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Association of interrupted aortic arch with aortic atresia as a variant of hypoplastic left heart syndrome (HLHS) is extremely unusual and may never be appropriate for life without a reliable origin for cerebral and coronary circulation. The in-patient with a sizable collateral artery involving the descending aorta in addition to right subclavian artery is presented.An isolated bronchial isomerism without associated malformations is well known becoming really rare and it has