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首页> 外文期刊>Journal of cardiac surgery. >Complete repair of Tetralogy of Fallot with absent pulmonary valve including the role of airway stenting.
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Complete repair of Tetralogy of Fallot with absent pulmonary valve including the role of airway stenting.

机译：完全没有肺动脉瓣修复法洛四联症，包括气道支架置入术。

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Tetralogy of Fallot (TOF) with absent pulmonary valve (APV) represents an extreme form of tetralogy where pulmonary insufficiency and mild annular stenosis often results in massive pulmonary arterial (PA) dilatation. The aneurysmal left and right PAs often compress the adjacent trachea and bronchi, leading to airway obstruction and respiratory failure in infancy. Between 1991 and 1997, 11 patients underwent a single stage repair of TOF and APV using a valved (10 patients) or nonvalved (1 patient) homograft conduit and PA reduction arterioplasty. There was one (1/11 [9.1%) perioperative and one (9.1%) late death. Both deaths were related to airway complications. Morbidity associated with postoperative respiratory complications and ventilator-dependency due to underlying tracheobronchomalacia is an important problem. Intermediate follow-up shows a high incidence of reintervention for conduit stenosis and/or insufficiency and tracheobronchial compression. These infants also required multiple hospitalizations for recurrent respiratory infections secondary to their tracheobronchomalacia. Stenting of the right and left main bronchi with balloon expandable metallic stents is a new experimental therapy that has been useful in two recent patients with respiratory failure despite satisfactory intracardiac repair. It may provide an attractive alternative therapy to prolonged mechanical ventilation with positive end expiratory pressure in patients with severe tracheobronchomalacia. Complete repair with a valved homograft conduit and reduction pulmonary arterioplasty in infancy at the time of diagnosis is the procedure of choice for infants with TOF with APV. With this approach the patient outcome is essentially determined by their airway status and airway management.
机译：缺乏肺动脉瓣（APV）的法洛四联症（TOF）代表四联症的一种极端形式，其中肺功能不全和轻度环形狭窄通常会导致大量肺动脉（PA）扩张。左，右动脉瘤通常压迫邻近的气管和支气管，导致婴儿气道阻塞和呼吸衰竭。 1991年至1997年之间，有11例患者接受了带瓣膜（10例）或非瓣膜（1例）同种异体移植导管和PA减少性动脉成形术，对TOF和APV进行了单阶段修复。围手术期有1例（1/11 [9.1％），晚期死亡有1例（9.1％）。两次死亡均与气道并发症有关。与潜在的气管支气管软化症相关的术后呼吸系统并发症和呼吸机依赖性相关的发病率是一个重要的问题。中级随访显示导管狭窄和/或供血不足和气管支气管压迫的再次干预发生率很高。这些婴儿还需要因其气管支气管软化症继发的反复呼吸道感染而多次住院。右主动脉支气管和左主支气管支架用球囊扩张式金属支架支架置入术是一种新的实验性治疗方法，尽管有满意的心脏内修复术，但仍对两名近期出现呼吸衰竭的患者有用。对于严重的气管支气管软化症患者，它可以为长期机械通气，呼气末正压提供有吸引力的替代疗法。对于患有APV的TOF婴儿，诊断时应使用带瓣的同种异体移植导管进行完全修复，并在婴儿期减少肺动脉成形术。通过这种方法，患者的预后基本上取决于他们的气道状态和气道管理。

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