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The magnetic resonance angiography (MRA) images of a 19 year old girl with persistent truncus arteriosus are shown. Clinically, she has pulmonary vascular disease. Her chest x ray (upper panel, middle column) failed to demonstrate a right pulmonary artery. MRA was performed to define the pulmonary anatomy better, as a non-invasive modality in this patient.
Orthogonal black blood studies of the heart and great vessels and 4CV FIESTA images were obtained, followed by gadolinium enhanced aortopulmonary arterial MRA.
The left pulmonary artery (lower panel, middle column) was seen to arise posterolaterally from the left side of the trunk, 3 cm above the truncal valve, dividing into upper and lower lobe branches as it entered the left lung. The absent right pulmonary artery was confirmed in the study.
However, there were two large aortopulmonary collateral vessels seen arising from the mid thoracic aorta, crossing in a tortuous turn to supply the right lung (upper and lower panels, right column). The upper vessel supplied the right upper lobe, while the lower vessel provided flow to the middle and lower lobes. The aortic arch was right sided.
The origin of pulmonary arteries in persistent truncus arteriosus is highly variable. Absence of either pulmonary artery branch in this condition has been reported only rarely. In those few cases, the pulmonary artery was absent on the side of the aortic arch, as in our patient.
MRA is an excellent and non-invasive way of delineating the cardiac anatomy in structural heart disease.
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