Ohuchi Hideo, Yasuda Kenji, Miyazaki Aya, Iwasa Toru, Sakaguchi Heima, Shin Ono, Mizuno Masanori, Negishi Jun, Noritake Kanae, Yamada Osamu
Department of Pediatric Cardiology, National Cerebral and Cardiovascular Center, 5-7-1, Fujishiro-dai, Suita, Osaka 565-8565, Japan.
Department of Pediatric Cardiology, National Cerebral and Cardiovascular Center, 5-7-1, Fujishiro-dai, Suita, Osaka 565-8565, Japan.
Int J Cardiol. 2014 May 1;173(2):277-83. doi: 10.1016/j.ijcard.2014.03.001. Epub 2014 Mar 5.
Non-cardiac complications, such as hepato-renal and metabolic problems, are emerging late after the Fontan operation due to its unique hemodynamics. Consequently, associations between clinical variables and postoperative outcome may change during the prolonged postoperative course.
To determine if child and adult Fontan patients differ in the impact of cardiac and non-cardiac variables on clinical outcome, we prospectively evaluated associations between hemodynamics, neurohumoral factors, exercise variables, hepato-renal function and metabolic variables and unscheduled hospitalization, including death in 167 consecutive child and 116 adult Fontan patients. When compared with child patients, the adult patients showed higher rates of medications, lower cardiac index, higher values of natriuretic peptides, greater renal dysfunction, more cholestatic livers, and more impaired responses to exercise (p<0.05-0.0001). During the follow-up of 3.7 ± 2.1 years, 64 clinical events (37 in adults), including 13 deaths, occurred. A high CVP and low arterial oxygen satutration strongly predicted the child events (p<0.001), whereas these prognostic parameters were marginal in the adults. Instead, renal dysfunction and metabolic abnormality predicted adult events (p<0.05). Neurohumoral activation, low albumin, hyponatremia, and impaired exercise variables equally predicted clinical events in child and adult Fontan patients.
Distinctive differences in predictive value of clinical variables exist between child and adult Fontan patients. In addition to cardiac issues, we should consider non-cardiac determinents of clinical outcome to maximize our efforts to improve prognosis for adult Fontan survivors.
由于Fontan手术独特的血流动力学特点，肝肾功能及代谢问题等非心脏并发症在Fontan手术后晚期出现。因此，在术后漫长的病程中，临床变量与术后结局之间的关联可能会发生变化。
为确定儿童和成人Fontan患者在心脏及非心脏变量对临床结局的影响方面是否存在差异，我们前瞻性评估了167例连续的儿童Fontan患者和116例成人Fontan患者的血流动力学、神经体液因子、运动变量、肝肾功能及代谢变量与非计划住院（包括死亡）之间的关联。与儿童患者相比，成人患者的用药率更高、心脏指数更低、利钠肽值更高、肾功能障碍更严重、肝脏胆汁淤积更明显、运动反应受损更严重（p<0.05 - 0.0001）。在3.7±2.1年的随访期间，发生了64例临床事件（成人37例），包括13例死亡。高中心静脉压和低动脉血氧饱和度强烈预测儿童的临床事件（p<0.001），而这些预后参数在成人中作用有限。相反，肾功能障碍和代谢异常预测成人的临床事件（p<0.05）。神经体液激活、低白蛋白、低钠血症及运动变量受损对儿童和成人Fontan患者的临床事件具有同等的预测作用。
儿童和成人Fontan患者在临床变量预测价值方面存在显著差异。除了心脏问题外，我们应考虑临床结局的非心脏决定因素，以最大程度地努力改善成人Fontan手术幸存者的预后。
