Early and late results of the modified Fontan operation for heterotaxy syndrome - 30 years of experience in 142 patients
Early and late results of the modified Fontan operation for heterotaxy syndrome - 30 years of experience in 142 patients
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DOI：
10.1016/j.jacc.2006.07.053
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发表时间：
2006-12-05
影响因子：
24
通讯作者：
Cetta, Frank
中科院分区：
文献类型：
--
作者：
Bartz, Peter J.;Driscoll, David J.;Cetta, Frank
目的评价改良Fontan手术治疗异位综合征患者的早期和远期疗效，评估传统上认为与不良预后相关的变量，以及评估Fontan手术后幸存者的健康状况。因此，Fontan手术被提出以提高存活率。从住院和门诊记录中提取手术前、手术后和术后的临床和血流动力学数据。结果142例异位综合征患者接受了改良的Fontan手术。76例(54%)患者出现脾功能不全。中位年龄9岁(2~35岁)。中位随访期为4年(0~23年)。共有61例死亡(43%)，其中32例(23%)在手术后30天内或出院前死亡。5年、10年和15年生存率分别为%、57%和53%。在现代(1995年至2004年)，早期死亡率为10%。在81名幸存者中，有41人(51%)获得了问卷。80%的人报告没有症状或症状轻微。然而，19例(46%)发生心律失常，5例(12%)发生血栓栓塞症，1例(2%)发展为蛋白缺失性肠病。结论Fontan手术后异位症患者的早期存活率有所提高，但晚期发病率和死亡率仍然很高。需要确定对这一高危群体进行长期治疗的更好战略。
OBJECTIVES We sought to evaluate the early and late results of the modified Fontan operation for patients with heterotaxy syndrome, assess variables traditionally known to correlate with poor outcome, and assess current health status of survivors after the Fontan procedure.BACKGROUND The natural history of unoperated cardiac lesions in heterotaxy is known to be poor. Therefore, the Fontan operation has been proposed to improve survival.METHODS Patients with heterotaxy were identified from the Mayo Clinic Fontan database. Hospital and outpatient records were abstracted for preoperative, operative, and postoperative clinical and hemodynamic data. All patients not known to be deceased were sent health status questionnaires.RESULTS A total of 142 patients with heterotaxy syndrome had a modified Fontan operation. Asplenia was present in 76 patients (54%). Median age at operation was 9 years (range 2 to 35 years). Median follow-up was 4 years (range 0 to 23 years). There were a total of 61 deaths (43%), with 32 (23%) within 30 days of operation or before hospital discharge. The 5-, 10-, and 15-year survival was 64%, 57%, and 53%, respectively. In the modern era (1995 to 2004) early mortality was 10%. Of the 81 survivors, questionnaires were available from 41 (51%). Eighty percent reported having no or mild symptoms. However, 19 (46%) had arrhythmias, 5 (12%) had a thromboembolic event, and 1 (2%) developed protein-losing enteropathy.CONCLUSIONS Early survival has improved for heterotaxy patients after the Fontan operation; however, late morbidity and mortality remain substantial. Better strategies for long-term treatment of this high-risk group need to be identified.