Sibley Heart Center Cardiology, Emory University, Children's Healthcare of Atlanta, Atlanta, Georgia.
Am J Cardiol. 2013 Dec 15;112(12):1943-7. doi: 10.1016/j.amjcard.2013.08.023. Epub 2013 Sep 25.
Fontan failure can occur even with normal systolic ventricular function and often in the context of significant liver disease. We hypothesized that Fontan failure is hemodynamically distinct from traditional heart failure and characterized by low systemic vascular resistance (SVR) index and preserved cardiac index. Twenty-seven symptomatic adult Fontan (SAF) patients who underwent catheterization from 2001 to 2011 constituted our study group. Fifty-four predominantly asymptomatic pediatric Fontan (PF) patients who underwent catheterization during the same period were randomly selected to perform a control:case cohort analysis. Clinical comparisons were made between the 2 groups. The adults were more symptomatic than the PF cohort (New York Heart Association classes I and II or III and IV: 48% or 52% [SAF] vs 94% or 6% [PF], respectively, p <0.01). SAF versus PF mean catheterization findings were central venous pressure 18 ± 6 versus 14 ± 3 mm Hg (p <0.01), SVR index 1,680 ± 368 versus 1,960 ± 550 dyn s/cm(5)/m(2) (p = 0.02), and cardiac index 2.7 ± 0.8 versus 2.8 ± 0.7 L/min/m(2) (p = 0.25). By imaging, the SAF cohort demonstrated a greater incidence of abnormal liver texture changes (96% vs 75%, p = 0.04) and nodularity (77% vs 42%, p = 0.02). In conclusion, adult patients with failing Fontan circulation had a lower SVR index and similar cardiac index compared with the pediatric cohort. Liver disease in the adults was more advanced. Our data suggest that Fontan failure is a distinct circulatory derangement with hemodynamic features similar to portal hypertension, albeit with limited ability to augment cardiac output.
即使心室收缩功能正常，Fontan 衰竭也可能发生，而且常常伴有严重的肝脏疾病。我们假设 Fontan 衰竭在血流动力学上与传统心力衰竭不同，其特征为全身血管阻力（SVR）指数低和心输出量指数正常。我们的研究组包括 2001 年至 2011 年期间接受心导管检查的 27 例有症状的成人 Fontan（SAF）患者。同期随机选择了 54 例主要无症状的儿科 Fontan（PF）患者进行对照：病例队列分析。对两组患者进行临床比较。成人比 PF 队列更有症状（纽约心脏协会心功能分级 I 和 II 或 III 和 IV：48%或 52%[SAF]比 94%或 6%[PF]，p<0.01）。SAF 与 PF 的平均心导管检查结果分别为中心静脉压 18±6 毫米汞柱与 14±3 毫米汞柱（p<0.01），SVR 指数 1680±368 与 1960±550 达因·秒·厘米-5·米-2（p=0.02），心输出量指数 2.7±0.8 与 2.8±0.7 升/分/米-2（p=0.25）。通过影像学检查，SAF 组显示异常肝脏纹理改变的发生率更高（96%比 75%，p=0.04）和结节（77%比 42%，p=0.02）。总之，与儿科患者相比，成人 Fontan 循环衰竭患者的 SVR 指数较低，心输出量指数相似。成人的肝脏疾病更严重。我们的数据表明，Fontan 衰竭是一种独特的循环紊乱，其血流动力学特征与门脉高压相似，尽管其增加心输出量的能力有限。
