Pulmonary interstitial emphysema is a clinical state occurring in newborns and infants that involves lung damage with air leak into the pulmonary interstitium. The data about this condition among newborns with critical heart defects are limited. We report a case of an infant with complex heart disease, 22q11.2 microdeletion, and TNNC1 gene mutation presenting with pulmonary interstitial emphysema. The infant was intubated for respiratory failure and underwent pulmonary artery banding. Weaning from mechanical ventilation was complicated with pulmonary interstitial emphysema and pneumothorax development. Pulmonary interstitial emphysema was fully resolved after lateral decubitus positioning on the affected side down. Complete repair of the common arterial trunk was accomplished at the age of 2 months with no signs of residual pulmonary interstitial emphysema. Lateral decubitus positioning may be effective as a non-invasive treatment in an infant with pulmonary interstitial emphysema and complex heart disease.

肺间质性肺气肿是一种发生在新生儿和婴儿中的临床状态，涉及肺损伤并导致空气漏入肺间质。有关患有严重心脏缺陷的新生儿的这种情况的数据有限。我们报告了一例患有复杂心脏病、22q11.2 微缺失和 TNNC1 基因突变并表现为肺间质性肺气肿的婴儿病例。该婴儿因呼吸衰竭而接受插管，并接受肺动脉结扎术。机械通气撤机后并发肺间质性肺气肿和气胸。患侧向下侧卧位后，肺间质性气肿完全消退。 2个月大时，总动脉干完全修复，没有残留肺间质性肺气肿的迹象。对于患有肺间质性肺气肿和复杂心脏病的婴儿，侧卧位可能是一种有效的非侵入性治疗方法。