Beauchesne Luc M, Warnes Carole A, Connolly Heidi M, Ammash Naser M, Tajik A Jamil, Danielson Gordon K
Division of Cardiovascular Diseases and Internal Medicine, Mayo Clinic, 200 First Street SW, Rochester, MH 55905, USA.
J Am Coll Cardiol. 2002 Jul 17;40(2):285-90. doi: 10.1016/s0735-1097(02)01952-6.
The goal of this study was to determine the presentation and outcome of the unoperated adult with congenitally corrected transposition of the great arteries.
The presentation of this disorder and the outcome in unoperated adults have not been well defined.
All unoperated patients > or =18 years old were evaluated for spectrum of disease, hemodynamic severity, timeliness of diagnosis and referral, and outcome.
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 > or =18 years; the diagnosis was missed in seven of these patients in a prior cardiology consultation, despite cardiac imaging. Systemic atrioventricular valve (SAVV) regurgitation (grade > or =3/4) was noted in 26 patients (59%). Thirty (68%) had surgical intervention, including SAVV 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 > or =2/4). In 16 (53%), SAVV regurgitation > or =3/4 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).
Patients with unoperated congenitally corrected transposition of the great arteries are often misdiagnosed in adulthood and are referred late despite symptomatic SAVV regurgitation and significant SV dysfunction. Although excellent early surgical results can be achieved, significant residual dysfunction of the SV is common.
本研究的目的是确定未接受手术治疗的先天性矫正型大动脉转位成年患者的临床表现及预后。
这种疾病的临床表现以及未接受手术治疗的成年患者的预后尚未得到明确界定。
对所有年龄≥18岁的未接受手术治疗的患者进行疾病谱、血流动力学严重程度、诊断和转诊及时性以及预后评估。
44例年龄在20至79岁（平均44岁）的患者接受了长达144个月的随访。其中29例（66%）在18岁及以上时首次确诊；在这些患者中，有7例尽管进行了心脏成像检查，但在之前的心脏科会诊中仍被漏诊。26例患者（59%）出现了体循环房室瓣反流（≥3/4级）。30例（68%）接受了手术干预，均包括体循环房室瓣置换，无早期死亡病例。术前，该亚组患者的体循环心室（SV）功能明显受损（射血分数[EF]为40±10%），且大多数患者有晚期症状（25例功能指数≥2/4）。16例（53%）患者的体循环房室瓣反流≥3/4且心室功能障碍已记录超过6个月。术后体循环心室的平均射血分数显著下降（34±11%，p = 0.006）。4例患者（13%）最终需要进行心脏移植。术前体循环心室射血分数低预示着最终需要进行移植（p = 0.001）。
未接受手术治疗的先天性矫正型大动脉转位患者在成年期常被误诊，尽管存在有症状的体循环房室瓣反流和明显的体循环心室功能障碍，但转诊往往较晚。尽管早期手术效果良好，但体循环心室仍普遍存在明显的残余功能障碍。
