The Misrouted Vessel: Two Different Presentations of Anomalous Right Pulmonary Artery

Anomalous origin of pulmonary arteries from the aorta is very rare, the incidence being about 0.1%. Either the right or left pulmonary artery can arise directly from the ascending aorta, while the other pulmonary artery retains its origin from the right ventricular outflow tract. Most of these cases are diagnosed during infancy, and only 5% of cases are reported in adults. It is usually associated with other congenital cardiac anomalies like PDA (most common, seen in two-thirds of cases), aortopulmonary septal defect, tetralogy of Fallot, hypoplastic aortic arch, patent foramen ovale, and ventricular septal defect.Our aim is to discuss two rare cases of AORPA and their associated anomalies with different presentations, and how collaterals change the course of the disease.The first case in this report is of an 8-month-old female with unremarkable birth history who presented with cough and dyspnoea since 2 months of age. She kept on deteriorating and was diagnosed to have severe TR, PAH, and patent foramen ovale on 2D echo. Further evaluation with CT showed cardiomegaly with dilated right atrium and ventricle, patent foramen ovale, and anomalous origin of the right pulmonary artery from the aorta. The patient was taken up for AORPA repair (ICR) but later succumbed due to low cardiac output syndrome.The second case in this report is of a 10-year-old female with unremarkable birth history who presented with intermittent episodes of SOB since 1 month of age and was on medical management. Further evaluation with CT showed a large PDA and anomalous origin of the right pulmonary artery from the aorta. The patient was taken for AORPA repair (ICR) and PDA ligation, and post-surgery, the patient was stable and resumed normal activities.These cases underscore the critical importance of cross-sectional imaging in surgical planning and aid the surgeon in devising an effective surgical approach. Moreover, they highlight how the presence or absence of PDA can significantly influence the clinical course—while the first infant, lacking a PDA, developed early cardiac failure, the second child survived up to 10 years without heart failure due to the presence of a large PDA providing a compensatory pathway. Thus, imaging helps in assessing potential prognostic outcomes and long-term management of the patient.