Hainstock Michael, Bardo Dianna, Rice Mary, Langley Stephen
Department of Pediatric Cardiology, Oregon Health & Science University, Portland, OR, USA.
Pediatr Cardiol. 2010 Aug;31(6):878-80. doi: 10.1007/s00246-010-9716-7. Epub 2010 May 19.
We present a patient with the combination of hypoplastic left heart syndrome (HLHS), large omphalocele, and hypoplastic left bronchus who underwent a modified Norwood procedure and was successfully discharged home. The child required tracheostomy with home ventilation and we elected to delay the primary repair of the omphalocele. To our knowledge, this is the first reported case with this combination of anomalies surviving till hospital discharge. Our experience demonstrates the potential viability of patients with concurrent HLHS, large abdominal wall defects, and airway anomalies and may additionally advocate for delayed repair of large omphaloceles in patients with complex congenital heart disease.
我们报告了一名患有左心发育不全综合征（HLHS）、巨大脐膨出和左支气管发育不全的患者，该患者接受了改良诺伍德手术并成功出院回家。患儿需要气管切开并在家中进行通气，我们选择延迟脐膨出的一期修复。据我们所知，这是首例报道的具有这种异常组合且存活至出院的病例。我们的经验证明了同时患有HLHS、巨大腹壁缺损和气道异常的患者具有潜在的生存能力，并且可能还主张对患有复杂先天性心脏病的患者延迟修复巨大脐膨出。
