Department of Cardiovascular Diseases, Mayo Clinic, Rochester, MN, USA.
Division of Pediatric Cardiology, Department of Pediatric and Adolescent Medicine, Mayo Clinic, Rochester, MN, USA.
Eur J Heart Fail. 2019 Jun;21(6):803-809. doi: 10.1002/ejhf.1365. Epub 2019 Jan 23.
The Fontan operation is associated with significant long-term morbidity and mortality, posing challenges in management. No haemodynamic classification has been established to aid management and prognostication of patients with Fontan palliation. Our aim was to assess haemodynamic profiles for Fontan patients and evaluate their relationships to prognosis.
Eighty-four adult Fontan patients without obstruction or significant valve disease undergoing cardiac catheterization for Fontan-related co-morbidities were divided into four different haemodynamic profiles based on normal/high Fontan pressures (FP) (< 15 or ≥ 15 mmHg) and low/normal cardiac index (CI) (< 2.5 or ≥ 2.5 L/min/m ). Fourteen patients had low CI/high FP (17%), 24 low CI/normal FP (29%), 20 normal CI/high FP (24%), and 26 normal CI/normal FP (31%). Demographic and clinical data were similar among groups except for a trend towards a higher prevalence of cirrhosis in the normal CI/high FP (50%) group. Systemic vascular afterload, assessed by systemic vascular resistance and effective arterial elastance, was higher in patients with low CI/high FP and low CI/normal FP as compared to normal CI profiles. Pulmonary vascular resistance indices were higher in patients with low CI/high FP than all other profiles. The normal CI/high FP haemodynamic profile was an independent predictor of mortality (hazard ratio 4.1, 95% confidence interval 1.2-11.5). Heterotaxy and protein-losing enteropathy were also predictors of mortality on the multivariate model.
The proposed haemodynamic classification provides prognostic information in adults post-Fontan with patients in the normal CI/high FP profile having worse survival. Whether this classification can guide management in these patients deserves further investigation.
Fontan 手术与重大的长期发病率和死亡率相关，对管理提出了挑战。目前还没有建立血流动力学分类来帮助管理和预测 Fontan 姑息治疗患者。我们的目的是评估 Fontan 患者的血流动力学特征，并评估它们与预后的关系。
84 名接受心脏导管检查以治疗 Fontan 相关合并症的无梗阻或明显瓣膜疾病的成年 Fontan 患者，根据正常/高 Fontan 压力（<15 或≥15mmHg）和低/正常心指数（<2.5 或≥2.5L/min/m）将其分为四种不同的血流动力学类型。14 例患者为低 CI/高 FP（17%），24 例为低 CI/正常 FP（29%），20 例为正常 CI/高 FP（24%），26 例为正常 CI/正常 FP（31%）。除了正常 CI/高 FP（50%）组肝硬化的患病率较高外，各组之间的人口统计学和临床数据相似。低 CI/高 FP 和低 CI/正常 FP 患者的全身血管后负荷，通过全身血管阻力和有效动脉弹性来评估，高于正常 CI 组。与所有其他类型相比，低 CI/高 FP 患者的肺血管阻力指数更高。正常 CI/高 FP 血流动力学类型是死亡的独立预测因子（危险比 4.1，95%置信区间 1.2-11.5）。非典型心脏和蛋白丢失性肠病也是多变量模型中死亡的预测因子。
所提出的血流动力学分类为 Fontan 术后成年人提供了预后信息，正常 CI/高 FP 谱中的患者生存状况较差。这种分类是否能指导这些患者的管理值得进一步研究。
