Tkebuchava T, Niederhäuser U, Weder W, von Segesser L K, Bauersfeld U, Felix H, Lachat M, Turina M I
Clinic for Cardiovascular Surgery, Children's Hospital, Zurich, Switzerland.
Ann Thorac Surg. 1996 Nov;62(5):1474-9. doi: 10.1016/0003-4975(96)00493-6.
Long-term observations in patients with Kartagener's syndrome (situs inversus, bronchiectasis, and sinusitis) are rare. The role of additional cardiac malformations and their surgical repair is not well known.
Nine patients (5 female and 4 male) with Kartagener's syndrome were identified and followed. Four patients had associated cardiac anomalies; 4 underwent total surgical repair at the ages of 4 (2 patients), 7, and 34 years.
The postoperative period was uneventful, and these 4 patients are doing well 7 months and 2, 9, and 19 years after repair. The other patients are being treated with conservative therapy and are in relatively good condition.
This disease can be temporarily benign when treated with antibiotics and physiotherapy. Associated cardiac anomalies seem to be quite common, and such patients need careful cardiologic follow-up. Surgical intervention can be safely performed in patients suffering from Kartagener's syndrome associated with a congenital cardiac malformation and produces good long-term results. Bilateral lung transplantation seems to be the therapy of choice in patients with respiratory insufficiency but without concomitant cardiac anomalies.
对卡塔格内综合征（内脏转位、支气管扩张和鼻窦炎）患者的长期观察较为罕见。额外心脏畸形及其手术修复的作用尚不清楚。
确定并随访了9例卡塔格内综合征患者（5例女性，4例男性）。4例患者伴有心脏异常；4例分别在4岁（2例）、7岁和34岁时接受了全面手术修复。
术后恢复顺利，这4例患者在修复后7个月以及2年、9年和19年情况良好。其他患者接受保守治疗，状况相对良好。
使用抗生素和物理疗法治疗时，这种疾病可能暂时呈良性。相关心脏异常似乎相当常见，此类患者需要仔细的心脏科随访。对于患有与先天性心脏畸形相关的卡塔格内综合征的患者，可安全地进行手术干预，并产生良好的长期效果。对于有呼吸功能不全但无伴随心脏异常的患者，双侧肺移植似乎是首选治疗方法。
