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Figure 1. Co-occurrence of IAA with APW [ 4 ]. Permission to reproduce granted by the original author.
department( ED) at OSH on DOL 14 with chief complaint of shortness of breath. The patient was described as having rapid breathing and mild grunting starting two days prior to the ED visit, with low oxygen saturation. There was a differential between upper and lower extremity blood pressures and saturations. Chest X-ray revealed a mildly enlarged cardiac silhouette and increased pulmonary markings. These clinical and diagnostic findings indicated pulmonary overcirculation and potential for distal systemic malperfusion. The patient was started on prostaglandins( PGE) and transferred to our center with concern for undiagnosed congenital heart disease.
Upon arrival, an echocardiogram was performed, and the patient was diagnosed with type A interrupted aortic arch( IAA) and a large type 1 aortopulmonary window( APW). Additional findings were a large patent ductus arteriosus
( PDA), a dilated left atrium( LA), an intact ventricular septum, and a small superior secundum ASD. The patient was admitted to the cardiac intensive care unit( CICU) and remained on PGE due to ductal-dependent systemic blood flow and supported with high flow nasal cannula( HFNC) therapy due to respiratory distress secondary to pulmonary overcirculation. A CTA was obtained which confirmed the diagnosis and revealed a large APW that nearly reached the transverse aortic arch towards the takeoff of the left subclavian artery( Figure 2A). Of note, the coronary arteries displayed origins above the level of the aortic valve commissural posts( Figure 2B). In addition, the distance of interruption appeared to be quite significant-- nearly 2 cm between the ascending aorta and the native descending aortic tissue( Figure 2C). Surgical intervention was undertaken on DOL 19.