Aortopulmonary Window and Interrupted Aortic Arch With Eisenmenger Syndrome in an Adult

A 33-year-old woman presented to our department with complaints of bluish discoloration of the body, thickening of the nail beds, and dyspnea on exertion for many years. She had history of repeated respiratory infections during infancy and early childhood. Her physical examination revealed cyanosis and clubbing with a pulse rate of 90 bpm, blood pressure of 128/80 mm Hg, and left parasternal heave. Auscultation demonstrated a loud pulmonary component of the second heart sound and a short ejection systolic murmur over the second left intercostal space. Her arterial oxygen saturation was 82% at room air in all 4 limbs. Her chest radiograph showed a cardiothoracic ratio of 55% with a prominent main pulmonary artery segment (Figure 1A). Two-dimensional transthoracic echocardiography (TTE) revealed a large defect between the ascending aorta and the main pulmonary trunk, suggesting an aortopulmonary window (APW) (Figure 1B and 1C and Movies I and II in the online-only Data Supplement). Color Doppler showed bidirectional flow across this APW (Figure 1D and Movie III in the online-only Data Supplement). The entire aortic arch could not be visualized in the suprasternal view. Three-dimensional (3D) TTE further delineated the spatial geometry of the APW and its proximity to the 2 semilunar …