Department of Cardiology and Cardiac Surgery, Istituto di Ricovero e Cura a Carattere Scientifico, Ospedale Pediatrico Bambino Gesù, Rome, Italy.
Ann Thorac Surg. 2012 Sep;94(3):860-4. doi: 10.1016/j.athoracsur.2012.04.043. Epub 2012 Jun 12.
We discuss 4 cases of plastic bronchitis (PB) after Fontan procedure observed at our Institution, with the aim to identify an effective treatment and possible factors favoring the onset of PB. Plastic bronchitis is an uncommon disease characterized by recurrent formation of large pale bronchial casts obstructing the tracheobronchial tree. The treatment includes inhaled or systemic steroids, aerosolized mucolytics, bronchoscopic lavage, direct bronchoscopic extraction and, in few reports, aerosolized fibrinolytic and pulmonary vasodilators.
Four cases of PB after Fontan procedure occurred in our center from January 2008 to January 2012.
All patients showed preserved ventricle function and underwent embolization of systemic-pulmonary collaterals prior to a Fontan procedure and all had chylothorax in the perioperative period. Two patients died; 1 of massive thrombosis of the superior vena cava and right pulmonary artery and the other of acute asphyxiation and refractory cardiac failure.
The exact causes of PB in Fontan patients remain unknown. An early diagnosis and a multiple therapy with steroids, mucolytics, pulmonary vasodilators, and aerosolized tissue plasminogen activator may be effective.
我们讨论了在我院观察到的 4 例 Fontan 手术后发生的塑料性支气管炎（PB）病例，旨在确定有效的治疗方法和可能促使 PB 发生的因素。塑料性支气管炎是一种罕见疾病，其特征为反复形成大而苍白的支气管铸型，阻塞气管支气管树。治疗方法包括吸入或全身皮质类固醇、雾化黏液溶解剂、支气管镜灌洗、直接支气管镜下取出，以及少数报道中的雾化纤维蛋白溶解剂和肺血管扩张剂。
我们中心于 2008 年 1 月至 2012 年 1 月期间出现了 4 例 Fontan 手术后发生的 PB 病例。
所有患者均显示心室功能正常，并在 Fontan 手术前进行了体肺侧支循环栓塞，所有患者在围手术期均出现乳糜胸。2 例患者死亡；1 例死于上腔静脉和右肺动脉的巨大血栓形成，另 1 例死于急性窒息和难治性心力衰竭。
Fontan 手术后 PB 的确切病因仍不清楚。早期诊断和皮质类固醇、黏液溶解剂、肺血管扩张剂和雾化组织型纤溶酶原激活剂的多疗法可能有效。
