Long-term survival, modes of death, and predictors of mortality in patients with Fontan surgery
Long-term survival, modes of death, and predictors of mortality in patients with Fontan surgery
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DOI：
10.1161/circulationaha.107.738559
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发表时间：
2008-01-01
期刊：
影响因子：
37.8
通讯作者：
Landzberg, Michael J.
中科院分区：
文献类型：
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作者：
Khairy, Paul;Fernandes, Susan M.;Landzberg, Michael J.
背景 - 为了更好地确定单心室生理患者的死亡决定因素，创建了一个数据库登记系统，纳入1985年或更早出生且在波士顿儿童医院接受随访的行Fontan手术的患者。
方法与结果 - 共有261例患者，其中121例（46.4%）为女性，首次Fontan手术的中位年龄为7.9岁：135例（51.7%）为右心房 - 肺动脉连接；25例（9.6%）为右心房至右心室连接；101例（38.7%）为全腔静脉 - 肺动脉连接。在中位随访12.2年期间，76例（29.1%）死亡，5例（1.9%）接受心脏移植，5例（1.9%）进行Fontan手术修正，21例（8.0%）进行Fontan转换。围手术期死亡率随时间稳步下降，占所有死亡病例的68.4%。在早期幸存者中，5年、10年、15年和20年时无死亡或移植的精算生存率分别为93.7%、89.9%、87.3%和82.6%，右心房 - 肺动脉连接与全腔静脉 - 肺动脉连接之间无显著差异。晚期死亡分类如下：7例（9.2%）为猝死，6例（7.9%）为血栓栓塞性死亡，5例（6.7%）与心力衰竭相关，2例（2.6%）为败血症，4例（5.2%）为其他原因。大多数猝死推测为心律失常源性，无明确的预测因素。血栓栓塞性死亡的独立危险因素为未进行抗血小板或抗凝治疗（风险比[HR]，91.6；P = 0.0041）以及临床诊断的心内血栓（HR，22.7；P = 0.0002）。心力衰竭死亡的独立预测因素为蛋白丢失性肠病（HR，7.1；P = 0.0043）、单一形态学右心室（HR，10.5；P = 0.0429）以及较高的右心房压力（HR，每1 mmHg为1.3；P = 0.0016）。
结论 - 在Fontan手术的围手术期幸存者中，逐渐减员主要源于血栓栓塞性、心力衰竭相关和猝死。
Background-To better define determinants of mortality in patients with univentricular physiology, a database registry was created of patients born in 1985 or earlier with Fontan surgery who were followed up at Children's Hospital Boston.Methods and Results-A total of 261 patients, 121 of whom (46.4%) were women, had a first Fontan surgery at a median age of 7.9 years: right atrium-to-pulmonary artery connection in 135 (51.7%); right atrium to right ventricle in 25 (9.6%); and total cavopulmonary connection in 101 (38.7%). Over a median of 12.2 years, 76 (29.1%) died, 5 (1.9%) had cardiac transplantation, 5 (1.9%) had Fontan revision, and 21 (8.0%) had Fontan conversion. Perioperative mortality decreased steadily over time and accounted for 68.4% of all deaths. In early survivors, actuarial freedom from death or transplantation was 93.7%, 89.9%, 87.3%, and 82.6% at 5, 10, 15, and 20 years, respectively, with no significant difference between right atrium to pulmonary artery versus total cavopulmonary connection. Late deaths were classified as sudden in 7 patients (9.2%), thromboembolic in 6 (7.9%), heart failure-related in 5 (6.7%), sepsis in 2 (2.6%), and other in 4 (5.2%). Most sudden deaths were of presumed arrhythmic origin with no identifiable predictor. Independent risk factors for thromboembolic death were lack of antiplatelet or anticoagulant therapy (hazard ratio [HR], 91.6; P = 0.0041) and clinically diagnosed intracardiac thrombus (HR, 22.7; P = 0.0002). Independent predictors of heart failure death were protein-losing enteropathy (HR, 7.1; P = 0.0043), single morphologically right ventricle (HR, 10.5; P = 0.0429), and higher right atrial pressure (HR, 1.3 per 1 mm Hg; P = 0.0016).Conclusion-In perioperative survivors of Fontan surgery, gradual attrition occurs predominantly from thromboembolic, heart failure-related, and sudden deaths.