Partial Anomalous Left Pulmonary Artery

Abstract

A 4-year-old girl with Kabuki syndrome was evaluated and diagnosed with an atrial septal defect on the basis of an abnormal cardiac physical examination. A chest radiograph was performed, which demonstrated multiple skeletal abnormalities and mild prominence of the pulmonary vasculature consistent with an atrial septal defect (Figure 1). A complete echocardiogram was performed under sedation, which demonstrated the atrial septal defect as well as dual pulmonary arterial supply to the left lung with a partial anomalous left pulmonary artery (LPA) from the right pulmonary artery (RPA) (Figure 2). Cardiac magnetic resonance (MR) imaging was performed to further delineate pulmonary arterial anatomy as well as possible tracheal compression from the partial anomalous LPA. Figure 1. Chest radiograph in the anteroposterior projection demonstrates irregularity of the right clavicle with separation of the hypoplastic distal half (arrowhead), butterfly vertebrae of T11 (arrow), absence of the left twelfth rib (asterisk), mild prominence of the pulmonary vasculature, and a normal cardiac silhouette. Figure 2. High parasternal short-axis echocardiogram with color compare shows the partial anomalous LPA (ALPA) arising from the RPA. AAo indicates ascending aorta; MPA, main pulmonary artery; and DRPA, distal RPA. A complete cardiac MR study was performed including gadolinium-enhanced MR angiography. The 3-dimensional reconstructed MR angiography data set demonstrates that the left lung receives blood supply from 2 left pulmonary arteries. One of …