Interrupted aortic arch (IAA) is an extremely rare CHD defined as the loss of luminal continuity between the ascending and descending aorta. Its clinical presentation, including cardiovascular shock, acidemia, or severe CHF in the first 2 weeks of life, is similar to severe coarctation of the aorta, although the developmental mechanisms involved in their generation seem to be different. IAA is characterized by a narrowed left ventricular OFT, with resulting severe obstruction associated with decreased growth, hypoplasia, and interruption of the aortic arch. In addition to VSD and narrowed subaortic area, there is complete atresia of a segment of the aortic arch. This anomaly entails a very poor prognosis without prompt surgical treatment.