occurred.


CONCLUSIONS
Once the diagnosis of anomalous origin of the pulmonary artery from the ascending aorta in the isolated form is established, the surgical correction should be immediately performed, not only because of the risk of developing pulmonary vascular disease, but also because of the excellent surgical results currently obtained.
Santos
Marco Aurélio
MA
Instituto Nacional de Cardiologia Laranjeiras, Rio de Janeiro, RJ, Brazil. vitoraze@cardiol.com
Azevedo
Vitor Manuel Pereira
VM
D001011:Aorta / Q000002:abnormalities* / Q000601:surgery; D002303:Cardiac Output, Low / Q000175:diagnosis / Q000209:etiology / Q000601:surgery; D005260:Female; D006330:Heart Defects, Congenital / Q000175:diagnosis / Q000601:surgery*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000209:etiology / Q000601:surgery*; D007223:Infant; D008173:Lung Diseases, Obstructive / Q000175:diagnosis / Q000601:surgery; D008297:Male; D011651:Pulmonary Artery / Q000002:abnormalities* / Q000601:surgery; D012189:Retrospective Studies