The Department of Cardiovascular Medicine, Mayo Clinic Rochester, MN 55906, United States.
Department of Internal Medicine, Wayne State University/Detroit Medical Center, Detroit, MI, United States.
Int J Cardiol. 2018 Nov 15;271:312-316. doi: 10.1016/j.ijcard.2018.05.039.
Elevation in central venous pressure (CVP) plays a fundamental pathophysiologic role in Fontan circulation. Because there is no sub-pulmonary ventricle in this system, CVP also provides the driving force for pulmonary blood flow. We hypothesized that this would make Fontan patients more susceptible to even low-level elevation in pulmonary vascular resistance index (PVRI), resulting in greater systemic venous congestion and adverse outcomes.
Adult Fontan patients and controls without congenital heart disease undergoing clinical evaluation that included cardiac catheterization and echocardiography were examined retrospectively. Outcomes including all-cause mortality and the development of Fontan associated diseases (FAD, defined as protein losing enteropathy, cirrhosis, heart failure hospitalization, arrhythmia, or thromboembolism) were assessed from longitudinal assessment.
As compared to controls (n = 82), Fontan patients (n = 164) were younger (36 vs 45 years, p < 0.001), more likely to be on anticoagulation or antiplatelet therapy, and more likely to have atrial arrhythmia or cirrhosis. There was a strong correlation between CVP and PVRI in the Fontan group (r = 0.79, p < 0.001), but there was no such relationship in controls. Elevated PVRI identified patients at increased risk for FAD (HR 1.92, 95% CI 1.39-2.41, p = 0.01), and composite endpoint of FAD and/or death (HR 1.89, 95% CI 1.32-2.53, p = 0.01) per 1 WU∗m increment.
Systemic venous congestion, which is the primary factor in the pathogenesis of FAD and death, is related to even low-level abnormalities in pulmonary vascular function. Multicenter studies are needed to determine whether interventions targeting pulmonary vascular structure and function can improve outcomes in the Fontan population.
中心静脉压（CVP）升高在 Fontan 循环中起着基本的病理生理作用。由于该系统中没有肺下腔室，因此 CVP 也为肺血流提供驱动力。我们假设这会使 Fontan 患者更容易受到肺血管阻力指数（PVRI）即使是低水平升高的影响，从而导致更严重的全身静脉充血和不良后果。
回顾性检查了接受临床评估（包括心导管检查和超声心动图）的成年 Fontan 患者和无先天性心脏病的对照者。从纵向评估中评估了包括全因死亡率和 Fontan 相关疾病（FAD，定义为蛋白丢失性肠病、肝硬化、心力衰竭住院、心律失常或血栓栓塞）的发展在内的结局。
与对照组（n=82）相比，Fontan 患者（n=164）年龄更小（36 岁 vs 45 岁，p<0.001），更可能接受抗凝或抗血小板治疗，并且更可能有心房心律失常或肝硬化。Fontan 组中 CVP 与 PVRI 之间存在很强的相关性（r=0.79，p<0.001），但对照组中没有这种关系。升高的 PVRI 确定了患有 FAD 风险增加的患者（HR 1.92，95%CI 1.39-2.41，p=0.01），以及 FAD 和/或死亡的复合终点（HR 1.89，95%CI 1.32-2.53，p=0.01）每增加 1 个 WU∗m。
导致 FAD 和死亡的主要因素——全身静脉充血与肺血管功能的轻度异常有关。需要进行多中心研究，以确定针对肺血管结构和功能的干预措施是否可以改善 Fontan 人群的结局。
