Kaulitz Renate, Ziemer Gerhard, Hofbeck Michael
Pädiatrische Kardiologie und Intensivmedizin, Universitätsklinikum, Tübingen.
Herz. 2004 Nov;29(7):686-94. doi: 10.1007/s00059-004-2572-6.
Atrial isomerism and visceral heterotaxy describe complex pathoanatomic findings with defects in the determination of lateralization. Differentiation of right and left atrial isomerism was based on the anatomy of the atrial appendages and bronchial tree and often associated with asplenia in right atrial isomerism and polysplenia in left atrial isomerism. In these pa tients complex cardiac anomalies determine clinical symptoms and diagnostic procedures. At primary diagnosis in the neonatal period, systemic venous and pulmonary venous connections and intracardiac anatomy are identified using the sequential segmental approach. Therapeutic decisions and palliative procedures can usually be based on these initial echocardiographic findings. As many of the patients were found to have a functionally univentricular heart, surgical procedures following the Fontan principle (lateral tunnel or extracardiac conduit procedure) were introduced in patients with heterotaxy syndrome and successfully performed. Early survival and long-term outcome vary depending on associated cardiac and systemic and pulmonary venous anomalies. The therapeutic options and mid-term results in patients with heterotaxy syndrome undergoing the Fontan procedure are reported.
心房异构和内脏异位描述了具有侧化确定缺陷的复杂病理解剖学发现。右心房异构和左心房异构的区分基于心耳和支气管树的解剖结构，右心房异构常与无脾症相关，左心房异构常与多脾症相关。在这些患者中，复杂的心脏异常决定了临床症状和诊断程序。在新生儿期初次诊断时，使用顺序节段法确定体静脉和肺静脉连接以及心内解剖结构。治疗决策和姑息性手术通常可基于这些初始超声心动图检查结果。由于发现许多患者患有功能性单心室心脏，因此针对异位综合征患者引入了遵循Fontan原则的手术程序（侧隧道或心外管道手术）并成功实施。早期生存率和长期预后因相关的心脏、体循环和肺静脉异常而异。本文报道了接受Fontan手术的异位综合征患者的治疗选择和中期结果。
