Presbitero P, Somerville J, Rabajoli F, Stone S, Conte M R
Grown-up Congenital Heart Unit, Royal Brompton National Heart and Lung Hospital, London.
Br Heart J. 1995 Jul;74(1):57-9. doi: 10.1136/hrt.74.1.57.
To assess the clinical course of adult patients with corrected transposition of the great arteries without associated anomalies.
All patients with corrected transposition of the great arteries without associated anomalies were reviewed with complete clinical and echocardiographic assessment. The complications were evaluated in each decade.
Tertiary centre with a specific unit dealing with "grown-up" adolescent and adult congenital heart disease, designated as a quaternary centre and a general hospital with a referral centre for "grown-up" congenital heart disease.
18 patients (nine male and nine female) aged 16-61 years followed for 1-30 years (mean 10 years).
There were no deaths. Six patients had a worsening ability index during follow up. Complications were: (a) complete heart block in seven, three of whom required pacemaker insertion; (b) significant left atrioventricular valve regurgitation in 50%, appearing only in the third decade (12%), with increasing frequency thereafter. Infective endocarditis was responsible for increasing left atrioventricular valve regurgitation in only one patient; (c) supraventricular arrhythmia appeared in the fifth decade, and occurred in all patients over the age of 60 years. One patient aged 61 had recurrent sustained ventricular tachycardia; and (d) congestive heart failure developed only after 50 years in 66%. One patient had severe left atrioventricular valve regurgitation; the function of the systemic ventricle was only moderately reduced in the other three. Three of the nine women had seven uneventful pregnancies.
Patients with corrected transposition of the great arteries without associated defects may remain undiagnosed until adult life. Symptoms occur rarely before the fourth and fifth decades, when rhythm disturbance, left atrioventricular valve regurgitation, and moderately impaired systemic ventricular function cause congestive cardiac failure. The role of pacemaker insertion or surgery for left atrioventricular valve regurgitation needs further assessment.
评估无相关异常的矫正型大动脉转位成年患者的临床病程。
对所有无相关异常的矫正型大动脉转位患者进行全面的临床和超声心动图评估复查。对每个十年中的并发症进行评估。
设有专门处理“成年”青少年及成人先天性心脏病的科室的三级中心，该中心被指定为四级中心，以及设有“成年”先天性心脏病转诊中心的综合医院。
18例患者（9例男性和9例女性），年龄16 - 61岁，随访1 - 30年（平均10年）。
无死亡病例。6例患者在随访期间能力指数恶化。并发症包括：（a）7例发生完全性心脏传导阻滞，其中3例需要植入起搏器；（b）50%的患者出现显著的左房室瓣反流，仅在第三个十年出现（12%），此后频率增加。仅1例患者因感染性心内膜炎导致左房室瓣反流加重；（c）室上性心律失常出现在第五个十年，60岁以上的所有患者均有发生。1例61岁患者发生复发性持续性室性心动过速；（d）66%的患者在50岁后出现充血性心力衰竭。1例患者有严重的左房室瓣反流；其他3例患者体循环心室功能仅中度降低。9例女性中有3例经历了7次平安的妊娠。
无相关缺陷的矫正型大动脉转位患者可能直到成年才被诊断出来。在第四和第五个十年之前很少出现症状，此时节律紊乱、左房室瓣反流和体循环心室功能中度受损会导致充血性心力衰竭。对于左房室瓣反流植入起搏器或进行手术的作用需要进一步评估。
