右心房异构引起的远期结果及心律失常

目的　回顾性研究右心房异构患儿的远期结果及有症状性心律失常的发生率及其与死亡的关系。方法　回顾 1980年 1月～ 2 0 0 0年 12月收治的 116例右心房异构婴幼儿和儿童的治疗和预后情况。将右心房异构的患儿分为肺静脉正常回流和肺静脉异位回流两组 ,通过比较两组患儿的预后来分析导致预后不良的因素 ,并对其中已经完成或准备接受外科手术治疗的 85例 ,分析其有症状性心律失常的类型、发病时间和发病诱因 ,从而了解有症状性心律失常对患儿的长期预后所造成的影响。结果　 116例中 ,大多数患儿 ( 96 % )的临床症状是紫绀 ,出现临床症状的年龄中位数为 1天(范围 1天～ 3 7岁 )。其中 31例 ( 2 7% )无手术适应证 ,最终死亡。早期肺静脉修复手术的死亡率为2 /8例 ) ,Fontan手术的早期死亡率为 2 6 % ( 5 /19) ,腔肺静脉分流术的早期死亡率为 8% ( 1/13) ,体肺循环动脉分流术的早期死亡率为 2 % ( 1/5 3)。晚期死亡与感染 (n =11)、原因不明的突然死亡 (n =7)和心律失常 (n =1)有关。肺静脉正常回流的患儿 ,其中 1、5、10和 15岁患儿的平均存活率分别为( 81± 5 ) %、( 6 7± 7) %、( 6 0± 8) %和 ( 4 3± 12 ) % ,与非阻塞性肺静脉异位回流患儿的平均存活率相似。导致高死亡率的危险因素包括肺静脉回流阻

Long-term outcome and cardiac arrhythmias in infants with right atrial isomerism

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.