Hashmi A, Abu-Sulaiman R, McCrindle B W, Smallhorn J F, Williams W G, Freedom R M
Division of Cardiology, The Hospital for Sick Children, Toronto, Ontario, Canada.
J Am Coll Cardiol. 1998 Apr;31(5):1120-6. doi: 10.1016/s0735-1097(98)00062-x.
We sought to determine, in a large series of patients with right atrial isomerism, factors associated with mortality.
Right atrial isomerism is associated with complex congenital heart disease and high morbidity and mortality.
All data from patients diagnosed with right atrial isomerism between January 1970 and March 1996 were reviewed.
A total of 91 consecutive patients (54 male) were identified. Most patients (89%) presented within the first month of life, 62% at birth. Cardiac abnormalities included common atrioventricular (AV) valve (81%), ventricular hypoplasia or single ventricle (73%), abnormal ventriculoarterial connections (96%), pulmonary outflow tract obstruction (84%), anomalous pulmonary venous drainage (87%) and pulmonary vein obstruction (30%). The overall mortality rate was 69%. No interventions were planned or performed in 24%, 95% of whom died. The mortality rate for patients requiring their first cardiovascular operation in the neonatal period was 75% versus 51% for those with later first operations (p < 0.05). The surgical mortality rate for patients undergoing pulmonary vein repair was 95%. Overall survival estimates were 71% at 1 month, 49% at 1 year and 35% at 5 years. Independent risk factors for decreased time to death included the absence of pulmonary outflow obstruction (relative risk [RR] 2.23, p < 0.03), presence of major AV valve anomaly (RR 5.23, p < 0.03) and obstructed pulmonary veins (RR 5.43, p < 0.0001).
Right atrial isomerism continues to have an associated high mortality despite surgical innovations. Management of pulmonary vein obstruction remains a serious problem and is associated with high mortality.
我们试图在一大组右心房异构患者中确定与死亡率相关的因素。
右心房异构与复杂先天性心脏病以及高发病率和死亡率相关。
回顾了1970年1月至1996年3月期间诊断为右心房异构患者的所有数据。
共确定了91例连续患者（54例男性）。大多数患者（89%）在出生后第一个月内就诊，62%在出生时就诊。心脏异常包括共同房室瓣（81%）、心室发育不全或单心室（73%）、心室动脉连接异常（96%）、肺流出道梗阻（84%）、肺静脉引流异常（87%）和肺静脉梗阻（30%）。总死亡率为69%。24%的患者未计划或进行任何干预，其中95%死亡。新生儿期首次进行心血管手术的患者死亡率为75%，而首次手术时间较晚的患者死亡率为51%（p<0.05）。接受肺静脉修复手术的患者手术死亡率为95%。总体生存估计为1个月时71%，1岁时49%，5岁时35%。死亡时间缩短的独立危险因素包括无肺流出道梗阻（相对危险度[RR]2.23，p<0.03）、存在主要房室瓣异常（RR 5.23，p<0.03）和肺静脉梗阻（RR 5.43，p<0.0001）。
尽管有手术创新，右心房异构仍然具有较高的死亡率。肺静脉梗阻的治疗仍然是一个严重问题，且与高死亡率相关。
