Shamim Adeel Ahmed, Khan Mubashir Zareen, Atiq Mehnaz Ahmed, Khan Mansoor Ahmed, Amanullah Muhammad Muneer
School of Medicine, Aga Khan University, Karachi, Pakistan.
J Pak Med Assoc. 2011 Aug;61(8):836-8.
Aorto-pulmonary window is a relatively rare congenital cardiac malformation with an overall incidence of 0.1%. Pulmonary hypertension develops quickly if the lesion is left untreated hence early surgical intervention is warranted after diagnosis. The surgery for correction of APW has evolved over years, currently an open repair on cardiopulmonary bypass (CPB) with a single patch technique yields good results. Mortality is affected by association of pulmonary hypertension and other cardiac malformations. We present a case of an infant with a large type II APW with a relatively low pulmonary vascular resistance. Hospital stay was complicated because of pulmonary arterial disease making it an important reason for correction in the first few months of life.
主-肺动脉窗是一种相对罕见的先天性心脏畸形，总体发病率为0.1%。如果病变不治疗，肺动脉高压会迅速发展，因此诊断后有必要尽早进行手术干预。多年来，主-肺动脉窗矫正手术不断发展，目前在体外循环（CPB）下采用单补片技术进行开放修复可取得良好效果。死亡率受肺动脉高压和其他心脏畸形的合并影响。我们报告一例患有大型II型主-肺动脉窗且肺血管阻力相对较低的婴儿病例。由于肺动脉疾病，住院治疗变得复杂，这使其成为在生命最初几个月进行矫正的重要原因。
