Silka M J, Hardy B G, Menashe V D, Morris C D
Congenital Heart Disease Research Center Division of Pediatric Cardiology, Oregon Health Sciences University, Portland 97201, USA.
J Am Coll Cardiol. 1998 Jul;32(1):245-51. doi: 10.1016/s0735-1097(98)00187-9.
This study sought to define 1) the risk of sudden death after operation for common congenital heart defects; and 2) factors associated with an increased risk of sudden death.
Although the prognosis for patients with congenital heart defects is improved by surgical treatment, they remain at a well recognized but poorly defined risk of late sudden death.
This population-based study evaluated all patients < 19 years old undergoing surgical treatment of common forms of congenital heart disease in the state of Oregon between 1958 and 1996. Patients were identified retrospectively through 1958, with prospective biannual follow-up beginning in 1982. The incidence and cause of late sudden death were evaluated for 3,589 patients surviving operation for the following defects: atrial, ventricular and atrioventricular septal defects; patent ductus arteriosus; pulmonary stenosis; aortic stenosis; coarctation of the aorta; tetralogy of Fallot; and D-transposition of the great arteries.
There were 41 unexpected late sudden deaths during 45,857 patient-years of follow-up, an overall event rate of 1/1,118 patient-years. Thirty-seven of the 41 late sudden deaths occurred in patients with aortic stenosis, coarctation, transposition of the great arteries or tetralogy of Fallot, an event rate of 1/454 patients-years. In contrast, only four sudden deaths occurred among the other defects, an event rate of 1/7,154 patient-years (p < 0.01). The risk of late sudden death increased incrementally 20 years after operation for tetralogy of Fallot, aortic stenosis and coarctation. However, risk was not dependent on patient age at operation or surgical era. The causes of sudden death were arrhythmia in 30 patients, circulatory (embolic or aneurysm rupture) in 7 and acute heart failure in 4.
The risk of late sudden death for patients surviving operation for common congenital heart defects is 25 to 100 times greater than an age-matched control population. This increased risk is primarily represented by patients with cyanotic or left heart obstructive lesions. The risk of sudden death appears to be time dependent, increasing primarily after the second postoperative decade.
本研究旨在确定1）常见先天性心脏病手术后猝死的风险；以及2）与猝死风险增加相关的因素。
尽管先天性心脏病患者通过手术治疗后预后有所改善，但他们仍面临着公认但定义不清的晚期猝死风险。
这项基于人群的研究评估了1958年至1996年间俄勒冈州所有19岁以下接受常见先天性心脏病手术治疗的患者。通过回顾性研究确定1958年以前的患者，并于1982年开始每两年进行一次前瞻性随访。对3589例因以下缺陷手术存活的患者的晚期猝死发生率和原因进行了评估：房间隔、室间隔和房室间隔缺损；动脉导管未闭；肺动脉狭窄；主动脉狭窄；主动脉缩窄；法洛四联症；以及大动脉转位。
在45857患者年的随访期间，有41例意外晚期猝死，总体事件发生率为1/1118患者年。41例晚期猝死中有37例发生在患有主动脉狭窄、主动脉缩窄、大动脉转位或法洛四联症的患者中，事件发生率为1/454患者年。相比之下，其他缺陷中仅发生4例猝死，事件发生率为1/7154患者年（p<0.01）。法洛四联症、主动脉狭窄和主动脉缩窄手术后20年，晚期猝死风险逐渐增加。然而，风险并不取决于手术时的患者年龄或手术时代。猝死原因中，30例为心律失常，7例为循环系统（栓塞或动脉瘤破裂），4例为急性心力衰竭。
常见先天性心脏病手术后存活患者的晚期猝死风险比年龄匹配的对照人群高25至100倍。这种增加的风险主要体现在患有青紫型或左心梗阻性病变的患者中。猝死风险似乎与时间有关，主要在术后第二个十年后增加。
