Interruption of the Ascending Aorta: A Hitherto Undescribed Lesion

We are describing a most unusual variant of aortic arch interruption. The ascending aorta was atretic above the sinutubular junction forming a blind-ending pouch. The coronary arteries were the only vessels arising from the aortic trunk. All the brachiocephalic vessels were perfused in retrograde fashion through the arterial duct. The aortic valve itself was dysplastic, stenotic, and permitted severe regurgitation. There was no subaortic obstruction. A modified first stage Norwood palliation was performed. Due to myocardial insufficiency transthoracic extracorporeal membrane oxygenation had to be commenced at the end of surgery. Unfortunately the child died due to severe intracranial hemorrhage.