Longitudinal Outcomes of Patients With Single Ventricle After the Fontan Procedure.
Longitudinal Outcomes of Patients With Single Ventricle After the Fontan Procedure.
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DOI：
10.1016/j.jacc.2017.03.582
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发表时间：
2017-06-06
影响因子：
24
通讯作者：
Pediatric Heart Network Investigators
中科院分区：
文献类型：
--
作者：
Atz AM;Zak V;Mahony L;Uzark K;D'agincourt N;Goldberg DJ;Williams RV;Breitbart RE;Colan SD;Burns KM;Margossian R;Henderson HT;Korsin R;Marino BS;Daniels K;McCrindle BW;Pediatric Heart Network Investigators
Fontan患者存活至成年期的多中心纵向客观数据缺乏。描述无移植存活率，并探索心室性能实验室指标与功能状态随时间变化的关系。在Fontan横断面研究（Fontan 1）后9.4 ± 0.4年重复进行运动试验、超声心动图、B型利钠肽（BNP）、功能健康评估和病史提取，并与既往值进行比较。考克斯回归分析探讨了中期死亡或心脏移植的危险因素。在546例受试者的原始队列中，466例再次联系，373例（80%）入组时年龄为21.2 ± 3.5岁。在进行配对检验的受试者中，预测最大VO 2降低的百分比（69 ± 14 vs. 61 ± 16，p <0.001，n = 95），射血分数降低（58 ± 11 vs. 55 ± 10，p <0.001，n=259），BNP升高（中位数（IQR）13 vs. 18（9，36）pg/mol，p <0.001，n = 340）。在最近的随访中，较低的儿科生活质量量表（PedsQL）身体综合评分与较差的运动表现相关（R2调整= 0.20，p <0.001，n = 274）。Fontan以来的累积并发症包括额外的心脏手术（32%）、导管介入（62%）、心律失常治疗（32%）、血栓形成（12%）和蛋白丢失性肠病（8%）。自Fontan 1以来，54例受试者（10%）接受了心脏移植（n = 23）或在未接受移植的情况下死亡（n = 31）。死亡/移植的间隔风险与Fontan 1评估的心室性能和功能健康状态较差相关，但与心室形态、受试者年龄或Fontan连接类型无关。Fontan队列中12年的中期无移植生存率为90%，与心室形态无关。运动表现下降，并与更差的功能健康状况。未来的干预措施可能侧重于保持运动能力。（临床试验注册号：NCT 00132782）
Multicenter longitudinal objective data for Fontan patients surviving into adulthood are lacking. Describe transplant-free survival and explore relationships between laboratory measures of ventricular performance and functional status over time. Exercise testing, echocardiography, B-type natriuretic peptide (BNP), functional health assessment, and medical history abstraction were repeated 9.4 ± 0.4 years after the Fontan Cross-Sectional Study (Fontan 1) compared to previous values. Cox regression analysis explored risk factors for interim death or cardiac transplantation. From the original cohort of 546 subjects, 466 were recontacted and 373 (80%) were enrolled at 21.2 ± 3.5 years of age. Among subjects with paired testing, percent predicted maximum VO2 decreased (69 ± 14 vs. 61 ± 16, p <0.001, n = 95), ejection fraction decreased (58 ± 11 vs. 55 ± 10, p <0.001, n=259), and BNP increased (Median (IQR) 13 vs. 18 (9,36) pg/mol, p <0.001, n = 340). At latest follow-up lower Pediatric Quality of Life Inventory (PedsQL) physical summary score was associated with poorer exercise performance (R2 adjusted = 0.20, p <0.001, n = 274). Cumulative complications since the Fontan included additional cardiac surgery (32%), catheter intervention (62%), arrhythmia treatment (32%), thrombosis (12%), and protein losing enteropathy (8%). Since Fontan 1, 54 subjects (10%) have received a heart transplant (n = 23) or died without transplantation (n = 31). The interval risk of death/transplantation was associated with poorer ventricular performance and functional health status assessed at Fontan 1, but was not associated with ventricular morphology, subject age or type of Fontan connection. Interim transplant-free survival over 12 years in this Fontan cohort was 90% and was independent of ventricular morphology. Exercise performance decreased and was associated with worse functional health status. Future interventions might focus on preserving exercise capacity. (Clinical Trials Registration #: NCT00132782)
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