[右心房异构婴儿的长期预后和心律失常]

Cheung Yiu-fai, Cheng Yan-wah, Chau Kai-tung Adolphus, Yung Tak-cheung, Tong Kai-sing

Division of Paediatric Cardiology, Grantham Hospital, Department of Paediatrics and Adolescent Medicine, The University of Hong Kong, Hong Kong Special Administrative Region, China.

Zhonghua Er Ke Za Zhi. 2004 Mar;42(3):166-71.

OBJECTIVE

The investigators compared the outcome of infants and children having right atrial isomerism with normal pulmonary venous drainage to those with anomalous drainage and determined factors associated with poor outcome. They further determined the prevalence of symptomatic cardiac arrhythmia in these patients and its relation to long-term morbidity and mortality.

METHODS

The authors made a retrospective review of management and outcome of 116 infants and children diagnosed to have right atrial isomerism between January 1980 and December 2000. The type, timing and precipitating factors of symptomatic cardiac arrhythmia that occurred in patients, among a cohort of 85 who had or are awaiting surgical interventions, were noted.

RESULTS

The 116 patients presented at a median of 1 day (range 1 day to 3.7 years) with cyanosis in the majority (96%). No interventions were planned in 31 (27%) patients who all died. The early surgical mortality for pulmonary venous repair was 25% (2/8), Fontan procedure 26% (5/19), cavopulmonary shunting 8% (1/13) and systemic-pulmonary arterial shunt insertion 2% (1/53). Late mortality was related to infection (n = 10), sudden death of unknown aetiology (n = 7) and documented arrhythmia (n = 1). Patients with obstructed anomalous pulmonary venous drainage had poor survival (P < 0.001). The mean (SEM) survival estimates for those with normal pulmonary venous drainage at 1, 5, 10 and 15 years were 81 (5)%, 67 (7)%, 60 (8)% and 43 (12)%, respectively, similar to those of patients with non-obstructed anomalous drainage (P = 0.06). Independent risk factors for mortality included pulmonary venous obstruction (relative risk RR 3.8, P = 0.001) and a single ventricle (RR 2.9, P = 0.016). Symptomatic cardiac arrhythmia occurred in 15/85 (18%) patients; 11 of whom had supraventricular tachycardia, and 1 atrial tachycardia, 1 atrial flutter, 1 ventricular tachycardia and 1 congenital complete heart block. The arrhythmias occurred before surgery in 4, early after surgery in 5, and late after surgery in 6 patients. Freedom from arrhythmia at 1, 5, 10, 15 and 20 years was (93 +/- 3)%, (86 +/- 4)%, (80 +/- 6)%, (73 +/- 9)% and (48 +/- 15)%, respectively. Logistic regression failed to identify any risk factors for symptomatic arrhythmia.

CONCLUSION

The long-term outcome of infants and children with right atrial isomerism, whether associated with normal or anomalous pulmonary venous drainage, remains unfavourable. Sepsis and sudden death are major causes of late mortality. While symptomatic cardiac arrhythmias are not uncommon. They do not seem to relate to the overall high mortality and occurrence of sudden death in this patient group. Nonetheless, detailed assessment and aggressive management of cardiac arrhythmias once they occur are warranted in light of the precarious single ventricular haemodynamics.

目的

研究者比较了具有正常肺静脉引流的右心房异构的婴幼儿和儿童与具有异常引流者的结局，并确定了与不良结局相关的因素。他们进一步确定了这些患者中症状性心律失常的发生率及其与长期发病率和死亡率的关系。

方法

作者回顾性分析了1980年1月至2000年12月期间诊断为右心房异构的116例婴幼儿和儿童的治疗及结局。记录了85例已接受或正在等待手术干预的患者中发生的症状性心律失常的类型、时间及诱发因素。

结果

116例患者就诊时的中位年龄为1天（范围1天至3.7岁），大多数患者（96%）有发绀。31例（27%）患者未计划进行任何干预，均死亡。肺静脉修复的早期手术死亡率为25%（2/8），Fontan手术为26%（5/19），腔肺分流术为8%（1/13），体肺分流术为2%（1/53）。晚期死亡率与感染（n = 10）、不明病因的猝死（n =）和有记录的心律失常（n = 1）有关。肺静脉异常引流受阻的患者生存率低（P < 0.001）正常肺静脉引流患者在1、5、10和15年时的平均（SEM）生存估计分别为81（5）%、67（7）%、60（8）%和43（12）%，与非阻塞性异常引流患者相似（P = 0.06）。死亡的独立危险因素包括肺静脉阻塞（相对危险度RR 3.8，P = 0.001）和单心室（RR 2.9，P = 0.016）。85例患者中有15例（18%）发生症状性心律失常；其中11例为室上性心动过速，1例为房性心动过速，1例为心房扑动，1例为室性心动过速，1例为先天性完全性心脏传导阻滞。心律失常发生在术前的有4例，术后早期的有5例，术后晚期的有6例。1、5、10、15和20年时无心律失常的比例分别为（93 ± 3）%、（86 ± 4）%、（80 ± 6）%、（73 ± 9）%和（48 ± 15）%。逻辑回归未能确定症状性心律失常的任何危险因素。