Outcome of the unoperated adult who presents with congenitally corrected transposition of the great arteries
Outcome of the unoperated adult who presents with congenitally corrected transposition of the great arteries
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DOI：
10.1016/s0735-1097(02)01952-6
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发表时间：
2002-07-17
影响因子：
24
通讯作者：
Danielson, GK
中科院分区：
文献类型：
--
作者：
Beauchesne, LM;Warnes, CA;Danielson, GK
目的：本研究的目的是确定先天性大动脉转位成人未手术的表现和结果。背景：在未手术的成人中，这种疾病的表现和结果还没有很好的定义。方法对所有大于或等于18岁的未手术患者进行疾病谱、血流动力学严重程度、诊断和转诊的及时性和结局的评估。结果44例患者，年龄20 ~ 79岁，平均44例，随访144个月。在29例（66%）中，首次做出正确诊断的年龄大于或等于18岁；在这些患者中，有7人在先前的心脏病学咨询中被误诊，尽管有心脏成像。26例（59%）患者出现系统性房室瓣膜（SAVV）反流（大于或等于3/4级）。30例（68%）接受了手术干预，包括所有SAW置换，无早期死亡。术前，该亚群有明显的全身心室功能障碍（SV）（射血分数[EF]， 40 +/- 10%），大多数有晚期症状（25例能力指数大于或等于2/4）。16例（53%）的患者在6个月的时间里记录了SAW反流大于或等于3/4和心室功能障碍。SV的平均EF术后显著降低（34±11%,p = 0.006）。4例（13%）患者最终需要心脏移植。术前SV EF差预测最终需要移植（p = 0.001）。结论：未手术先天性纠正大动脉转位的患者在成年期经常被误诊，尽管有症状性SAW反流和明显的SV功能障碍，但他们的转诊时间较晚。虽然早期手术可以取得良好的效果，但SV明显的残余功能障碍是常见的。
OBJECTIVES The goal of this study was to determine the presentation and outcome of the unoperated adult with congenitally corrected transposition of the great arteries.BACKGROUND The presentation of this disorder and the outcome in unoperated adults have not been well defined.METHODS All unoperated patients greater than or equal to18 years old were evaluated for spectrum of disease, hemodynamic severity, timeliness of diagnosis and referral, and outcome.RESULTS Forty-four patients aged 20 to 79 years (mean, 44) were followed up to 144 months. In 29 (66%), the correct diagnosis was first made at age greater than or equal to18 years; the diagnosis was missed in seven of these patients in a prior cardiology consultation, despite cardiac imaging. Systemic atrioventricular valve (SAVV) regurgitation (grade greater than or equal to3/4) was noted in 26 patients (59%). Thirty (68%) had surgical intervention, including SAW replacement in all, with no early mortality. Preoperatively, this subset had significant dysfunction of the systemic ventricle (SV) (ejection fraction [EF], 40 +/- 10%), and most had advanced symptoms (25 with ability index greater than or equal to2/4). In 16 (53%), SAW regurgitation greater than or equal to3/4 and ventricular dysfunction had been documented for >6 months. The mean EF of the SV decreased significantly postoperatively (34 +/- 11%, p = 0.006). Four patients (13%) eventually required cardiac transplantation. Poor preoperative EF of the SV predicted eventual need for transplantation (p = 0.001).CONCLUSIONS Patients with unoperated congenitally corrected transposition of the great arteries are often misdiagnosed in adulthood and are referred late despite symptomatic SAW regurgitation and significant SV dysfunction. Although excellent early surgical results can be achieved, significant residual dysfunction of the SV is common.