教育演講3:台大兒童醫院心臟團隊經驗分享論壇:
先天性心臟病治療演變及發展
State-of-the-Art Therapy for Congenital Heart Disease

程 序 表

E3-4
Surgical correction and palliation for complex congenital heart disease
黃書健
台大醫院心臟外科

  The treatment for congenital heart disease evolved in the fifty years. The first palliative procedure for Tetralogy of Fallot was Blalock-Taussig Shunt. The corrective surgery progressed rapidly as the cardiopulmonary bypass was developed. Deep hypothermic circulatory arrest further increased the possibility for correct the complex congenital heart, and also the neonatal cardiac surgery. Currently, despite the challenge in surgical technique and peri-operative care, most of complex congenital heart disease could be repaired with low mortality and morbidity.
  In the biventricular congenital heart diseases, early corrective surgery was advocated. Most of the patients with Tetralogy of Fallot are repaired in their infancy. Total anomalous pulmonary venous return(TAPVR) and transposition of great arteries(TGA) are repaired in neonatal stage, with excellent outcome. Surgical strategy for double outlet right ventricle(DORV) are decided by their specific hemodynamic status, and usually bi-ventricular repair could be done either primarily or staged. The truncus arteriosus was repaired in early infancy with modified Rastelli operation.
  The tricuspid valve repair for Ebstein's anomaly are developed in various centers, and the more physiologic repair method could restore the right ventricular performance. For the patients with pulmonary atresia, VSD and major aortopulmonary collateral arteries (PA-VSD, MAPCAs), usually staged operation is needed, and unifocalization with Rastelli operation could restore their bi-ventricular circulation.
  For patients could not achieve bi-ventricular repair, staged single-ventricular reconstruction is the current standard therapy.
  The first stage operation should focused on the un-obstructed systemic outflow, control of the pulmonary blood flow and preserve pulmonary artery geometry, with well mixed systemic and pulmonary venous return. The procedures included Norwood S1P, BT shunt, PA banding, atrial septectomy to achieve the goal. The second stage operation is generally performed around 6-month-old, the procedures composed of the superior caval vein to pulmonary artery anastomosis, and closure of other systemic pulmonary shunts. The third stage is total cavopulmonary anastomosis, with or without fenestration. The traditional atrial-pulmonary Fontan operation was no longer performed today.
  The patients with congenital heart disease might also combined with airway anomalies. Slide tracheoplasty is the preferred treatment for congenital tracheal stenosis, which could be performed simultaneous during cardiac surgery or separate procedure.