背景:Fontan生理学会导致其他器官系统的慢性变化,这可能会影响长期存活和心脏移植的成功。对Fontan的心脏外影响的评估和治疗不足可能会导致不良结局。Fontan并发症的严重程度分级/顺序共识定义缺乏,这限制了对Fontan特异性发病率如何影响患者预后的理解。
结果:Fontan患者和生理学专家小组,包括儿科,成人先天性,心力衰竭,和重症监护心脏病学,和小儿肾脏病学,肝病学和心理学,召开会议以制定Fontan并发症的定义。定义是使用严重性分级创建的,序数:1级-轻度;2级中度;3级-重度;4级-致残或危及生命。定义创建后,由21名Fontan循环衰竭专家组成的第二个小组使用改良的Delphi方法对定义进行修改和投票,直至就最终定义达成共识(>90%的一致性,未建议进一步修改).经过三轮修改和投票,就Fontan的所有具体定义达成了共识。Fontan的定义的并发症和发病率包括:解剖Fontan通路阻塞,紫癜,由静脉功能不全引起的全身静脉异常,房性心律失常,室性心律失常,心动过缓,慢性胸腔积液,慢性腹水,蛋白质丢失性肠病,塑料支气管炎,咯血和肺出血,睡眠呼吸暂停,Fontan相关性肝病,门静脉和肝静脉曲张疾病,急性肾损伤影响临床治疗,红细胞增多症,血栓性疾病,反复或严重的细菌感染,皮肤萎缩,肾上腺功能不全,先前中风的身体影响,情绪/行为障碍和神经发育障碍。
结论:共识,Fontan特异性心脏和心脏外并发症的严重程度分级定义已完成,可用于研究.它们将允许未来对Fontan患者结果进行稳健分析。
BACKGROUND: Fontan physiology leads to chronic changes in other organ systems that may affect long-term survival and the success of heart transplantation. Inadequate assessment and treatment of the extra-cardiac effects of Fontan may contribute to poor outcomes. Severity-graded/ordinal
consensus definitions of Fontan complications are lacking, which limits understanding of how Fontan-specific morbidity affects patients\' outcomes.
RESULTS: A panel of Fontan patient and physiology experts, including pediatric, adult congenital, heart failure, and critical-care cardiology as well as pediatric nephrology, hepatology and psychology, convened to develop definitions of Fontan complications. Definitions were created by using a severity-graded ordinal scale: grade 1, mild; grade 2, moderate; grade 3, severe; grade 4, disabling or life threatening. Following definition creation, a second panel of 21 experts in Fontan circulatory failure used a modified Delphi methodology to modify and vote on definitions until
consensus (> 90% agreement without recommended further modification) was reached on final definitions. After 3 rounds of modifications and voting,
consensus agreement was achieved on all Fontan-specific definitions. The defined complications and morbidities of Fontan include: anatomic Fontan pathway obstruction, cyanosis, systemic venous abnormalities resulting from venous insufficiency, atrial arrhythmia, ventricular arrhythmia, bradycardia, chronic pleural effusions, chronic ascites, protein-losing enteropathy, plastic bronchitis, hemoptysis and pulmonary hemorrhage, sleep apnea, Fontan-associated liver disease, portal and hepatic variceal disease, acute kidney injury affecting clinical treatment, polycythemia, thrombotic disease, recurrent or severe bacterial infection, skin atrophy, adrenal insufficiency, physical impact of previous stroke, mood/behavior disorder, and neurodevelopmental disorder.
CONCLUSIONS: Consensus and severity-graded definitions of Fontan-specific cardiac and extra-cardiac complications were achieved and are available for use in research. They will allow future robust analyses of Fontan patient outcomes.