Outcome after Fontan failure and takedown to an intermediate palliative circulation

Outcome after Fontan failure and takedown to an intermediate palliative circulation
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DOI:
10.1016/j.athoracsur.2007.02.092
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发表时间:
2007-09-01
影响因子:
4.6
通讯作者:
McElhinney, Doff B.
McElhinney, Doff B.
中科院分区:
医学2区
文献类型:
--
作者:
Almond, Christopher S. D.;Mayer, John E.;McElhinney, Doff B.

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背景资料。对于由于各种原因导致的急性或亚急性Fontan循环衰竭的患者,Fontan降至中间姑息循环是一种重要的治疗选择。关于这些患者的后续结果或他们再次尝试Fontan完成治疗的可能性,我们知之甚少。对在波士顿儿童医院接受Fontan循环的患者进行了随访,这些患者在Fontan完成后1年内接受了Fontan循环到中间姑息循环。在1979至2006年间,53名患者接受了Fontan停药治疗,平均年龄为2.3岁(范围为0.3至36.5岁)。Fontan手术中12例(22%),术后1个月内31例(58%),1个月至1年10例(18%)。总体而言,29名患者(55%)在摘除后的早期存活,19名患者最终成功完成Fontan矫治术,中位数为4.6年;除1名患者外,其余患者均存活,中位数为6.4年。19例中有13例(68%)有导致Fontan衰竭的可治疗的异常。服用Fontan可以有效稳定急性或亚急性衰竭的Fontan循环,尽管尽管Fontan服用,仍有相当数量的患者过早死亡。在围手术期存活的受试者通常可以接受平安无事的Fontan再手术。对于所有Fontan循环衰竭的患者和接受Fontan停药的患者,都应该对可治疗的异常进行彻底的评估。
Background. Fontan takedown to an intermediate palliative circulation is an important treatment option for patients with acute or subacute failure of a Fontan circulation from a variety of causes. Little is known about the subsequent outcome of these patients or their potential candidacy for a second attempt at Fontan completion.Methods. Patients followed up at Children's Hospital Boston who underwent takedown of a Fontan circulation to an intermediate palliative circulation within 1 year of Fontan completion were reviewed.Results. Between 1979 and 2006, 53 patients underwent Fontan takedown at a median age of 2.3 years ( range, 0.3 to 36.5 years). Takedown was performed during the Fontan procedure itself in 12 patients ( 22%), within the first postoperative month in 31( 58%), and between 1 month and 1 year in 10 ( 18%). Overall, 29 patients ( 55%) survived the early period after takedown, and 19 ultimately underwent successful Fontan completion a median of 4.6 years after takedown; all but one was alive a median of 6.4 years later. Thirteen ( 68%) of the 19 had treatable abnormalities contributing to Fontan failure.Conclusions. Fontan takedown can provide effective stabilization of the acutely or subacutely failing Fontan circulation, although a substantial number of patients die early despite Fontan takedown. Subjects surviving the perioperative period can often undergo uneventful redo Fontan. A thorough evaluation for treatable abnormalities should be performed in all patients with a failing Fontan circulation and in patients who undergo Fontan takedown.