Sagin-Saylam G, Somerville J
Grown-Up congenital Heart (GUCH) Unit, Royal Brompton Hospital, London.
Heart. 1996 Jan;75(1):72-7. doi: 10.1136/hrt.75.1.72.
To assess the clinical, functional state, and complications late (15-20 years) after palliative Mustard operation.
Examination and evaluation of all patients presenting in adolescence and adult life after palliative Mustard operation for transposition of the great arteries and pulmonary vascular disease.
Grown-up Congenital Heart Unit specialised in the care of adolescents and adults with congenital heart disease, designated as having "quaternary" status within a tertiary referral centre for cardiac diseases.
Database searched for patients referred after palliative Mustard for classic transposition of the great arteries. Ten patients aged 18-31 (mean (SD) 25.9 (5.2)) years with a palliative Mustard operation performed at age 1.7-15 (mean (SD) 9 (4.6)) years were fully evaluated by echocardiography, exercise testing, Holter monitoring, and magnetic resonance imaging or radionuclide ventriculography, or both 15-20 years later.
One patient died aged 25 years with biventricular failure (ability index 3/4), haemoptysis, and atrial flutter, eight were well (ability index 2), and one disabled (ability index 3). Arterial oxygen saturation at rest was 85-98% (mean (SD) 93.8 (4)%) decreasing to 59-87% (mean 77 (9.5)%) after limited exercise. Symptomatic arrhythmias occurred in four patients, atrial flutter being the most common, and two had sinus node dysfunction. Significant tricuspid regurgitation occurred in four patients.
Patients with palliative Mustard operation have a low incidence of symptomatic ventricular dysfunction and despite exercise limitation by hypoxia, continue to live active, near normal lives until their thirties.
评估姑息性Mustard手术后晚期(15 - 20年)的临床情况、功能状态及并发症。
对所有在青少年期及成年期接受姑息性Mustard手术治疗大动脉转位及肺血管疾病的患者进行检查和评估。
成人先天性心脏病单元,专门负责照顾患有先天性心脏病的青少年和成人,在三级心脏病转诊中心内被指定为具有“四级”地位。
在数据库中搜索因姑息性Mustard手术治疗经典大动脉转位而转诊的患者。10例年龄在18 - 31岁(平均(标准差)25.9(5.2)岁)、在1.7 - 15岁(平均(标准差)9(4.6)岁)时接受姑息性Mustard手术的患者,在15 - 20年后通过超声心动图、运动试验、动态心电图监测以及磁共振成像或放射性核素心室造影(或两者)进行全面评估。
1例患者在25岁时死于双心室衰竭(心功能指数3/4)、咯血和心房扑动,8例情况良好(心功能指数2),1例残疾(心功能指数3)。静息时动脉血氧饱和度为85% - 98%(平均(标准差)93.8(4)%),有限运动后降至59% - 87%(平均77(9.5)%)。4例患者出现有症状的心律失常,最常见的是心房扑动,2例有窦房结功能障碍。4例患者发生显著的三尖瓣反流。
接受姑息性Mustard手术的患者有症状性心室功能障碍的发生率较低,尽管因缺氧限制运动,但直到三十多岁仍能积极生活,生活接近正常。