Prieto L R, Hordof A J, Secic M, Rosenbaum M S, Gersony W M
Department of Pediatrics, College of Physicians and Surgeons of Columbia University, New York, NY, USA.
Circulation. 1998 Sep 8;98(10):997-1005. doi: 10.1161/01.cir.98.10.997.
The outcome of patients with corrected transposition of the great arteries (CTGA) is variably affected by associated intracardiac defects, tricuspid valve competence, and systemic right ventricular (RV) function. The relative importance of these factors in long-term outcome has not been evaluated.
Since 1958, 40 patients with CTGA were studied to determine risk factors for poor outcome, including age, open heart surgery, tricuspid insufficiency (TI), cardiac rhythm, pulmonary overcirculation, and RV dysfunction. Follow-up ranged from 7 to 36 years (mean 20 years). Intracardiac repair was performed in 21 patients; 19 were unoperated or had closed heart procedures. For the purposes of this study the designation TI(S) refers to at least moderately severe TI as delineated by echocardiogram and/or angiography. TI(s) was the only independently significant factor for death (P=0.01), and in turn, only the presence of a morphologically abnormal TV predicted TI(s)(P=0.03). Twenty-year survival without TI(S)was 93%, but only 49% with TI(S). Poor long-term postoperative outcome was due to TI(S) in all but 1 patient; 20-year survival rates for operated patients with and without TI(s)were 34% and 90%, respectively (P=0.002). Similarly, 20-year survival rates for unoperated patients with and without TI(s)were 60% and 100%, respectively, whether or not attempts to repair the TI were made (P=0.08).
TI(S)represents the major risk factor for CTGA patients; RV dysfunction appears to be almost always secondary to long-standing TI. Decisions related to surgical interventions with or without associated lesions must be strongly influenced by the status of the tricuspid valve.
矫正型大动脉转位（CTGA）患者的预后受到相关心内缺损、三尖瓣功能以及体循环右心室（RV）功能的不同程度影响。这些因素在长期预后中的相对重要性尚未得到评估。
自1958年以来，对40例CTGA患者进行了研究，以确定预后不良的危险因素，包括年龄、心脏直视手术、三尖瓣关闭不全（TI）、心律、肺循环过度以及RV功能障碍。随访时间为7至36年（平均20年）。21例患者接受了心内修复；19例未接受手术或接受了闭式心脏手术。在本研究中，TI(S)指经超声心动图和/或血管造影确定的至少为中度严重的TI。TI(s)是死亡的唯一独立显著因素（P=0.01），反过来，只有形态异常的三尖瓣（TV）存在才预示着TI(s)（P=0.03）。无TI(S)的患者20年生存率为93%，但有TI(S)的患者仅为49%。除1例患者外，所有术后长期预后不良均归因于TI(S)；有和无TI(s)的手术患者20年生存率分别为34%和90%（P=0.002）。同样，无论是否尝试修复TI，有和无TI(s)的未手术患者20年生存率分别为60%和100%（P=0.08）。
TI(S)是CTGA患者的主要危险因素；RV功能障碍似乎几乎总是长期TI的继发表现。对于伴有或不伴有相关病变的手术干预决策，必须受到三尖瓣状况的强烈影响。
