Behandlung des hypoplastischen Linksherz-Syndroms

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• 作者：Prof. Dr. H. H. Kramer (1) (2)
  O. Jung (1)
  G. Fischer (1)
  J. Scheewe (1)
  
• 关键词：Hypoplastisches Linksherzsyndrom ; Norwood ; Operation ; Fontan ; Operation ; Glenn ; Operation ; Kavopulmonale Anastomose ; Hypoplastic left heart syndrome ; Norwood operation ; Fontan operation ; Glenn operation ; Cavopulmonary anastomosis
• 刊名：Der Gyn?kologe
• 出版年：2006
• 出版时间：January 2006
• 年：2006
• 卷：39
• 期：1
• 页码：78-86
• 全文大小：967KB
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  6. Kumar KR, Newburger JW, Gauvreau K, Kamenir SA, Hornberger LK (1999) Comparison of outcome when hypoplastic left heart syndrome and transposition of the great arteries are diagnosed prenatally versus when diagnosis of these two conditions is made only postnatally. J Am Cardiol 83: 1649鈥?653 CrossRef
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• 作者单位：Prof. Dr. H. H. Kramer (1) (2)
  O. Jung (1)
  G. Fischer (1)
  J. Scheewe (1)
  
  1. Kinderherzzentrum, Universit盲tsklinikum Schleswig-Holstein, Kiel
  2. Kinderherzzentrum, Universit盲tsklinikum Schleswig-Holstein, Schwanenweg 20, 24105, Kiel
  

文摘

In contrast to other types of congenital heart defects, the treatment of hypoplastic left heart syndrome (HLHS) has become comparably successful only within the last decade. Postnatal management of circulatory disturbances of HLHS was previously often performed, similarly to other types of neonatal shock, without considering the peculiarities of postnatal hemodynamics. It is of overwhelming importance to limit pulmonary hyperperfusion by reducing systemic afterload and avoiding artificial respiration. The invention of selective hypothermic cerebral perfusion using the modified Blalock-Taussig shunt has decreased the need for long circulatory arrest times involving the brain, and promises a better neurological outcome. Postoperatively, sophisticated hemodynamic monitoring is mandatory to provide sufficient systemic oxygen delivery. 伪-blockers are usually given for strong afterload reduction. Hospital mortality is as low as 10鈥?5% in centers experienced with the Norwood operation. The next surgical steps to create a serial systemic and pulmonary circulation involve superior cavopulmonary anastomosis performed as early as possible (4鈥? months) and finally total cavopulmonary connection at an age of 3鈥? years.